Clinical characteristics and diagnostic indicators of macrophage activation syndrome in patients with systemic lupus erythematosus and adult-onset Still's disease / 北京大学学报(医学版)

OBJECTIVE@#To analyze and compare the clinical and laboratory characteristics of macrophage activation syndrome (MAS) in patients with systemic lupus erythematosus (SLE) and adult-onset Still's disease (AOSD), and to evaluate the applicability of the 2016 European League Against Rheumatism/American College of Rheumatology/Paediatric Rheumatology International Trials Organization classification criteria for MAS complicating systemic juvenile idiopathic arthritis (sJIA) in different auto-immune diseases contexts and to propose new diagnostic predictive indicators.@*METHODS@#A retrospective analysis was conducted on the clinical and laboratory data of 24 SLE patients with MAS (SLE-MAS) and 24 AOSD patients with MAS (AOSD-MAS) who were hospitalized at Peking University People's Hospital between 2000 and 2018. Age- and sex-matched SLE (50 patients) and AOSD (50 patients) diagnosed in the same period without MAS episodes were selected as controls. The cutoff values for laboratory indicators predicting SLE-MAS and AOSD-MAS were determined using receiver operating characteristic (ROC) curves. Furthermore, the laboratory diagnostic predictive values for AOSD-MAS were used to improve the classification criteria for systemic juvenile idiopathic arthritis-associated MAS (sJIA-MAS), and the applicability of the revised criteria for AOSD-MAS was explored.@*RESULTS@#Approximately 60% of SLE-MAS and 40% of AOSD-MAS occurred within three months after the diagnosis of the underlying diseases. The most frequent clinical feature was fever. In addition to the indicators mentioned in the diagnosis criteria for hemophagocytic syndrome revised by the International Society for Stem Cell Research, the MAS patients also exhibited significantly elevated levels of aspartate aminotransferase and lactate dehydrogenase, along with a significant decrease in albumin. Hemophagocytosis was observed in only about half of the MAS patients. ROC curve analysis demonstrated that the optimal discriminative values for diagnosing MAS was achieved when SLE patients had ferritin level≥1 010 μg/L and lactate dehydroge-nase levels≥359 U/L, while AOSD patients had fibrinogen levels≤225.5 mg/dL and triglyceride levels≥2.0 mmol/L. Applying the 2016 sJIA-MAS classification criteria to AOSD-MAS yielded a diagnostic sensitivity of 100% and specificity of 62%. By replacing the less specific markers ferritin and fibrinogen in the 2016 sJIA-MAS classification criteria with new cutoff values, the revised criteria for classifying AOSD-MAS had a notable increased specificity of 86%.@*CONCLUSION@#Secondary MAS commonly occurs in the early stages following the diagnosis of SLE and AOSD. There are notable variations in laboratory indicators among different underlying diseases, which may lead to misdiagnosis or missed diagnosis when using uniform classification criteria for MAS. The 2016 sJIA-MAS classification criteria exhibit high sensitivity but low specificity in diagnosing AOSD-MAS. Modification of the criteria can enhance its specificity.

Increased human neutrophil lipocalin and its clinical relevance in adult-onset Still's disease / 中华医学杂志(英文版)

BACKGROUND@#Human neutrophil lipocalin (HNL) has been used extensively to differentiate acute bacterial infection from febrile diseases as a biomarker to reflect the activation of the neutrophil. The serum HNL levels in the adult-onset Still's disease (AOSD) patients with and without infection, as well as the healthy controls (HCs), were analyzed statistically in this study to evaluate the value of HNL for the diagnosis of AOSD.@*METHODS@#A total of 129 AOSD patients were enrolled, from whom blood samples were drawn and the AOSD diagnosis was confirmed through the review of the medical records, where the systemic score, demographic characteristics, clinical manifestations, and laboratory parameters were also collected for the patients; in addition, a total of 40 HCs were recruited among the blood donors from the healthcare center with the relevant information collected. The HNL test was done for the blood samples with the enzyme-linked immunosorbent assay and the analyses were done for the correlations of HNL with clinical manifestations and diagnostic effectiveness.@*RESULTS@#The serum HNL increased significantly in the patients with only AOSD as compared with that in the HCs (139.76 ± 8.99 ng/mL vs . 55.92 ± 6.12 ng/mL; P  < 0.001). The serum HNL level was correlated with the white blood cell (WBC) count ( r  = 0.335, P  < 0.001), neutrophil count ( r  = 0.334, P  < 0.001), erythrocyte sedimentation rate ( r  = 0.241, P  = 0.022), C-reactive protein ( r  = 0.442, P  < 0.0001), and systemic score ( r  = 0.343, P  < 0.0001) in the AOSD patients significantly. Patients with fever, leukocytosis ≥15,000/mm 3 , and myalgia in the HNL-positive group were observed relatively more than those in the HNL-negative group ( P  = 0.009, P  = 0.023, and P  = 0.007, respectively). HNL was a more sensitive indicator than ferritin and C-reactive protein (CRP) to differentiate the AOSD patients with bacterial infection from AOSD-only patients, and the Youden index was 0.6 for HNL and 0.29 for CRP.@*CONCLUSION@#Serum HNL can be used as a biomarker for the diagnosis of the AOSD, and HNL is also observed to be associated with the disease activity.

Role of mean platelet volume in differential diagnosis of adult-onset Still's disease and sepsis

SUMMARY OBJECTIVES: Mean platelet volume is a simple biomarker for inflammatory disease. The purpose of this study is to evaluate the role of mean platelet volume in distinguishing adult-onset Still's disease from sepsis. METHODS: We retrospectively selected 68 patients with adult-onset Still's disease and 55 patients with sepsis between January 2015 and December 2019. Related laboratory data were collected and analyzed. RESULTS: There were no significant differences in white blood cell counts, neutrophils, lymphocytes, and C-reactive protein between adult-onset Still's disease group and sepsis group. However, patients in adult-onset Still's disease group showed higher ferritin and platelets and lower mean platelet volume and platelet distribution width than those in sepsis group (p<0.01 for both). Receiver operating characteristic curve analysis was performed to distinguish adult-onset Still's disease and sepsis. The area under the curve of mean platelet volume was 0.761 (95%CI 0.673-0.849), with a sensitivity of 79.1%, a specificity of 63.3%, and a cutoff value of 10.9 fL. In contrast, the area under the curve of combined ferritin and mean platelet volume was 0.90l (95%CI 0.837-0.965), with higher sensitivity (82.8%) and specificity (96.2%). Therefore, mean platelet volume could be used as a supplementary indicator to distinguish adult-onset Still's disease from sepsis. CONCLUSION: We suggest that mean platelet volume could be used as a supplementary biomarker for differential diagnosis of adult-onset Still's disease and sepsis in addition to ferritin.

The role of platelet to mean platelet volume ratio in the identification of adult-onset still's disease from sepsis

OBJECTIVES: Inflammatory factors exert a significant role in the development of adult-onset Still's disease (AOSD) and sepsis. Although platelet counts and platelet parameters have long served as indicators for inflammatory diseases, their role in the differential diagnosis between adult-onset stilĺs disease and sepsis remains unclear. We designed this retrospective study to explore whether the platelet to mean platelet volume (MPV) ratio (PMR) can help to distinguish AOSD from sepsis. METHODS: A total of 110 AOSD patients and 84 sepsis patients were enrolled in the study. Seventy-three AOSD patients and 56 sepsis patients between January 2010 and June 2017 were enrolled in the test cohort to analyze PMR values, which was then validated in the validation cohort (37 AOSD patients and 28 sepsis patients between June 2017 and December 2019). RESULTS: The values of PMR were significantly higher in AOSD patients than in sepsis patients (test cohort, validation cohort, and entire cohort), In the test cohort, logistic regression analysis showed that PMR was an independent risk factor of AOSD (odds ratios [OR]: 9.22, 95% confidence interval [CI] 2.15-39.46, p=0.003). Further receiver operating characteristic curve (ROC) analysis showed that the area under the ROC curve was 0.735 (95% CI 0.631-0.839, p<0.001) for PMR alone and 0.925 (95% CI 0.869-0.980, p<0.001) for the combination of PMR and serum ferritin. Consistently, the validation cohort exhibited analogous results. CONCLUSIONS: PMR could be used as a single indicator or a complementary indicator to distinguish AOSD from sepsis.

Doença de Still do adulto: um desafio diagnóstico / Adult-onset Still's disease: a diagnostic challenge

A doença de Still do adulto é uma rara condição inflamatória, cujo diagnóstico é um desafio, por se tratar de diagnóstico de exclusão, após vasta investigação. Manifesta-se com febre alta diária, amigdalite não supurativa, artrite, rash evanescente, leucocitose e hiperferritinemia. O presente caso demonstra a doença de Still do adulto e sua vasta investigação, motivando a realização de revisão bibliográfica sobre inovações na fisiopatologia, no diagnóstico e no tratamento.

Adult onset Still's disease is a rare inflammatory condition, the diagnosis of which is a challenge, because it is a diagnosis of exclusion, and demands extensive investigation. It manifests with high daily fever, nonsuppurative tonsillitis, arthritis, evanescent rash, leukocytosis, and hyperferritinemia. The present case de­monstrates adult-onset Still's disease and its extensive inves­tigation, motivating literature review on innovations of its pathophysiology, diagnosis, and treatment.

Afectación articular en un paciente joven con enfermedad de Still del adulto: a propósito de un caso / Joint involvement in a young patient with adult Still's disease: in relation to a case

Introducción: La enfermedad de Still del adulto es una enfermedad reumática, inflamatoria, sistémica y crónica que generalmente comienza sobre los 40 años de edad, pero puede aparecer en edades más tempranas. Se caracteriza por gran variedad de manifestaciones generales, articulares, fiebre y rash principalmente. Etiopatogénicamente, el proceso inflamatorio crónico que se genera durante el curso de la enfermedad es el responsable de las manifestaciones clínicas y del daño articular que se produce. Objetivo: dar a conocer los elementos básicos para el diagnóstico de la enfermedad de Still del adulto. Caso clínico: se presenta el caso de un paciente masculino, de 21 años de edad, que acude a consulta con manifestaciones clínicas que permiten realizar el diagnóstico de una Enfermedad de Still del Adulto. Conclusiones: La enfermedad de Still del Adulto, a pesar de no tener un patrón de deformidades tan amplio como la artritis reumatoide, también produce daño articular que puede comprometer la función de la articulación afectada. Resulta importante identificar precozmente las manifestaciones clínicas que hacen sospechar su diagnóstico para minimizar, retrasar o prevenir la afectación de las articulaciones(AU)

Introduction: adult Still's disease is a rheumatic, inflammatory, systemic and chronic disease that usually begins around 40 years of age, but may appear at younger ages. It is characterized by a great variety of general, joint, fever and rash manifestations. Etiopathogenically, the chronic inflammatory process that is generated during the course of the disease is responsible for the clinical manifestations and joint damage that occurs. Objective: to present the basic elements for the diagnosis of adult Still's disease. Clinical case: the case of a male patient, 21 years of age, who comes to the clinic with clinical manifestations that allow the diagnosis of an Adult Still's Disease. Conclusions: adult Still's disease, despite not having a pattern of deformities as extensive as rheumatoid arthritis, also produces joint damage that can compromise the function of the affected joint. It is important to identify early clinical manifestations that make your diagnosis suspect to minimize, delay or prevent the involvement of the joints(AU)

Oral mucosa lesions as atypical manifestation of adult-onset Still´s disease

Abstract: Adult-onset Still's disease is a systemic inflammatory disorder of unknown etiology, characterized by skin rash, spiking fever, arthralgias or arthritis, and leukocytosis. The typical skin rash is evanescent, salmon-pink, nonpruritic and maculopapular, predominantly on the extremities. It is considered one of the major Yamaguchi's criteria in adult-onset Still's disease. However, atypical skin lesions are also described. Here, a 61-year-old woman with sore throat, spiking fever, polyarthritis and evanescent salmon-pink nonpruritic maculopapular skin rash on the extremities was diagnosed with adult-onset Still's disease. In addition, atypical brown macules on oral mucosa, localized on the inner lips and tongue were also observed. Biopsy revealed a neutrophilic infiltrate. Despite treatment and improvement of the adult-onset Still's disease, the atypical oral mucosal lesions persisted.

Enfermedad de still en el adulto: relato de caso y revisión literaria / Still's disease in adults: case report and literary review

La enfermedad de Still se caracteriza por ser una enfermedad inflamatoria, sis-témica, rara, sin etiología y patogénesis establecidas. Su tríada clásica de manifesta-ción es fiebre alta de origen desconocido, artralgia persistente y erupción cutánea de color salmón, además de manifestaciones sistémicas. El diagnóstico es clínico y requiere la exclusión de enfermedades infecciosas, neoplásicas y otras enfermeda-des autoinmunes, pues no posee marcadores biológicos específicos. Los exámenes de laboratorio son inespecíficos y muestran una actividad inmunológica exacerbada. El tratamiento incluye medicamentos antiinflamatorios no esteroideos, corticosteroi-des, inmunosupresores, gammaglobulina intravenosa y agentes biológicos. Describi-mos el caso de un paciente de 25 años con fiebre vespertina diaria sin foco conocido y artralgia migratoria, sin erupciones cutáneas.

Still's disease is characterized by being a rare systemic inflammatory disease with no established etiology and pathogenesis. Its classic manifestation triad is high fever of unknown origin, persistent arthralgia and salmon-colored rash, in addition to systemic manifestations. The diagnosis is clinical and requires the exclusion of infec-tious, neoplastic and other autoimmune diseases, as it does not have specific biologi-cal markers. Laboratory tests are non-specific and show exaggerated immune activity. Treatment includes non-steroidal anti-inflammatory drugs, corticosteroids, immu-nosuppressants, intravenous gammaglobulin and biological agents. We describe the case of a 25-year-old patient with daily afternoon fever with no known focus and migratory arthralgia without rash.

Síndrome ASIA: prótesis mamarias y enfermedad de Still / ASIA syndrome: breast implant and Still's disease

Las enfermedades del tejido conectivo vinculadas a implantes mamarios de siliconas han sido tema de discusión. En la última década, la siliconosis ha sido incluida dentro del síndrome autoinmune/inflamatorio inducido por adyuvante (ASIA) junto al síndrome de la guerra del Golfo, síndrome de miofascitis macrofágica y fenómenos post vacunales. El ASIA puede manifestarse como lupus, artritis reumatoidea, o más raramente como enfermedad de Still del adulto. Presentamos el caso de una paciente con fiebre prolongada y criterios clínicos compatibles con ASIA y enfermedad de Still. Se resecaron las prótesis y la anatomía patológica descartó linfoma anaplásico ALK (-) vinculado a prótesis. Los médicos debemos estar alertas ante la aparición de estas nuevas entidades asociadas a los implantes mamarios de siliconas.

Connective tissue diseases associated with silicone breast implants have been widely discussed. In the last decade, siliconosis has been included in the autoimmune/inflammatory syndrome induced by adjuvants (ASIA) next to Gulf War syndrome, macrophage myofascitis and postvaccination phenomena. The ASIA syndrome may appear as lupus, rheumatoid arthritis, or more rarely, as adult Still's disease. We discuss the case of a patient with prolonged fever and clinical criteria for ASIA and Still's disease. The prostheses were resected and pathology showed absence of breast implant associated anaplastic lymphoma ALK (-). Physicians should be alert to these new entities linked to silicone breast implants.

Enfermedad de Still del adulto, a propósito de un caso: un desafío diagnóstico / Still disease of the adult, about a case: a diagnostic challenge

La enfermedad de Still del adulto (ESA) es una patología inflamatoria sistémica infrecuente, que se caracteriza por la tríada de fiebre, exantema evanescente y artralgias. Mujer de 57 años con historia de 7 meses de lesiones tipo máculas-habones asalmonadas, en relación a peaks febriles intermitentes. Asocia odinofagia, mialgias y artralgias. En los exámenes destaca anemia leve, VHS, PCR y ferritina elevadas. Estudio viral e inmunológico negativo. La tomografía computada de tórax, abdomen y pelvis (TC-TAP) reveló múltiples adenopatías axilares, pélvicas y esplenomegalia. La biopsia cutánea y de un linfonodo axilar, descartó patología vasculítica y neoplásica respectivamente, apoyando el diagnóstico de ESA. Se trató con prednisona 40 mg/día, con buena respuesta y sin recidivas. La ESA constituye un verdadero desafío diagnóstico, pues su clínica inespecífica y la ausencia de marcadores propician un diagnóstico tardío. Su pronóstico se relaciona con un tratamiento precoz, por lo que es fundamental un diagnóstico oportuno.

Adult-onset Still's disease (ESA) is an infrequent systemic inflammatory pathology, characterized by the triad of febrile syndrome, evanescent rash and arthralgias. A 57-year-old woman with a 7-months history of salmon-colored rash in relation to intermitent febrile peaks, asociated with odynophagia, myalgias and arthralgias. General exams showed mild anemia, elevated ESR, CRP and ferritin. Viral and immunological studies were negative. Computed tomography of the chest, abdomen and pelvis revealed multiple axillary and pelvic lymph nodes and splenomegaly. The skin and axillary lymph node biopsies, ruled out vasculitic and neoplastic pathologies respectively, supporting the diagnosis of ESA. Prednisone 40 mg daily was administered with good response and no evidence of recurrence. No evidence of relapse in subsequent controls. ESA reprsents a great diagnostic challenge, since its nonspecific clinic and the absence of pathognomonic studies lead to a late or erroneous diagnosis. Its prognosis is related to an early treatment; therefore it is essential to make en early diagnosis.

Adult Onset Stills Disease: A Case Report.

Adult onset still’s disease (AOSD) is a rare inflammatory disorder of unknown etiology. Because of lack of a defined diagnostic test the diagnosis of AOSD can only be made after excluding infectious, malignant, and autoimmune diseases. A 21-year-old female patient was admitted in our hospital with intermittent high-grade fever, polyarthritis and rash. On further history, examination and laboratory investigation she was found to be fulfilling all the Yamaguchi criteria for AOSD. All other causes consisting of acute or chronic infections, hematological malignancies, and other rheumatic disorders were excluded by laboratory investigations. Patient was treated as AOSD with non-steroidal anti-inflammatory drugs and steroids and is completely free of symptoms.

Delta Neutrophil Index as an Early Marker for Differential Diagnosis of Adult-Onset Still's Disease and Sepsis

PURPOSE: To investigate clinical implications of delta neutrophil index (DNI) to discriminate adult onset Still's disease (AOSD) from sepsis. MATERIALS AND METHODS: We reviewed the medical records of 13 patients with AOSD and 33 gender and age-matched patients with sepsis. In all subjects, microbial tests were performed to exclude or confirm sepsis. All laboratory data were measured two or three times during the first 3 days and represented by their mean levels. DNI was measured automatically by ADVIA 2120 for the first 3 days. RESULTS: There were no significant differences in white blood cell counts, neutrophil proportion, erythrocyte sedimentation rate and C-reactive protein between two groups. AOSD patients had notably lower DNI than sepsis patients regardless of the presence of bacteremia or not. However, both DNI and ferritin were not significant independent factors for predicting sepsis in the multivariate logistic regression analysis. Meanwhile, the area under the receiver operating characteristic curve (AUROC) of DNI was slightly higher than that of ferritin. When we set DNI of 2.75% as the cut-off value for predicting sepsis, 11 (84.6%) of AOSD patients had a DNI value below 2.75% and 2 (15.4%) of them had a DNI over 2.75% (relative risk for sepsis 176). CONCLUSION: We suggest that DNI may be a useful marker for differential diagnosis of AOSD from sepsis in the early phase as supplementary to ferritin.

Revisando doença de Still do adulto / Reviewing adult-onset Still's disease

A doença de Still do adulto (DSA) é uma doença inflamatória sistêmica rara, de etiologia desconhecida, caracterizada por febre associada a rash maculoso cor de salmão durante os picos febris, podendo apresentar odinofagia, artralgia/artrite, além de hepatoesplenomegalia e linfadenopatia. Laboratorialmente não possui nenhum exame que seja específico desta enfermidade, apresentando elevação dos níveis de PCR, VHS e ferritina, além de leucocitose e elevação de enzimas de dano hepático. Trata-se de um diagnóstico de exclusão, e deve ser lembrado nos casos de febre de origem indeterminada (FOI), propiciando um diagnóstico precoce e evitando gastos desnecessários com exames subsidiários e internações hospitalares prolongadas.

Adult-onset Still's disease is a rare inflammatory systemic condition of unknown aetiology. The main clinical feature is fever accompained by salmon-pink maculous rash that appears along with fever spikes. Other symptoms are odynophagia, arthralgia or arthritis, enlargement of the spleen and liver, and lymphadenopathy. Although there are no specific laboratorial tests to confirm the condition, C-reactive protein, VHS, ferritin, white blood cells count and liver enzymes are usually elevated. Diagnosis is established by exclusion and must be considered in patients presenting with fever of obscure origin. Early identification reduces costs by avoiding unnecessary tests and long hospital admission.

Enfermedad de Still del adulto: una gran simuladora: experiencia clínica basada en 20 casos / Adult Still's disease: retrospective review of 20 patients

Background: Adult Still's disease is an inflammatory disorder characterized by quotidian fevers, and an evanescent rash. Its presentation can be acute or subacute. Aim: To report our experience with Adult Still's disease. Material and methods: Systematic retrospective review of medical records of nine men and 11 women aged between 17 and 57 years, with Still's disease, followed in two public hospitals of Metropolitan Santiago. Results: Eighty percent of patients had a prior different diagnosis. All presented with fever and joint involvement. Eighty percent had malaise, 80 percent had odynophagia, 80 percent had an evanescent rash, 70 percent had myalgias, 50 percent had lymph node enlargement and 40 percent had splenomegaly. Laboratory showed ¡eukocytosis in 80 percent and a high erythrocyte sedimentation rate in all. High ferritin levels were detected in 80 percent, and became an important diagnosis clue. Initial treatment was based on non steroidal antiinflammatory drugs, however 80 percent required steroids and 35 percent required methotrexate. Azathioprine, sulphalazine, hydroxychloroquine and ¡eflunomide were used occasionally. Eleven patients had a single episode, nine had a relapsing disease and four had a chronic or persistent mode. Conclusions: Adult Still's disease must be suspected in patients with fever of unknown origin. An early diagnosis and adequate treatment of the disease are associated with a favorable evolution and prognosis.

Adult - onset Still's disease : A review.

Objective: This article is an attempt to review recent literature regarding pathogenesis and clinical and laboratory findings in adult-onset Still's disease (AOSD). Materials and methods: A search was conducted in PubMed and Ovid for English language publications, using individual or linked search terms "adult-onset Still's disease," "adult Still's disease," "Still's disease," "AOSD," and other related terms, from 1996 to 2009, and the clinically relevant articles were subsequently selected. Results: More than 1000 titles were reviewed by the authors, and the most important concepts were selected from 143 full-text articles. Conclusion: Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disorder of unknown etiology and pathogenesis, usually presenting with high spiking fever accompanied by systemic manifestations. The disease is an entity with heterogeneous pathology; and diverse suggested etiologies, clinical manifestations and prognoses. There is no single diagnostic test for AOSD; rather, the diagnosis is based on a set of criteria, the most important of which are indeed clinical, but they also include paraclinical ones. Treatment aims at both minimizing inflammation and halting disease progression. For the former, nonsteroidal anti-inflammatory drugs have limited efficacy; so glucocorticoids in conjunction with disease-modifying antirheumatic drugs are also used. Novel therapeutic approaches such as anti-tumor necrosis factor blockade and monoclonal antibodies are promising.

Valor de la ferritina sérica para el diagnóstico de la enfermedad de Still del adulto / Value of serum ferritin for the diagnosis of adult Still's disease

La enfermedad de Still del adulto es una enfermedad inflamatoria sistémica de etología desconocida, de rara presentación; se caracteriza por fiebre alta, artritis, leucocitosis neutrofílica y exantema evanescente. Los valores de ferritina sérica por encima de 3 000 ng/mL son de gran valor para establecer el diagnóstico. Se reportó un paciente de 25 años de edad con enfermedad de Still del adulto quien presentó fiebre prolongada, rash dérmico, odinofagia y artritis. Se realizaron exámenes complementarios mediante los que se demostró la existencia de leucocitosis, hipertransaminemia, ferritina sérica muy elevada, anticuerpos antinucleares y factor reumatoides negativos. Se administró indometacina con la cual se eliminaron los síntomas. Se concluyó que los niveles de ferritina sérica elevados son de valor para el diagnóstico de enfermedad de Still del adulto activa y su disminución se correlaciona con la evolución favorable del cuadro clínico.

Adult Still's disease is a rare systemic inflammatory disease of unknown etiology. It is characterized by high fever, arthritis, neutrophilic leukocytosis and evanescent exanthema. The values of serum ferritin over 3 000 ng/mL are very useful to establish the diagnosis. A 25-year-old patient with adult Still's disease that presented prolonged fever, rash, odinophagia and arthritis was presented. The existence of leukocytosis, hypertransaminemia, very elevated serum ferritin, antinuclear antibodies and negative rheumatoid factors was confirmed by complementary tests. The symptoms disappeared on administering indomethacin. It was concluded that the high levels of serum ferritin have a great value to diagnose active adult Still's disease and that its reduction is correlated with the favorable evolution of the clinical picture.

Celiac disease: association with adult-onset Still's disease: apropos of a clinical case.

Adult-onset Still's disease (AOSD) is a rheumatic disorder of unknown etiology characterized by a triad of fever, polyarthritis and evanescent rash. We present a case report of a 28-year-old female who presented with complaints of fever, joint pains, rash, weakness for the past 4 years and diarrhea for the past 2 years. On investigation the patient was diagnosed to be a case of AOSD. Duodenal biopsy report was suggestive of celiac disease with a positive IgA tissue transglutaminase and anti-endomysial antibody. The patient was started on weekly methotrexate and gluten-free diet and her symptoms gradually improved. The patient remains in our follow-up and is doing well.

Enfermedad de Still del adulto / Still disease of adult

La enfermedad de Still del adulto es una afección de baja frecuencia, caracterizada clínicamente por una poliartritis febril acompañada de una erupción cutánea evanescente no pruriginosa. La sospecha clínica se confirma con la punción articular; el examen del liquido sinovial y el estudio histológico de la biopsia sinovial. El tratamiento consiste en la administración de antiinflamatorios, corticoides y/o metotrexato.

Hyperferritinemia and Pyrexia of Unknown Origin

By the time a patient is formally categorized as having 'Pyrexia of Unknown Origin', a battery of tests have been performed and many physicians have run out of differential diagnoses. Adult Still's Disease is considered a rheumatologic disorder and is, therefore, not considered if the patient does not have joint symptoms. We present the case of such a patient who presented with high spiking fever, and review how we gained confidence in establishing the diagnosis