Infecciones más frecuentes en pacientes con Lupus Eritematoso Sistémico internados en el Servicio de Clínica Médica del Hospital Nacional / Most frequent infections in patients with Systemic Lupus Erythematosus admitted to the Medical Clinic Service of the Hospital Nacional

Introducción: el lupus eritematoso sistémico (LES) se conoce como una enfermedad autoinmune multisistémica, cuya causa es desconocida, se caracteriza por la presencia de complejos inmunes y autoanticuerpos. En series internacionales se describe una presentación de procesos infecciosos en estos pacientes hasta en un 75 % de los casos, las infecciones ocurren tanto al inicio de la enfermedad como en etapas tardías, y son causa directa de muerte en 30 % a 60 % de los casos y motivo de hospitalización hasta de 30 %. Objetivos: determinar los procesos infecciosos más frecuentes en pacientes con lupus eritematoso sistémico internados en el Servicio de Clínica Médica del Hospital Nacional en el periodo 2022-2023. Metodología: diseño observacional, descriptivo de corte transversal de pacientes con diagnóstico de lupus eritematoso sistémico según criterios de European League Against Rheumatism (EULAR)-2019 que se encuentran internados en el servicio de Clínica Médica del Hospital Nacional (Itaugua-Paraguay) en el periodo de 2022-2023. Resultados: la edad media de los pacientes fue de 34 años, con predominio del sexo femenino en el 88.18 %. Las infecciones del aparato respiratorio fue la más frecuentemente diagnosticada en 50 (45.45 %) pacientes, seguido por la infección de vías urinarias 47 (42.38 %) pacientes, el condicionante de riesgo predominante fue el uso de corticoides en un total 105 (96.40 %) pacientes, se estableció en un total de 97 (88.18 %) pacientes con antibioticoterapia, dentro del aspecto demográfico de la zona urbana 56 (50.90 %) pacientes y rural 54(49.1 %). Conclusión: la infección del aparato respiratorio fue la infección más frecuente, el condicionante de riesgo predominante es el uso de corticoides y recibieron antibioticoterapia la cual en monoterapia fue la más utilizada

Introduction: systemic lupus erythematosus (SLE) is known as a multisystem autoimmune disease, whose cause is unknown, and is characterized by the presence of immune complexes and autoantibodies. In international series, presentation of infectious processes is described in these patients in up to 75 % of cases. Infections occur both at the beginning of the disease and in late stages, and are a direct cause of death in 30 % to 60 % of patients. Cases and reason for hospitalization up to 30 %. Objectives: determine the most frequent infectious processes in patients with systemic lupus erythematosus admitted to the Medical Clinic Service of the Hospital Nacional in the period 2022-2023. Methodology: observational, descriptive cross-sectional design of patients with a diagnosis of systemic lupus erythematosus according to criteria of European League Against Rheumatism (EULAR)-2019 who are admitted to the Medical Clinic service of the Hospital Nacional (Itauguá-Paraguay) in the period of 2022-2023. Results: the average age of the patients was 34 years, with a predominance of the female sex in 88.18 %. Respiratory system infections were the most frequently diagnosed in 50 (45.45 %) patients, followed by urinary tract infection in 47 (42.38 %) patients. The predominant risk factor was the use of corticosteroids in a total of 105 (96.40 %) patients, it was established in a total of 97 (88.18 %) patients with antibiotic therapy, within the demographic aspect of the urban area 56 (50.90 %) patients and rural 54 (49.1 %). Conclusion: respiratory tract infection was the most frequent infection, the predominant risk factor is the use of corticosteroids and they received antibiotic therapy, which in monotherapy was the most used.

IL-1β and IL-17 in cutaneous lupus erythematous skin biopsies: could immunohistochemicals indicate a tendency towards systemic involvement?

Abstract Background: Only a fraction of patients with cutaneous lupus erythematosus (CLE) will eventually progress toward systemic disease (SLE). Objective: To find inflammatory biomarkers which could predict the progression of cutaneous lupus erythematosus (CLE) into systemic lupus erythematosus (SLE) using immunohistochemical (IHC) assays. Methods: Immunohistochemical markers for cytotoxic, inflammatory, and anti-inflammatory responses and morphometric methods were applied to routine paraffin sections of skin biopsies, taken from lesions of 59 patients with discoid lupus, subacute lupus, and lupus tumidus. For the diagnosis of SLE, patients were classified by both the American College of Rheumatology (ACR-82) and the Systemic Lupus International Collaborating Clinics (SLICC-12) systems. Results: Skin samples from CLE/SLE +patients presented higher expression of IL-1β (ARC-82: p = 0.024; SLICC-12: p = 0.0143) and a significantly higher number of cells marked with granzyme B and perforin (ARC: p = 0.0097; SLICC-12: p = 0.0148). Biopsies from CLE/SLE- individuals had higher expression of IL-17 (ARC-82: p = 0.0003; SLICC-12: p = 0.0351) and presented a positive correlation between the density of granzyme A+and FoxP3+ cells (ARC-82: p = 0.0257; SLICC-12: p = 0.0285) and CD8+ cells (ARC-82: p = 0.0075; SLICC-12: p = 0.0102), as well as between granulysin-positive and CD8+ cells (ARC-82: p = 0.0024; SLICC-12: p = 0.0116). Study limitations: Patients were evaluated at a specific point in their evolution and according to the presence or not of systemic disease. The authors cannot predict how many more, from each group, would have evolved towards SLE in the following years. Conclusions: In this cohort, immunohistochemical findings suggested that patients with a tendency to systemic disease will show strong reactivity for IL-1β, while those with purely cutaneous involvement will tend to express IL-17 more intensely.

Complete congenital heart block in the offspring of an asymptomatic woman with isolated high titer anti-ro antibody

@#Among pregnant women, 1-2% are anti-Ro positive and while half of them have symptoms of connective tissue disease, the rest are asymptomatic. The presence of anti-Ro is of concern because of the risk of congenital heart block in the child. We report the case of an asymptomatic 27-year-old G2P1(1001) woman, who presented with persistent fetal bradycardia in her 21st week of gestation (AOG) and was found to have elevated titers for anti-Ro (>320 U/ml). Hydroxychloroquine 200 mg/day and prednisone 10 mg/day were given from the 33rd week of gestation up until the delivery. At 37 weeks AOG, she delivered a live male neonate with a complete heart block. On the 6th day of life, the infant remained bradycardic, hence a pacemaker was inserted and heart rate maintained at 100-120 bpm. On subsequent follow-ups, the mother and child did not develop any systemic manifestations and the infant was thriving well. While a diseased condition may not be apparent in a pregnant anti-Ro positive woman, the risk of neonatal lupus (NL) is demonstrated in this patient’s case. This report illustrates how prenatal care of an asymptomatic woman led to the discovery of a fetal abnormality and served to prepare the family and the medical team to ably handle the birth and subsequent care of a neonate with NL.

Childhood-onset systemic lupus erythematosus (cSLE) and malignancy: a nationwide multicentre series review

Abstract Background Increased malignancy frequency is well documented in adult-systemic lupus erythematosus (SLE), but with limited reports in childhood-onset SLE (cSLE) series. We explored the frequency of malignancy associated with cSLE, describing clinical and demographic characteristics, disease activity and cumulative damage, by the time of malignancy diagnosis. Method A retrospective case-notes review, in a nationwide cohort from 27 Pediatric Rheumatology centres, with descriptive biopsy-proven malignancy, disease activity/damage accrual, and immunosuppressive treatment were compiled in each participating centre, using a standard protocol. Results Of the 1757 cSLE cases in the updated cohort, 12 (0.7%) developed malignancy with median time 10 years after cSLE diagnosis. There were 91% females, median age at cSLE diagnosis 12 years, median age at malignancy diagnosis 23 years. Of all diagnosed malignancies, 11 were single-site, and a single case with concomitant multiple sites; four had haematological (0.22%) and 8 solid malignancy (0.45%). Median (min-max) SLEDAI-2 K scores were 9 (0-38), median (min-max) SLICC/ACR-DI (SDI) score were 1 (1-5) Histopathology defined 1 Hodgkin's lymphoma, 2 non-Hodgkin's lymphoma, 1 acute lymphoblastic leukaemia; 4 gastrointestinal carcinoma, 1 squamous cell carcinoma of the tongue and 1 anal carcinoma; 1 had sigmoid adenocarcinoma and 1 stomach carcinoid; 3 had genital malignancy, being 1 vulvae, 1 cervix and 1 vulvae and cervix carcinomas; 1 had central nervous system oligodendroglioma; and 1 testicle germ cell teratoma. Conclusion Estimated malignancy frequency of 0.7% was reported during cSLE follow up in a multicentric series. Median disease activity and cumulative damage scores, by the time of malignancy diagnoses, were high; considering that reported in adult series.

Continuous Unilateral Cephalalgia Due to Systemic Lupus Erythematosus: A Case Report / Cefaleia unilateral contínua por lúpus eritematoso sistêmico: relato de caso

BACKGROUND: Hemicrania continua is a rare form of cephalalgia featuring a chronic and persistent headache in only one side of the head. OBJECTIVES: In this report, we present a case of a patient with hemicrania continua and systemic lupus erythematosus (SLE). METHODS: We collected patient data through the electronic medical record. Afterward, we reviewed the literature regarding hemicrania continua and its pathophysiology and correlation with neurovascular alterations, inflammation, and SLE. RESULTS: A 42-year-old woman visited the emergency department due to worsening constant unilateral cephalalgia that had been present for the past 6 months. The patient reported a highly intense (10/10) headache in the entire left hemicrania that radiated to the left shoulder. During physical examination, she presented with nystagmus, vertigo, and aggravated cephalalgia associated to body movement and, despite having no optic nerve thickening. In addition, she had jaundice, tachycardia, and splenomegaly. Complimentary exams found deep anemia, depletion in complement system and anti-nuclear factors, suggesting a possible hemolytic anemia (AIHA) due to SLE. Treatment was initiated with hydrocortisone and prednisone, associated with amitriptyline, fluoxetine and diazepam, reaching full remission. CONCLUSION: These syndromes have aggravated each other, and possibly the explanation for the cephalalgia remission was the control of AIHA and SLE. It features a rare case in literature and thus warrants discussion.

INTRODUÇÃO: Hemicrania contínua é uma forma rara de cefaléia caracterizada por cefaleia crônica e persistente em apenas um lado da cabeça. OBJETIVOS: Neste relato apresentamos o caso de um paciente com hemicrania contínua e lúpus eritematoso sistêmico (LES). MÉTODOS: Coletamos dados dos pacientes por meio do prontuário eletrônico. Posteriormente, revisamos a literatura sobre a hemicrania contínua e sua fisiopatologia e correlação com alterações neurovasculares, inflamação e LES. RESULTADOS: Uma mulher de 42 anos recorreu ao serviço de urgência devido ao agravamento da cefaleia unilateral constante, presente nos últimos 6 meses. O paciente relatou cefaleia de alta intensidade (10/10) em toda a hemicrânia esquerda com irradiação para o ombro esquerdo. Ao exame físico apresentava nistagmo, vertigem e cefaléia agravada associada à movimentação corporal e, apesar de não apresentar espessamento do nervo óptico. Além disso, ela apresentava icterícia, taquicardia e esplenomegalia. Os exames complementares evidenciaram anemia profunda, depleção do sistema complemento e fatores antinucleares, sugerindo uma possível anemia hemolítica (AIHA) por LES. Iniciou-se tratamento com hidrocortisona e prednisona, associadas a amitriptilina, fluoxetina e diazepam, atingindo remissão completa. CONCLUSÃO: Essas síndromes agravaram-se mutuamente e possivelmente a explicação para a remissão da cefaléia foi o controle da AIHA e do LES. Apresenta um caso raro na literatura e, portanto, merece discussão.

Evaluation of core decompression outcome in systemic lupus erythematosus with hip osteonecrosis: a retrospective cohort study

Abstract Background Osteonecrosis is a major cause of morbidity for patients with systemic lupus erythematosus (SLE). Although core decompression is an approved and trusted technique to prevent further joint deterioration, this surgical method seems to be less beneficial for SLE patients. We aimed to evaluate the outcomes of core decompression in SLE patients with primary stages of femoral head osteonecrosis. Methods In this study, 23 patients (39 affected hip joints) with osteonecrosis of the femoral head with stage II of the disease, based on the Ficat-Arlet classification system, underwent core decompression. Also, patients demographic characteristics, clinical data, medication history, comorbidities, immunological findings, hip plain radiographs, history of total hip arthroplasty after core decompression, and patients satisfaction with joint function according to the Oxford hip score questionnaire were obtained. Results In the study, 53.8% of affected joints showed signs of radiographic deterioration in follow-up imaging. Sixty-one and a half percent (61.5%) of patients had unsatisfactory joint performance. A third (33.3%) of affected hip joints underwent total hip arthroplasty up to 5 years from core decompression. SLE patients with a history of receiving bisphosphonate were 83.2% less dissatisfied with their joint function than patients without a history of bisphospho-nate use (P < 0.02). Of the 23 studied cases, the mean cumulative dose of prednisolone before and after core decompression surgery was 46.41 mg and 14.74 mg respectively. Besides, one case (2.6%) that had a high anti-phospholipid antibodies level during follow-up did not have any radiographic deterioration, and 9 cases (23.1%) had some degrees of radiographic deterioration. Conclusions The patients group that used bis-phosphonate, had a higher level of satisfaction with joint function after core decompression. Patients with high-level anti-phospholipid antibodies are related to a poor prognosis after core decompression.

What is known about the effects of vitamin D in neuropsychiatric lupus?

Abstract Systemic lupus erythematosus (SLE) is an autoimmune disease that can affect several organs and systems. The central and/or peripheral nervous system can suffer from complications known as neuropsychiatric lupus (NPSLE). Studies have associated the manifestations of SLE or NPSLE with vitamin D deficiency. It has been shown that hypovitaminosis D can lead to cognition deficits and cerebral hypoperfusion in patients with NPSLE. In this review article, we will address the main features related to vitamin D supplementation or serum vitamin D levels with neuropsychiatric manifestations, either in patients or in animal models of NPSLE.

Severe Hypocalcemia as an Atypical Manifestation of Seronegative Celiac Disease in a Patient with Systemic Lupus Erythematosus: Case Report

Aim: To describe the clinical picture and diagnosis of an episode of severe hypocalcemia in a patient with systemic lupus erythematosus (SLE) in remission, with chronic diarrhea that led to the diagnosis of celiac disease (CD). Case presentation: 22-year-old patient, diagnosed with SLE at age 10, in remission. He consulted for a two-month history of paresthesias, muscle spasms, myalgias, and episodes of tetany in the previous week, associated with malabsorptive postprandial diarrhea. His laboratory tests showed severe hypocalcemia, vitamin D deficiency, severe hypomagnesemia, and mild hypokalemia. In the study for CD, serology reported normal anti-tissue transglutaminase IgA, antigliadin IgA, and IgG and low total IgA levels. Enteroscopy revealed erosive bulbo-duodenitis, with villous atrophy and increased intraepithelial lymphocytes in the pathology, which, together with the immunohistochemical study, allowed the diagnosis of CD, Marsh 3a type. Management was initiated with a gluten-free diet, with a positive clinical response. Conclusion: CD should be suspected in patients with SLE who present with diarrhea, abdominal pain, nausea/vomiting, recurrent oral aphthosis, and anemia. In rare seronegative CD cases, other causes of villous atrophy, mainly infectious, toxic, and immunological, must be ruled out. There is an association between CD and SLE, so diagnosis must be early and timely with the best testing scheme available to achieve effective treatment before complications occur.

Objetivo: Describir la presentación clínica y diagnóstico de un episodio de hipocalcemia grave en un paciente con lupus eritematoso sistémico (LES) en remisión, con diarrea crónica que condujo al diagnóstico de enfermedad celíaca (EC). Presentación del caso: Paciente de 22 años, con diagnóstico de LES a los 10 años, en remisión. Consultó por 2 meses de evolución de parestesias, espasmos musculares, mialgias y episodios de tetania en la semana previa, asociado a diarrea posprandial malabsortiva. Sus laboratorios mostraron hipocalcemia grave, déficit de vitamina D, hipomagnesemia grave e hipopotasemia leve. En el estudio para EC la serología reportó antitransglutaminasa tisular IgA, antigliadina IgA e IgG normales y niveles de IgA total bajos. La enteroscopia evidenció bulboduodenitis erosiva, con atrofia de las vellosidades y aumento de linfocitos intraepiteliales en la patología, que junto al estudio inmunohistoquímico permitieron concluir el diagnóstico de EC, tipo Marsh 3a. Se inició el manejo con una dieta libre de gluten, con una respuesta clínica positiva. Conclusión: La EC debe sospecharse en pacientes con LES que inicien con diarrea, dolor abdominal, náuseas/vómitos, aftosis oral recurrente y anemia. En los casos de EC seronegativa, los cuales son poco comunes, se deben descartar otras causas de atrofia de las vellosidades principalmente infecciosas, tóxicas e inmunitarias. Existe una asociación entre EC y LES, por lo cual el diagnóstico debe ser precoz y oportuno con el mejor esquema de pruebas disponible para lograr realizar un tratamiento efectivo antes de que se presenten las complicaciones.

Evolución de la Enfermedad por el virus del Chikungunya en pacientes con Lupus Eritematoso Sistémico

Introducción: La Enfermedad por el virus del Chikungunya (CHIKV) es una virosis que se caracteriza por fiebre acompañada de dolores articulares. La epidemia de esta enfermedad en los últimos dos años causó más de cien mil casos confirmados y sospechosos, incluido personas con Lupus Eritematoso Sistémico (LES). Objetivo: Los objetivos del siguiente trabajo consistieron en determinar la evolución de la Enfermedad por el CHIKV en pacientes con LES, caracterizar a la población incluida en el estudio, determinar la frecuencia de la Enfermedad por el CHIKV en pacientes con LES, describir las manifestaciones clínicas de la Enfermedad por el CHIKV en pacientes con LES y registrar la impresión de los pacientes en relación con la actividad de la enfermedad y las manifestaciones clínicas que aparecieron posterior a la Enfermedad por el CHIKV. Metodología: El estudio realizado fue de tipo descriptivo de corte transversal. Se incluyeron 101 pacientes con Lupus Eritematoso Sistémico, a quienes se les encuestó mediante un formulario virtual. Los investigadores establecieron contacto con los pacientes a través de llamadas telefónicas o mensajería, y si los pacientes aceptaban participar en el estudio, se les enviaba el formulario o, en algunos casos, se completaba durante la llamada telefónica, si el paciente lo solicitaba. Resultados: El valor de la media de edad es de 38,2 ± 11,8 años. El 85,1% (86/101) de los pacientes corresponden al sexo femenino, mientras que el 14,9% (15/101) corresponde al sexo masculino. El 37,6% (38/101) presentó la enfermedad por el CHIKV, la cual se caracterizó en la fase aguda por la presencia de artralgias en un 84,2% (32/38), fiebre en un 71,1% (27/38), debilidad en un 57,9% (22/38) y cefalea en un 55,3% (21/38). En la fase subaguda, las artralgias se manifestaron mayoritariamente en un 73,7% (28/38) de los pacientes. La fase crónica estuvo presente en el 71,1% (27/38) con la persistencia de artralgias en un 81,48% (22/27) de los pacientes acompañada de otros síntomas como la artritis en un 40,74% (11/27). A causa de la enfermedad por el CHIKV, el 21,1% (8/38) recibió la indicación en la fase subaguda o crónica de realizar cambios en su medicación para el LES. El 62,5% (5/8) recibió la indicación de aumentar la dosis del corticoesteroide, mientras que el 50% (4/8) de aumentar la dosis del inmunosupresor. Según la impresión de los pacientes, en cuanto a la actividad de la enfermedad de base, posterior a la enfermedad por el CHIKV, presentaron una exacerbación del LES en un 10,5% (4/38) de los pacientes, de los cuales un 50% (2/4) refirieron exacerbación de síntomas ya existentes antes de la infección y la aparición de nuevos síntomas en un 50% (2/4). El 5,2% (2/38) tuvo requerimiento de hospitalización debido a la enfermedad por el CHIKV en la fase aguda. Conclusión: Las artralgias fueron frecuentes en las fases aguda, subaguda y crónica. En términos de tratamiento, algunos pacientes ajustaron sus medicaciones para el LES, aumentando la dosis de inmunosupresores o corticoides. En cuanto a la evolución, la mayoría de los pacientes no experimentaron una exacerbación de la enfermedad de base debido a la enfermedad por el CHIKV, al igual que solo un par de pacientes requirió hospitalización.

Introduction: Chikungunya virus (CHIKV) disease is a viral disease characterised by fever accompanied by joint pain. The epidemic of this disease in the last two years has caused more than 100,000 confirmed and suspected cases, including people with systemic lupus erythematosus (SLE). Objective: The objectives of the following work were to determine the evolution of CHIKV disease in patients with SLE, to characterise the study population, to determine the frequency of CHIKV disease in patients with SLE, to describe the clinical manifestations of CIHKV disease in patients with SLES, and to record the patients' impression of disease activity in relation to CHIKV disease in patients with SLE. Methodology: The study was a descriptive cross-sectional study. It included 101 patients with Systemic Lupus Erythematosus, who were surveyed using a virtual form. The investigators contacted patients by phone calls or messages, and if patients agreed to enter the study, the form was sent to them or, in some cases, completed during the telephone call, if requested by the patient. Results: The mean age value is 38.2 ± 11.8 years. The female sex accounted for 85.1% (86/101) of the patients, while 14.9% (15/101) were male. Chikungunya virus disease occurred in 37.6% (38/101) and was characterised in the acute phase by arthralgias in 84.2% (32/38), fever in 71.1% (27/38), weakness in 57.9% (22/38) and headache in 55.3% (21/38). In the subacute phase, arthralgias manifested in 73.7% (28/38) of patients. The chronic phase was present in 71.1% (27/38) with persistence of arthralgias in 81.48% (22/27) of patients accompanied by other symptoms such as arthritis in 40.74% (11/27). Due to CHIKV disease, 21.1% (8/38) received an indication in the subacute or chronic phase to make changes in their medication for SLE. 62.5% (5/8) received an indication to increase the dose of corticosteroid, while 50% (4/8) received an indication to increase the dose of immunosuppressant. According to the patients' impression of the baseline disease activity following CHIKV disease, patients reported an exacerbation of SLE in 10.5% (4/38) of patients, of whom 50% (2/4) reported exacerbation of symptoms already existing before infection, the appearance of new symptoms in 50% (2/4). Only 5.2% (2/38) referred to hospitalization due to CHKV disease in the acute phase. Conclusion: Arthralgias were frequent in the acute, subacute, and chronic phases. In terms of treatment, some patients adjusted their SLE medications, increasing the dose of immunosuppressants or corticosteroids. In terms of evolution, most patients did not experience an exacerbation of their baseline disease due to CHIKV disease, and only a couple of patients required hospitalization.

Características clínicas de pacientes con Lupus Eritematoso Sistémico, al debut y en recaídas

Introducción. Las formas clínicas de presentación del lupus eritematoso sistémico (LES) son variadas, así como las recaídas que ocurren en el curso de la enfermedad. Objetivo. Describir las formas de presentación del debut y las recaídas de pacientes con LES. Metodología. Estudio observacional, descriptivo, de corte transversal, de pacientes con LES, que acudieron al Hospital Nacional, durante el periodo 2022 a 2023. Las variables analizadas fueron las variables sociodemográficas, clínicas y de laboratorio, al debut y durante las recaídas. Resultados. Se incluyeron 47 pacientes, entre 14 y 68 años de edad (media: 29,6 ±12 años), 91,5% del sexo femenino, 59,5% del interior del país, con educación secundaria (68,1%) y ocupación ama de casa (55,3%). El tiempo de enfermedad fue en promedio 48,7 ±37.1 meses. La forma más frecuente de debut fue la poli-artralgia (55,3%), seguido de nefritis (27,7%). En la recaída, la nefritis fue más frecuente (44,7%), seguida de dolor poliarticular (40,4%). La causa de recaída más frecuente fue el abandono del tratamiento (34,0%). Conclusión. La mayoría de las pacientes eran mujeres jóvenes que debutaron la enfermedad con artralgias, seguida de la afectación renal. Las recaídas se presentaron con nefritis. La causa principal de las recaídas fue el abandono del tratamiento.

Introduction: The clinical forms of presentation of systemic lupus erythematosus (SLE) are varied, as well are the relapses that occur in the course of the disease. Objective: To describe the onset and relapses in a cohort of patients with SLE. Methodology. Observational, descriptive, cross-sectional study of patients with SLE who attended the National Hospital, during the period 2022 to 2023. The variables analyzed were sociodemographic, forms of onset and relapses. Results: 47 patients between 14 and 68 years of age (mean: 29.6 ±12 years) were included in the study, 91.5% female, 59.5% from the interior of the country, with secondary education (68.1%) and housewife occupation (55.3%). The duration of illness was on average 48.7 ±37.1 months. The most common form of onset was polyarthralgia (55.3%), followed by nephritis (27.7%). In relapse, nephritis was more frequent (44.7%), followed by polyarticular pain (40.4%). The most frequent cause of relapse was treatment abandonment (34.0%). Conclusion: The majority of patients were young women who presented the disease with arthralgia, followed by kidney involvement. Relapses presented with nephritis. The main cause of relapses were treatment abandonment.

Linfadenopatías generalizadas como manifestación inicial de lupus eritematoso sistémico: a propósito de un caso clínico

La presencia de linfadenopatías generalizadas se ha asociado de forma usual con lupus eritematoso sistémico (LES), sin embargo, no es usual encontrar este hallazgo como manifestación inicial de la enfermedad. Existen múltiples diagnósticos diferenciales que incluyen la linfadenitis necrotizante histiocítica de Kikuchi, la enfermedad de Castleman, infecciones y el linfoma cuando se presenta este hallazgo como síntoma inicial de LES. Presentamos el caso de un hombre de 56 años que se presentó con 2 meses de linfadenopatía generalizada sin datos al examen o antecedentes que sugirieran diagnóstico de LES; se sospechó inicialmente linfoma o enfermedad infecciosa y se realizó un estudio exhaustivo incluido biopsia de ganglio cervical. La investigación de laboratorio finalmente reveló leucopenia, proteinuria significativa, ANA y anti-dsDNA positivos e hipocomplementemia, lo que confirma el diagnóstico de enfermedad autoinmune tipo LES. Este caso ilustra la importancia de reconocer esta forma de presentación inusual, dado que se trata de una enfermedad potencialmente fatal.

The presence of generalized lymphadenopathy has usually been associated with systemic lupus erythematosus (SLE), however, it is not usual to find this finding as an initial manifestation of the disease. There are multiple differential diagnoses that include Kikuchi histiocytic necrotizing lymphadenitis, Castleman disease, infections and lymphoma when this finding is presented as an initial symptom of SLE. We present the case of a 56-year-old man who presented with 2 months of generalized lymphadenopathy without examination findings or history suggesting a diagnosis of SLE; Lymphoma or infectious disease was initially suspected and an exhaustive study was performed, including cervical lymph node biopsy. Laboratory investigation finally revealed leukopenia, significant proteinuria, positive ANA, positive anti-dsDNA, and hypocomplementemia, confirming the diagnosis of SLE-type autoimmune disease. This case illustrates the importance of recognizing this unusual presentation, given that it is a potentially fatal disease.

Anti-C1q como Marcador de Nefritis Lúpica / Anti-C1q as Lupus Nephritis Marker

Introducción: Los autoanticuerpos anti-C1q han sido propuestos como un marcador útil en el lupus eritematoso sistémico por su asociación con la nefritis lúpica. Objetivo: Determinar la prevalencia de anti-C1q en pacientes con lupus eritematoso sistémico y otras enfermedades reumáticas para la evaluar la asociación con la nefropatía lúpica. Métodos: Se incluyeron 179 pacientes con lupus eritematoso sistémico y 82 con otras enfermedades reumáticas. La nefritis lúpica fue diagnosticada en 70 (39 por ciento) de los pacientes con lupus eritematoso sistémico. Los anticuerpos anti-C1q IgG se determinaron por ELISA. Las asociaciones se evaluaron por análisis de regresión logística. Resultados: La prevalencia de anti-C1q fue de 37 poe ciento (66/179) en los pacientes con lupus eritematoso sistémico y de 9 por ciento (7/82) en controles (OR = 6,3; IC 95 por ciento 2,8-14,1; p < 0,001). El anti-C1q fue asociado con proteinuria (OR = 2,6; IC 95 por ciento 1,2-6,0; p < 0,022); eritrosedimentación elevada (OR = 3,2; IC 95 por ciento 1,5-6,7; p < 0,003) y anti-DNAdc (OR = 3,9; IC 95 por ciento 1,7-9,1; p < 0,002). En el modelo de regresión logística ajustado para demografía y anti-DNAdc, aunque la OR del anti-C1q para la nefritis fue 2 veces más alta que en ausencia del anti-C1q, solo se aproximó a la significación estadística. La positividad simultánea de anti-C1q y anti-DNAdc estuvo asociada a la nefritis lúpica (OR = 4,3; IC 95 por ciento 1,9-9,5; p < 0,001). Conclusiones: El anti-C1q se presentó con mayor frecuencia en pacientes con lupus eritematoso sistémico que en los controles. El anti-C1q combinado con anti-DNAdc resultó fuertemente asociado a la nefritis lúpica(AU)

Introducción: Anti-C1q autoantibodies have been proposed as useful marker in systemic lupus erythematosus due to their association with lupus nephritis. Objective: To determine the prevalence of anti-C1q in patients with systemic lupus erythematosus and other rheumatic diseases to evaluate the association with lupus nephropathy. Methods: One hundred seventy-nine patients with systemic lupus erythematosus and 82 with other rheumatic diseases were included. Lupus nephritis was diagnosed in 70 (39percent) of patients with systemic lupus erythematosus. Anti-C1q IgG antibodies were determined by ELISA. Associations were evaluated by logistic regression analysis. Results: The prevalence of anti-C1q was 37percent (66/179) in patients with systemic lupus erythematosus and 9percent (7/82) in controls (OR = 6.3; 95percent CI 2.8-14). .1; p < 0.001). Anti-C1q was associated with proteinuria (OR = 2.6; 95percent CI 1.2-6.0; p < 0.022); elevated erythrocyte sedimentation rate (OR = 3.2; 95percent CI 1.5-6.7; p < 0.003) and anti-dsDNA (OR = 3.9; 95percent CI 1.7-9.1; p < 0.002). In the logistic regression model adjusted for demographics and anti-dsDNA, although the OR of anti-C1q for nephritis was 2-fold higher than in the absence of anti-C1q, it only approached statistical significance. Simultaneous positivity of anti-C1q and anti-dsDNA was associated with lupus nephritis (OR = 4.3; 95percent CI 1.9-9.5; p < 0.001). Conclusions: Anti-C1q occurred more frequently in patients with systemic lupus erythematosus than in controls. Anti-C1q combined with anti-dsDNA was strongly associated with lupus nephritis(AU)

Fascitis necrotizante asociada a esteatohepatitis en el curso del lupus eritematoso sistémico / Necrotizing Fasciitis Associated with Steatohepatitis in The Course of Systemic Lupus Erythematosus

Introducción: La fascitis necrotizante es un cuadro muy grave causado por una infección bacteriana de la piel y de tejidos blandos subcutáneos, cuya evolución es hacia la destrucción y necrosis de los tejidos en un corto espacio de tiempo; el lupus eritematoso sistémico es una enfermedad autoinmune de causa desconocida que quienes la padecen tienen una mayor probabilidad de contraer infecciones debido al mal funcionamiento del sistema inmunológico y/o los efectos secundarios causados por los medicamentos. Objetivo: Observar la importancia de un tratamiento rápido y eficaz de la fascitis necrotizante en un paciente con lupus eritematoso sistémico y esteatohepatitis no alcohólica. Presentación de caso: Se presentó el caso clínico de un paciente de 30 años con diagnóstico de lupus eritematoso sistémico que desarrolló de forma concomitante de fascitis necrotizante y esteatohepatitis no alcohólica. A pesar de un tratamiento adecuado, el paciente fue agresivo. Tuvo una estadía hospitalaria de 83 días, con una evolución desfavorable que conllevó a la muerte(AU)

Introduction: Necrotizing fasciitis is a very serious condition caused by a bacterial infection of the skin and subcutaneous soft tissues, whose evolution is towards the destruction and necrosis of the tissues in a short space of time; Systemic lupus erythematosus is an autoimmune disease of unknown cause that sufferers are more likely to contract infections due to poor immune system function and/or side effects caused by medications. Objective: To observe the importance of rapid and effective treatment of necrotizing fasciitis in a patient with systemic lupus erythematosus and non-alcoholic steatohepatitis. Case report: We report the clinical case of a 30-year-old patient diagnosed with systemic lupus erythematosus who concomitantly developed necrotizing fasciitis and nonalcoholic steatohepatitis. Despite adequate treatment, the patient was aggressive. The patient had a hospital stay of 83 days, with an unfavorable evolution that led to his death(AU)

Frecuencia de eventos cardiovasculares en pacientes con lupus eritematoso sistémico / Frequency of cardiovascular events in patients with systemic lupus erythematosus

Resumen Introducción: el progreso en los tratamientos para el lupus eritematoso sistémico (LES) resultó en una disminución de la mortalidad; sin embargo, la enfermedad cardiovascular y las complicaciones infecciosas aún son las principales causas de muerte. La evidencia apoya la participación del sistema inmunológico en la generación de la placa aterosclerótica, así como su conexión con las enfermedades autoinmunes. Objetivos: describir la frecuencia de eventos cardiovasculares (ECV) en el Registro de Lupus Eritematoso Sistémico de la Sociedad Argentina de Reumatología (RELESSAR) transversal, así como sus principales factores de riesgo asociados. Materiales y métodos: estudio descriptivo y transversal para el cual se tomaron los pacientes ingresados en el registro RELESSAR transversal. Se describieron las variables sociodemográficas y clínicas, las comorbilidades, score de actividad y daño. ECV se definió como la presencia de al menos una de las siguientes patologías: enfermedad arterial periférica, cardiopatía isquémica o accidente cerebrovascular. El evento clasificado para el análisis fue aquel posterior al diagnóstico del LES. Se conformaron dos grupos macheados por edad y sexo 1:2. Resultados: 1515 pacientes mayores de 18 años participaron del registro. Se describieron 80 pacientes con ECV (5,3%). En este análisis se incluyeron 240 pacientes conformando dos grupos. La edad media fue de 47,8 (14,4) y 47,6 (14,2) en el grupo con y sin ECV respectivamente. Los pacientes con ECV tuvieron mayor duración del LES en meses, mayor índice de Charlson, mayor SLICC (Systemic Lupus International Collaborating Clinics/American College of Rheumatology), mayor frecuencia de manifestaciones neurológicas, síndrome antifosfolípido, hospitalizaciones y uso de ciclofosfamida. Las únicas variables asociadas en el análisis multivariado fueron el índice de Charlson (p=0,004) y el SLICC (p<0,001). Conclusiones: los ECV influyen significativamente en nuestros pacientes, y se asocian a mayor posibilidad de daño irreversible y comorbilidades.

Abstract Introduction: progress in treatments for systemic lupus erythematosus (SLE) has resulted in a decrease in mortality; however, cardiovascular and infectious diseases remain the leading causes of death. Evidence supports the involvement of the immune system in the generation of atherosclerotic plaque, as well as its connection to autoimmune diseases. Objectives: to describe the frequency of cardiovascular disease (CVD) in the cross-sectional RELESSAR registry, as well as its associated variables. Materials and methods: a descriptive and cross-sectional study was performed using patients admitted to the cross-sectional RELESSAR registry. Sociodemographic variables, clinical variables, comorbidities, activity and damage scores were described. CVD was defined as at least one of the following: peripheral arterial disease, ischemic heart disease, or cerebrovascular accident. All patients with at least one CVD were included in our analysis (heart attack, central nervous system vascular disease, and peripheral arteries atherosclerotic disease). The event classified for the analysis was that after the diagnosis of SLE. SLE diagnosis was previous to CVD. Two groups matched by age and sex, 1:2 were formed. Results: a total of 1515 patients older than 18 years participated in the registry. Eighty patients with CVD (5.3%) were described in the registry. Two-hundred and forty patients were included, according to two groups. The mean age was 47.8 (SD 14.4) and 47.6 (SD 14.2) in patients with and without CVD, respectively. Patients with CVD had a longer duration of SLE in months, a higher Charlson index, a higher SLICC, increased frequency of neurological manifestations, antiphospholipid syndrome, hospitalizations, and use of cyclophosphamide. The associated variables in the multivariate were the Charlson Index (p=0.004) and the SLICC (p<0.001). Conclusions: CVDs have a significant influence on our patients, being associated with a greater possibility of damage and comorbidities.

Role of HLA alleles polymorphism in systemic lupus erythematosus: A prospective study from North India

Background: Systemic lupus erythematosus (SLE) is a chronic autoimmune disorder and has complex etiopathogenesis. The most appropriate hypothesis states that genetic susceptibility in the presence of environmental risk factors predisposes to SLE. HLA class II alleles are critical to immune response and are highly polymorphic. Various alleles in HLA-DR and -DQ regions were analyzed in SLE patients and healthy controls to see their role in susceptibility or protection to SLE. Materials and Methods: This was a prospective observational study, in which a total of 100 SLE patients and 100 controls were analyzed. HLA typing was done by polymerase chain reaction (PCR)-sequence-specific oligonucleotide (SSO) method (SSO probe). Results: DR?1*0301 was significantly increased in SLE patients when compared to controls and had the highest odds ratio. Other risk factor alleles found to be increased were DR?1*0701, DQ?1*0202, and DQ?1*0301, which had a significant positive association with SLE, suggesting their role in susceptibility to SLE. In contrast, DR?1*0401, DR?1*1401, DR?1*1404, DR?1*1501, DQ?1*0501, and DQ?1*0201 showed statistically significant reduction in SLE patients, while these were much more common in controls, suggesting their protective role. Conclusion: This study is only the second study in patients from North India and it determines the role of DR?1*0301, DR?1*0701, DQ?1*0202, and DQ?1*0301 alleles as risk factors in SLE patients.

Alopecia androgénica en una paciente con lupus eritematoso sistémico

El cabello no solo es una cuestión de imagen personal, a continuación, se expone un caso clínico de una paciente con alopecia androgénica y lupus eritematoso sistémico (LES) de larga data con resultados subóptimos luego de algunos tratamientos realizados con anterioridad, el manejo de la alopecia es un desafío médico pues no solo se trata de un padecimiento estético si no de posibles factores de riesgo para patologías psiquiátricas, por lo que es importante el diagnóstico y tratamiento oportunos. Existen numerosos tratamientos para la alopecia androgénica desde tratamientos tópicos, vía oral, mesoterapia, entre otros. En este caso en particular se presenta la respuesta satisfactoria tras el inicio de minoxidil vía oral y tópica.

Hair is not only a matter of personal image, below is a clinical case of a patient with long-standing androgenic alopecia and lupus with suboptimal results after some previous treatments, the management of alopecia is a medical challenge because it is not only an aesthetic condition but also possible risk factors for psychiatric pathologies, timely diagnosis and treatment is important. There are numerous treatments for androgenic alopecia from topical treatments, oral route and mesotherapy among others. In this particular case, a satisfactory response is presented after starting oral and topical minoxidil.

Vasculitis pauciinmune asociada a lupus eritematoso sistémico: a propósito de un caso

Introducción: la poliangeítis microscópica (PAM) hace parte del grupo de vasculitis asociadas a anticuerpos anticitoplasmáticos de neutrófilos (ANCA), cuya presencia de anticuerpos contra mieloperoxidasa (MPO) se observa en la mayoría de los casos (70-95 %), asimismo, el compromiso renal presagia mayores tasas de morbilidad y mortalidad, sin embargo, la coexistencia con lupus eritematoso sistémico (LES) es poco frecuente y con mayor énfasis en la variante de LES del adulto mayor o de inicio tardío. Objetivo: dar a conocer un caso poco común de dos enfermedades autoinmunes, lo cual ha sido reportado como síndrome de superposición y brindar información útil que permita ampliar los diagnósticos diferenciales. Presentación del caso: se presenta el caso de un paciente masculino de 76 años con historia de poliartralgias progresivas en deterioro del estado general, con debut en síndrome confusional agudo, caída de filtrado glomerular, microhematuria y proteinuria casi nefrótica. A la evaluación inicial se encontró anemia y trombocitopenia severa, en perfil inmune ANA 1/320 y complemento consumido, cumpliendo criterios para LES, ANCA reactivo específicamente MPO ANCA (1/320) y sospecha de glomerulonefritis con patrón rápidamente progresivo (GNRP). Dado el contexto clínico, se decidió comenzar con pulsos de metilprednisolona consecutivos, seguidos de prednisolona oral y, como terapia de mantenimiento, se instauró ciclofosfamida. Finalmente, con una biopsia renal se confirmó el diagnóstico de vasculitis sistémica pauciinmune con formación de crescencia celular. La evolución clínica del paciente fue satisfactoria, logrando la estabilización de órganos y la normalización de la función renal, hematopoyética y el estado neurológico. Discusión y conclusión: dado que la presentación clínica de LES es heterogénea, se ha reportado su asociación con vasculitis, las cuales comparten un compromiso común de órganos como articulaciones, piel y riñones. Los hallazgos paraclínicos, como por biopsia, fueron consistentes tanto con PAM como con LES, por lo tanto, se diagnosticó como un caso de superposición. Este caso demuestra el enigma del diagnóstico y la complejidad en el manejo de entidades poco frecuentes, de etiologías inmunológicas superpuestas que ponen en peligro la vida de quien la(s) padece, de no tener un diagnóstico oportuno y un tratamiento temprano.

Introduction: Microscopic polyangiitis (MPA) is part of the group of vasculitis associated with antineutrophil cytoplasmic antibodies (ANCA), whose presence of antibodies against myeloperoxidase (MPO) is observed in most cases (70-95 %), likewise, the renal compromise portends higher rates of morbidity and mortality. However, coexistence with Systemic Lupus Erythematosus (SLE) is infrequent, and with greater emphasis on the older adult or late-onset variant of SLE (1). Purpose: Present a rare case of two autoimmune diseases, which has been reported as overlapping syndrome and provide useful information that allows expanding differential diagnoses. Case report: We present the case of a 76-year-old male patient with a history of progressive polyarthralgias in deteriorating general condition, with debut in acute confusional syndrome, drop in glomerular filtration rate, microhematuria and almost nephrotic proteinuria. At the initial evaluation anemia and severe thrombocytopenia were found, in the immune profile ANA 1/320, complement consumed; fulfilling criteria for SLE, ANCAs reactive specifically MPO ANCA (1/320) and suspected Rapidly Progressive Renal Insufficiency (PRRI). Given the clinical context, it was decided to start consecutive pulses of methylprednisolone followed by oral prednisolone, and cyclophosphamide was established as maintenance therapy. Finally, a renal biopsy confirmed the diagnosis of pauci-immune systemic vasculitis with cell crescent formation. These findings were consistent with both SLE and PAM, thus it was diagnosed as a case of overlap. The clinical evolution of the patient was satisfactory, achieving organ stabilization and normalization of renal function, hematopoietic function, and neurological status. Discussion and conclusion: RPGN associated with ANCA present non-nephrotic proteinuria, glomerular filtration rate drop. The most common is anti-glomerular basement membrane (AMBG) antibodies. Serological detection of antineutrophil cytoplasmic antibodies, PR3 and MPO should be available. Microscopic polyangiitis could be determined by renal biopsy, with the presence of cell growth, absence of immune deposits and MPO ANCA positivity. This case is a diagnostic enigma, and the complexity in the management of rare entities, of immunological etiology, superimposed that endanger the life of those who suffer from it, if they do not have a timely diagnosis and early treatment.

Alopecia androgénica en una paciente con lupus eritematoso sistémico / Androgenic alopecia in a patient with systemic lupus erythematosus

El cabello no solo es una cuestión de imagen personal, a continuación, se expone un caso clínico de una paciente con alopecia androgénica y lupus eritematoso sistémico (LES) de larga data con resultados subóptimos luego de algunos tratamientos realizados con anterioridad, el manejo de la alopecia es un desafío médico pues no solo se trata de un padecimiento estético si no de posibles factores de riesgo para patologías psiquiátricas, por lo que es importante el diagnóstico y tratamiento oportunos. Existen numerosos tratamientos para la alopecia androgénica desde tratamientos tópicos, vía oral, mesoterapia, entre otros. En este caso en particular se presenta la respuesta satisfactoria tras el inicio de minoxidil vía oral y tópica.

Hair is not only a matter of personal image, below is a clinical case of a patient with long-standing androgenic alopecia and lupus with suboptimal results after some previous treatments, the management of alopecia is a medical challenge because it is not only an aesthetic condition but also possible risk factors for psychiatric pathologies, timely diagnosis and treatment is important. There are numerous treatments for androgenic alopecia from topical treatments, oral route and mesotherapy among others. In this particular case, a satisfactory response is presented after starting oral and topical minoxidil.

Acute pancreatitis caused by Systemic Lupus Erythematosus activity: A case report and literature review / Pancreatitis aguda por actividad del lupus eritematoso sistémico: Reporte de caso y revisión de la literatura

A 32-year-old woman with systemic lupus erythematosus came to the rheumatology outpatient clinic reporting abdominal pain for a week, along with fever, arthralgias, myalgias, alopecia, asthenia and dyspnea on exertion over the last two months. She was hypotensive and tachycardic, requiring admission to the intensive care unit. She was diagnosed with lupus-related acute pancreatitis, an unusual complication occurring in less than 1% of cases. Most cases are mild and self-limited; however, severe and life-threatening events with multiple organ failure are possible. This article is a case report of lupus-related critical acute pancreatitis, and a literature review.

Mujer de 32 años con lupus eritematoso sistémico acude a consulta externa de reumatología por dolor abdominal de una semana de evolución, además de fiebre, artralgias, mialgias, alopecia, astenia y disnea de esfuerzo de 2 meses de evolución. También presentó hipotensión y taquicardia, por lo que requirió ingreso en la unidad de cuidados intensivos. Le diagnosticaron pancreatitis aguda relacionada con el lupus, que es una complicación inusual que ocurre en menos del 1% de los pacientes. La mayoría de los casos son leves y autolimitados, sin embargo, es posible que se presenten eventos graves y potencialmente mortales, con disfunción multiorgánica. Este artículo es un reporte de caso de una pancreatitis aguda crítica relacionada con lupus y una revisión de la literatura.

Caso clínico de enfermería: modelo de adaptación en adolescente con síndrome de Felty y Rhupus / Nursing Clinical Case: Model of Adaptation in Adolescent with Felty and Rhupus Syndrome / Relato de caso de enfermagem: modelo de adaptação em adolescente com síndrome de Felty e Rhupus

Introducción. El síndrome de Rhupus es la superposición de dos enfermedades autoinmunes, la artritis reumatoide o artritis idiopática juvenil y el lupus eritematoso sistémico, la prevalencia es de 7-401 por 100,000 niños. El síndrome de Felty se caracteriza por la tríada de artritis idiopática juvenil, esplenomegalia y neutropenia; padecer más de una patología reumática es un extraño fenómeno estimado entre el 0.01-2%. Objetivo. Describir el proceso de atención de enfermería integral en una adolescente con Rhupus y síndrome de Felty, bajo el modelo de adaptación de Callista Roy. Metodología. Caso clínico de enfermería en una paciente de 15 años seleccionada en hospitalización pediátrica, con previo consentimiento informado; intervenida a través del proceso de atención de enfermería estructurado según la taxonomía de la North American Nursing Diagnosis Association, la Clasificación de Resultados de Enfermería, y la Clasificación de Intervenciones de Enfermería, con intervenciones intrahospitalarias y seguimiento con tele-enfermería. Resultados. Mejoría de la ambulación y afrontamiento de problemas evidenciados por el aumento del bienestar de la paciente y la familia. Conclusiones. Ante una enfermedad desconocida, el proceso de atención de enfermería, con intervenciones directas y acompañamiento continuo, permite realizar una atención integral, a fin de lograr la adaptación de la paciente y su familia. Palabras clave: Adaptación Psicológica; Atención de Enfermería; Enfermería; Síndrome de Felty; Lupus Eritematoso Sistémico; Resiliencia Psicológica.

Introduction. Rhupus syndrome is the overlap of two autoimmune diseases, rheumatoid arthritis or juvenile idiopathic arthritis, and systemic lupus erythematosus, with a prevalence of 7-401 per 100,000 children. Felty's syndrome is characterized by the triad of juvenile idiopathic arthritis, splenomegaly, and neutropenia; experiencing more than one rheumatic pathology is a rare phenomenon estimated between 0.01-2%. Objective. Describe the comprehensive nursing care process in an adolescent with Rhupus and Felty's syndrome, under the adaptation model of Callista Roy. Methodology. Nursing case study of a 15-year-old patient selected in pediatric hospitalization, with prior informed consent; intervened through the structured nursing care process according to the taxonomy of the North American Nursing Diagnosis Association, the Nursing Outcomes Classification, and the Nursing Interventions Classification, with in-hospital interventions and follow-up through tele-nursing. Results. Improvement in ambulation and coping with problems evidenced by the increased well-being of the patient and the family. Conclusions. Faced with an unknown disease, the nursing care process, with direct interventions and continuous support, allows for comprehensive care to achieve the adaptation of the patient and her family. Keywords: Adaptation, Psychological; Nursing Care; Nursing; Felty Syndrome; Lupus Erythematosus, Systemic; Resilience, Psychological.

Introdução. A síndrome de Rhupus é a sobreposição de duas doenças autoimunes, artrite reumatoide ou artrite idiopática juvenil e lúpus eritematoso sistêmico, a prevalência é de 7-401 por 100,000 crianças. A síndrome de Felty é caracterizada pela tríade de artrite idiopática juvenil, esplenomegalia e neutropenia; sofrer de mais de uma patologia reumática é um fenômeno estranho estimado entre 0.01-2%. Objetivo. Descrever o processo de assistência integral de enfermagem em uma adolescente com Rhupus e síndrome de Felty, sob o modelo de adaptação de Callista Roy. Metodologia. Caso clínico de enfermagem em uma paciente de 15 anos selecionada em internação pediátrica, com prévio consentimento informado; ela teve intervenção por meio do processo de cuidado de enfermagem estruturado segundo a taxonomia da North American Nursing Diagnosis Association, a Classificação dos Resultados de Enfermagem e a Classificação das Intervenções de Enfermagem, com intervenções intra-hospitalares e acompanhamento com tele-enfermagem. Resultados. Melhora na deambulação e enfrentamento de problemas evidenciados pelo aumento do bem-estar do paciente e da família. Conclusões. Diante de uma doença desconhecida, o processo de assistência de enfermagem, com intervenções diretas e acompanhamento contínuo, permite um cuidado integral, de forma a alcançar a adaptação do paciente e de sua família. Palavras-chave: Adaptação Psicológica; Cuidados de Enfermagem; Enfermagem; Síndrome de Felty; Lúpus Eritematoso Sistêmico; Resiliência Psicológica.