Recomendaciones de la Sociedad Argentina de Reumatología en el manejo de la arteritis de células gigantes / Recommendations of the Argentine Society of Rheumatology in the management of giant cell arteritis

La arteritis de células gigantes (ACG) es una vasculitis sistémica que afecta a personas adultas; compromete vasos arteriales de mediano y gran calibre, con potenciales complicaciones de gravedad, como la ceguera, y es considerada una emergencia médica. El objetivo de estas guías fue desarrollar las primeras recomendaciones argentinas para su tratamiento, basadas en la revisión de la literatura mediante metodología GRADE. Un panel de expertos en vasculitis elaboró las preguntas en formato PICO (población, intervención, comparador y outcomes), y luego un panel de expertos en metodología efectuó la revisión de la bibliografía con la extracción de la evidencia para cada una de las preguntas. Se realizó un focus group de pacientes para conocer sus preferencias y experiencias. Finalmente, con la información recabada, el panel de expertos en vasculitis procedió a la votación de las recomendaciones que a continuación se presentan.

Giant cell arteritis (GCA) is a systemic vasculitis affecting adult patients and involving large and medium vessels. Potential serious complications as blindness may occur and it is considered a medical emergency. The objective of elaborating this guideline was to develop first Argentinian GCA treatment recommendations using GRADE methodology. An expert panel generated clinically meaningful questions addressing aspects of the treatment of GCA in the Population, Intervention, Comparator and Outcome (PICO) format and then a group of methodology experts reviewed and extracted data from literature summarizing available evidence. A patient's focus group discussion took place gathering information on their preferences and experiences. Finally, the vasculitis expert panel, with all the information obtained, voted recommendations here presented.

Guías Argentinas de Vasculitis Suplemento. Tablas arteritis de células gigantes / Argentine Vasculitis Guidelines Supplement. Giant cell arteritis tables

La arteritis de células gigantes (ACG) es una vasculitis sistémica que afecta a personas adultas; compromete vasos arteriales de mediano y gran calibre, con potenciales complicaciones de gravedad, como la ceguera, y es considerada una emergencia médica. El objetivo de estas guías fue desarrollar las primeras recomendaciones argentinas para su tratamiento, basadas en la revisión de la literatura mediante metodología GRADE. Un panel de expertos en vasculitis elaboró las preguntas en formato PICO (población, intervención, comparador y outcomes), y luego un panel de expertos en metodología efectuó la revisión de la bibliografía con la extracción de la evidencia para cada una de las preguntas. Se realizó un focus group de pacientes para conocer sus preferencias y experiencias. Finalmente, con la información recabada, el panel de expertos en vasculitis procedió a la votación de las recomendaciones que a continuación se presentan.

Circulating Galectin-1 and Galectin-3 in Sera From Patients With Systemic Sclerosis: Associations With Clinical Features and Treatment.

Systemic Sclerosis (SSc) is a rheumatic disease characterized by fibrosis, microvascular damage and immune dysregulation. Two major subsets, limited cutaneous systemic sclerosis (lcSSc) and diffuse cutaneous systemic sclerosis (dcSSc) can be defined, according to the extent of skin involvement. Increasing evidence indicates a role for galectins in immune and vascular programs, extracellular matrix remodeling and fibrosis, suggesting their possible involvement in SSc. Here, we determined serum levels of galectin (Gal)-1 and Gal-3 in 83 SSc patients (dcSSc n = 17; lcSSc n = 64; ssSSc n = 2), and evaluated their association with clinical manifestations of the disease. Patients with dcSSc showed lower Gal-3 levels, compared to lcSSc (p = 0.003), whereas no considerable difference in Gal-1 levels was detected between groups. Remarkably, higher concentrations of Gal-1 were associated with the presence of telangiectasias (p = 0.015), and higher concentrations Gal-3 were associated with telangiectasias (p = 0.021), diarrhea (p = 0.039) and constipation (p = 0.038). Moreover, lower Gal-3 levels were associated with the presence of tendinous retractions (p = 0.005). Patients receiving calcium blockers (p = 0.048), methotrexate (p = 0.046) or any immunosuppressive treatment (p = 0.044) presented lower concentrations of Gal-3 compared to those not receiving such treatments. The presence of telangiectasia and the type of SSc maintained their statistical association with Gal-3 (ß 0.25; p = 0.022 and ß 0.26; p = 0.017, respectively) in multiple linear regression models. In conclusion, serum levels of Gal-3 are associated with clinical manifestations of SSc. Among them, the presence of telangiectasias could be explained by the central role of this lectin in the vascularization programs.

Estenosis de las arterias cubital y radial en pacientes con esclerosis sistémica, comparado con un grupo control / Ulnar and radial stenosis in systemic sclerosis

Objetivos: describir la frecuencia de estenosis arterial (cubital y radial) en pa-cientes con esclerosis sistémica (ES); analizar la relación entre estenosis macro-vascular y úlceras digitales. Método: se incluyeron 57 pacientes con ES, según la clasificación del Colegio Americano de Reumatología de 1980 y 21 pacientes sin ES. Se realizó ecografía doppler arterial de miembros superiores. Resultados: la estenosis en al menos una arteria cubital se objetivó en 31% de pacientes con ES (18/57) (p=0.003). Se objetivó estenosis radial en 9 de 57 pacientes con ES (15%) y en uno de los 21 controles (p=0.19). En el modelo multivariado, los predicto-res de úlceras digitales fueron inicio de Raynaud antes de los 40 años (OR 5.3 IC95% 1.54-18.22, p=0.008) y patrón tardío en la capilaroscopia (OR 4.4 IC95% 1.29-15.63, p=0.018). Conclusiones: un tercio de los pacientes ES presentó este-nosis cubital. El compromiso de los grandes vasos no se asoció a úlceras digitales.

Objectives: to describe the frequency of ulnar and radial stenosis in SSc patients. Analyze the correlation between arterial stenosis and digital ulcers. Methods: we included 57 SSc consecutive patients who fulfilled ACR 1980 classification criteria, and 21 healthy controls. An arterial ecodoppler was performed to all participants. Results: the presence of stenosis in at least one ulnar artery was observed in 18 of 57 patients with SSc (31%) and in none of the 21 controls (p=0.003). Stenosis was present in at least one radial artery in 9 of 57 SSc patients (15%) (p=0.19). In multivariate model, the best predictors of digital ulcers were age at onset of Ray-naud phenomenon before 40 years (OR 5.3 95%CI 1.54-18.22, p=0.008) and late SD pattern (OR 4.4 95%CI 1.29-15.63, p=0.018). Conclusion: in the present series, ulnar stenosis was observed frequently in SSc patients. Stenosis of large vessels was not associated with digital ulcers.

Analysis of 65 Renal Biopsies From Patients With Rheumatoid Arthritis (1976-2015): Change in Treatment Strategies Decreased Frequency and Modified Histopathological Findings.

INTRODUCTION: No inherent renal lesions are known in rheumatoid arthritis (RA), but urinary abnormalities and renal dysfunction have been described. OBJECTIVE: First, we describe the histopathological findings of renal biopsies (RBs) in patients with RA and associated clinical manifestations. Second, we evaluated time evolution of RA and the relationship between drugs and renal disease. Last, we investigate whether changes in the management of RA from 1976 to 2015 influenced RBs indication, frequency, and type of histopathological findings. PATIENTS AND METHODS: This is a retrospective and observational study conducted at a university hospital from Argentina. Patients with a diagnosis of RA (ACR, 1987) and RBs between 1976 and 2015 were included. Sixty-five patients met the inclusion criteria. The histopathological findings and associated clinical manifestations were evaluated. Time evolution of RA and the relationship between drugs and renal disease were also determined. To clarify these issues, we characterized 3 groups according to changes in the management of RA: 1976-1989, 1990-2002, and 2003-2015. RESULTS: The most common histopathological finding was renal amyloidosis in 31% (n = 20), followed by mesangial glomerulonephritis in 18% (n = 12), membranous nephropathy in 17% (n = 11), extracapillary proliferative glomerulonephritis in 15% (n = 10), focal segmental glomerular sclerosis in 9% (n = 6), minimal change nephropathy in 5% (n = 3), and tubulointerstitial nephritis in 5% (n = 3). Time evolution of renal amyloidosis was significantly higher than other RBs (15 ± 12 vs 7 ± 6.5 years). Nephrotic syndrome was the most common clinical manifestation (60%) followed by hematuria (46%) with or without proteinuria. Membranous nephropathy was related to the use of gold salts in 45% of cases, and its frequency decreased since 1990. Before 2003, renal amyloidosis was the leading cause of kidney disease, but mesangial glomerulonephritis reached the same frequency between 2003 and 2015. We found that RBs decreased 20% in the second period (1990-2002) and 40% in the last period (2003-2015). Nephrotic syndrome remained the main RB indication during the entire study period. CONCLUSION: This is the first report on RBs findings in patients with RA from Latin America. We found a significant reduction in RBs frequency and modified histological patterns throughout the study period, although RB indication was not modified. Changes in the management of RA might have influenced these findings.

Hypoxanthine-guanine phosphoribosyltransferase deficiency in a patient with a Madrid II mutation.

The hypoxanthine-guanine phosphoribosyltransferase deficiency is an inborn error of purine metabolism, linked to the X chromosome. The clinical phenotypes associated with HPRT deficiency varied according to the level of enzyme deficiency, with a large spectrum of neurologic features like self-injurious behaviour in patients with complete deficiency. We report a 20-year-old man who had asymmetric polyarthritis, tophi, hyperuricemia, nephrolithiasis and mild neurologic symptoms with undetectable levels of HPRT activity in lysed erythrocytes. The genetic study identified the c.143G>A mutation in exon 3, GAA CGT (CTT>GAA CAT CTT (48arg>his). The presence of gouty arthropathy and chronic hyperuricemia in a young patient with neurological symptoms, suggests HPRT deficiency for which it is necessary its enzyme and molecular determination.

Impact of genetic ancestry and sociodemographic status on the clinical expression of systemic lupus erythematosus in American Indian-European populations.

OBJECTIVE: American Indian-Europeans, Asians, and African Americans have an excess morbidity from systemic lupus erythematosus (SLE) and a higher prevalence of lupus nephritis than do Caucasians. The aim of this study was to analyze the relationship between genetic ancestry and sociodemographic characteristics and clinical features in a large cohort of American Indian-European SLE patients. METHODS: A total of 2,116 SLE patients of American Indian-European origin and 4,001 SLE patients of European descent for whom we had clinical data were included in the study. Genotyping of 253 continental ancestry-informative markers was performed on the Illumina platform. Structure and Admixture software were used to determine genetic ancestry proportions of each individual. Logistic regression was used to test the association between genetic ancestry and sociodemographic and clinical characteristics. Odds ratios (ORs) were calculated with 95% confidence intervals (95% CIs). RESULTS: The average American Indian genetic ancestry of 2,116 SLE patients was 40.7%. American Indian genetic ancestry conferred increased risks of renal involvement (P < 0.0001, OR 3.50 [95% CI 2.63- 4.63]) and early age at onset (P < 0.0001). American Indian ancestry protected against photosensitivity (P < 0.0001, OR 0.58 [95% CI 0.44-0.76]), oral ulcers (P < 0.0001, OR 0.55 [95% CI 0.42-0.72]), and serositis (P < 0.0001, OR 0.56 [95% CI 0.41-0.75]) after adjustment for age, sex, and age at onset. However, age and sex had stronger effects than genetic ancestry on malar rash, discoid rash, arthritis, and neurologic involvement. CONCLUSION: In general, American Indian genetic ancestry correlates with lower sociodemographic status and increases the risk of developing renal involvement and SLE at an earlier age.

[History of systemic sclerosis]. / Historia de la esclerosis sistémica.

Systemic sclerosis (Scleroderma) is a generalized disease of the connective tissue, whose etiology is unknown, and which produces fibrosis and degenerative changes in the skin (scleroderma), synovial membrane, arterial vessels, skeletal muscle, and certain internal organs, especially the gastrointestinal tract, lung, heart, and kidney. Patients with cutaneous alterations, which could have been scleroderma cases, have been described since the most distant ancient times, and since the end of XIX century its recognition has been accurate. In the past century more precise diagnoses and treatments were possible because of the knowledge of its pathogenesis and its classification into clinic-serologic subgroups. We intend to recall the names and contributions of the doctors who collaborated with the advances throughout history.

El ecocardiograma en la esclerodermia / Echocardiogram in scleroderma / O ecocardiograma na esclerodermia

Marco teórico. La esclerosis sistémica es una enfermedad crónica caracterizada por afección microvascular y fibrosis tisular. El compromiso cardíaco es frecuente, pero se manifiesta clínicamente en un tercio de los casos y se asocia a peor evolución. El objetivo es describir las alteraciones ecocardiográficas en pacientes enfermos y compararlos con un grupo control. Material y métodos. Se estudiaron 54 pacientes (57 ± 13 años; 93% sexo femenino) y 11 controles (48 ± 20 años; 80% de mujeres). Se les realizaron exámenes ecocardiográficos de acuerdo a recomendaciones de la Sociedad Americana de Ecocardiografía. Resultados. El grupo esclerodermia presentó mayor tamaño del ventrículo derecho: 20,6 ± 4,5 mm vs 15,4 ± 4,3 mm; p=0,02. El análisis Doppler evidenció mayor presión sistólica pulmonar (27,3 ± 5,5 mm Hg vs 20,3 ± 3 mm Hg; p=0,0001) y menor tiempo eyectivo del ventrículo derecho (270 ± 57 mseg vs 333 ± 32 mseg; p=0,001) en el grupo de enfermos. El Doppler tisular reveló mayor tamaño de la onda A' del ventrículo izquierdo (15,3 ± 3,6 cm/ seg vs 12,7 ± 2,6 cm/seg; p=0,01), mayor tamaño de la onda A' del ventrículo derecho (17,6 ± 3,6 cm/seg vs 13,6± 3,4 cm/seg; p=0,001) con menor relación E'/A' (0,9 ± 0,39 vs 1,13 ± 0,18; p=0,01) en los pacientes portadores de colagenopatía. Conclusiones. Los pacientes con esclerosis sistémica presentaron hallazgos que sugieren menor distensibilidad ventricular como consecuencia probable de la mayor presión pulmonar y de la afectación intrínseca del miocardio.

Background. Scleroderma is an infrequent and chronic collagen vascular disease characterized by the vascular engagement of the small arteries and tisular fibrosis. Cardiac manifestations, though common, are present in only one third of the patients and there are associated to a deleterious outcome. The aim of this study was to describe the echocardiographic features present in patients with scleroderma and compare them with a control group. Methods. Fifty four patients with scleroderma (57 ± 13 years; 93% female sex) and 11 normal patients (48 ± 20 years; 80% female sex) were included. Echocardiographic studies were achieved following the American Society of Echocardiography guidelines. Results. Patients with scleroderma had larger right ventricles: 20.6 ± 4.5 mm vs 15.4 ± 4.3 mm; p=0.02. Doppler analysis showed a greater systolic pulmonary pressure (27.3 ± 5.5 mm Hg vs 20.3 ± 3 mm Hg; p=0.0001) and a shorter right ventricle ejective time (270 ± 57 ms vs 333 ± 32 ms; p=0.001) in the scleroderma group. Tisular Doppler revealed a greater left ventricular A' wave (15.3 ± 3.6 cm/sec vs 1.7 ± 2.6 cm/sec; p=0.01), a taller right ventricle A'wave (17.6 ± 3.6 cm/sec vs 13.6 ± 3.4 cm/sec; p=0.001) and a smaller E'/A' ratio (0.9 ± 0.39 vs 1.13 ± 0.18; p=0.01) in patients with scleroderma. Conclusions. Patients with scleroderma had a larger right ventricle and higher pulmonary pressure. The tisular Doppler findings suggest a lesser ventricular distensibility. It may be a consequence of the greater pulmonary pressure and the intrinsic myocardial damage.

Introdução. A esclerose sistêmica é uma doença crônica caracterizada por fibrose microvascular e tecida. As manifestações cardíacas são comuns, mas estão presentes em apenas um terço dos pacientes e estão associadas a piores resultados. O objetivo deste estudo foi descrever as características ecocardiográficas presentes em pacientes com esclerodermia em comparação com um grupo controle. Material e métodos. Foram estudados 54 pacientes (57 ± 13 anos, 93% do sexo feminino) e 11 controles (48 ± 20 anos, 80% mulheres). Os exames ecocardiográficos foram realizados de acordo com recomendações da Sociedade Americana de Ecocardiografia. Resultados. O grupo esclerodermia mostrou ventrículo direito maior: 20,6 ± 4,5 mm vs 15,4 ± 4,3 mm, p=0,02. A Ecocardiografia Doppler revelou aumento da pressão sistólica pulmonar (27,3 ± 5,5 mm Hg vs 20,3 ± 3 mm Hg; p=0,0001) e menor tempo de ejeção do ventrículo direito (270 ± 57 mseg vs 333 ± 32 mseg, p=0,001) no grupo de pacientes com esclerodermia. O Doppler tissular revelou aumento do tamanho da onda A' do ventrículo esquerdo (15,3 ± 3,6 cm/seg vs 12,7 ± 2,6 cm/seg; p=0,01), aumento do tamanho da onda A' do ventrículo direito (17,6± 3,6 cm/seg vs 13,6± 3,4 cm/seg; p=0,001) com menor E'/A' (0,9 ± 0,39 vs 1, 13 ± 0,18; p=0,01) em pacientes com doença de colágeno. Conclusões. Pacientes com esclerose sistêmica apresentou os resultados sugerindo menor distensibilidad ventricular, como conseqüência provável da maior pressão pulmonar e os danos intrínsecos do miocárdio.

Frecuencia de manifestaciones sistémicas en pacientes con síndrome de Sjögren primario en Argentina / Frequency of systemic manifestations in patients with primary Sjögren's syndrome in Argentina

Del 20–71% de los pacientes con síndrome de Sjögren (SS) desarrolla manifestaciones sistémicas. Objetivos: El objetivo fue evaluar las características clínicoserológicas y frecuencia la de manifestaciones sistémicas en pacientes con SS primario. Material y métodos: Estudio retrospectivo con revisión de historias clínicas de pacientes con Sd de Sjögren primario visitados en el Hospital Británico de Buenos Aires en el período desde Enero de 2000 a Agosto de 2008. Resultados: Se incluyeron 41 paciente que cumplían criterios de clasificación Europeoamericanos 2002 para SS, todos de sexo femenino. La edad media fue 57,85±12,42 años (rango 26–79). El tiempo de evolución fue de 9,28 años (rango 0,08–24). Treinta y tres (80,49%) presentaron manifestaciones sistémicas. Las más frecuentes fueron artritis, vasculitis cutánea y polineuropatía. Este grupo presentó más frecuentemente títulos de AAN ≥1/640 e hipocomplementemia; aunque no estadísticamente significativas. La frecuencia de manifestaciones sistémicas halladas fue mayor a la reportada en otras series. Conclusiones: Un abordaje multidisciplinario enfocado en las manifestaciones sistémicas debería ser el nuevo estándar para el manejo del SS (AU)

Twenty to 71% of patients with Sjögren's syndrome (SS) will develop systemic manifestations. Objective: to characterize the clinical-serological presentation and the frequency of systemic manifestations in patients with primary SS. Methods: Retrospective study including patients with SS visited in “Hospital Británico de Buenos Aires” during the period from January 2000 to August 2008.Results: Forty-one patients fulfilled the 2002 American-European classification criteria for SS. All patients were women. Mean age at enrollment was 57,85±12,42 years (range 26–79). Mean duration of the disease was 9,28 years (range 0,08–24). Thirty-three (80,49%) developed systemic manifestations. The most frequent were arthritis, cutaneous vasculitis and polyneuropathy. This group featured more frequently ANA titles ≥1/640 and hypocomplementemia; although no statistical significance was found. The frequency of systemic manifestations found was greater than reported in the literature.Conclusions: A multidisciplinary approach focusing also on systemic manifestations should be the new standard for management of SS (AU)

[Palindromic rheumatism. Report of one case]. / Reumatismo palindrómico (Enfermedad de Hench Rosemberg). Caso clínico con respuesta a los glucocorticoides.

Palindromic rheumatism is characterized by multiple recurrent episodes of arthritis and periarthritis (mono or oligoarticular) that may last hours or days, disappearing without sequels. We report a 69-year-old male with a history of hypertension and a presumptive diagnosis of gout due to recurrent episodes of arthritis and periarthritis in the last thirty years. They involved at least two joints, lasted few days and were self limited. The patient was admitted due to arthritis and periarthritis of both wrists, knees, ankles, elbows and hands. He presented with fever (38-39 degrees C), intense articular pain and anorexia. With a presumptive diagnosis of palindromic rheumatism and the lack of response to non steroidal anti inflammatory drugs, methylprednisolone 20 mg/od per os was started, with an excellent response.

Reumatismo palindrómico (Enfermedad de Hench Rosemberg): Caso clínico con respuesta a los glucocorticoides / Palindromic rheumatism: Report of one case

Palindromic rheumatism is characterized by multiple recurrent episodes of arthritis and periarthritis (mono or oligoarticular) that may last hours or days, disappearing without sequels. We report a 69-year-old male with a history of hypertension and a presumptive diagnosis of gout due to recurrent episodes of arthritis and periarthritis in the last thirty years. They involved at least two joints, lasted few days and were self limited. The patient was admitted due to arthritis and periarthritis of both wrists, knees, ankles, elbows and hands. He presented with fever (38-39º C), intense articular pain and anorexia. With a presumptive diagnosis of palindromic rheumatism and the lack of response to non steroidal anti infammatory drugs, methylprednisolone 20 mg/od per os was started, with an excellent response.

[Frequency of systemic manifestations in patients with primary Sjögren's syndrome in Argentina]. / Frecuencia de manifestaciones sistémicas en pacientes con síndrome de Sjögren primario en Argentina.

UNLABELLED: Twenty to 71% of patients with Sjögren's syndrome (SS) will develop systemic manifestations. OBJECTIVE: To characterize the clinical-serological presentation and the frequency of systemic manifestations in patients with primary SS. METHODS: Retrospective study including patients with SS visited in "Hospital Británico de Buenos Aires" during the period from January 2000 to August 2008. RESULTS: Forty-one patients fulfilled the 2002 American-European classification criteria for SS. All patients were women. Mean age at enrollment was 57.85 ± 12.42 years (range 26-79). Mean duration of the disease was 9.28 years (range 0.08-24). Thirty-three (80.49%) developed systemic manifestations. The most frequent were arthritis, cutaneous vasculitis and polyneuropathy. This group featured more frequently ANA titles ≥ 1/640 and hypocomplementemia; although no statistical significance was found. The frequency of systemic manifestations found was greater than reported in the literature. CONCLUSIONS: A multidisciplinary approach focusing also on systemic manifestations should be the new standard for management of SS.

Maternal and fetal outcomes of 72 pregnancies in Argentine patients with systemic lupus erythematosus (SLE).

The purpose of the following study was to analyze maternal and fetal outcomes in pregnant patients with systemic lupus erythematosus (SLE) and the influence of SLE exacerbations on those pregnancies. Seventy-two pregnancies in 61 SLE patients treated between January 1986 and February 2004 in Hospital de Clínicas "José de San Martin" were reviewed retrospectively. Patient age was 28.1 +/- 6.2 years (mean+/-standard deviation [SD]). Mean SLE duration was 4.5 +/- 3.2 years (range 6 months-10 years). No patient acquired the disorder during gestation. Four (5.5%) patients had signs of active disease at the beginning of her pregnancy. Sixteen patients, accounting for 20 pregnancies, had a history of lupus nephritis. Nine patients met secondary antiphospholipid syndrome criteria and had 13 pregnancies. There were 14 exacerbations of the disease during pregnancy (19.4%), with most flares being mild. The most common obstetric complications were gestational hypertension in 15 pregnancies (20.8%) and preeclampsia in 8 pregnancies (11%). Forty-six percent of pregnancies ended in preterm deliveries. There were 62 live births (1 twin birth; 85%), 6 stillbirths (8%), and 5 spontaneous abortions (7%). Thirty-nine percent of newborns had low birth weight. Adequate pregnancy follow-up and delivery care by an interdisciplinary team in Argentine SLE patients with no pre-gestational preparation resulted in maternal and fetal outcomes similar to those seen in world reference centers.

Juvenile rheumatoid arthritis and bronchiolitis obliterans organized pneumonia.

Diverse pleuropulmonary manifestations, including pleural effusion, rheumatoid nodulosis, fibrosis, obliterans brochiolitis, bronchiectasias, vasculitis, drug-induced lung disease, and obliterans bronchiolitis with organized pneumonia, have been described in patients with rheumatoid arthritis (RA). Bronchiolitis obliterans organized pneumonia (BOOP) is an uncommon condition described in patients with RA but not in juvenile RA (JRA). We described a patient with JRA who developed a BOOP.

Multiple avascular necrosis in a patient with systemic lupus erythematosus/systemic sclerosis overlap syndrome.

A white female patient developed overlapping features of systemic lupus erythematosus (SLE) and systemic sclerosis (SSc) with severe pulmonary compromise. She was treated with steroids and azathioprine, which improved her clinical condition and spirometric status. In May 2002 she presented with continuous pain in her left ankle that continued even during rest and under treatment with nonsteroidal anti-inflammatory drugs (NSAIDs). Magnetic resonance imaging (MRI) showed multiple avascular necrosis (AVN). Rest and kinesitherapy were indicated for 1 year, and gradually an orthosis was introduced allowing the patient to walk normally.

Manifestaciones respiratorias del síndrome de superposición y la enfermedad mixta del tejido conectivo / Respiratory manifestations of the syndrome of superposition and the mixed connective tissue disease

Se realizó un estudio prospectivo para estudiar las manifestaciones respiratorias en el Síndrome de Superposición [S de S] y la enfermedad mixta de tejido conectivo [EMTC]. El objetivo fue establecer la frecuencia, tipo de afección, y comparar la incidencia entre las dos afecciones. Se estudian los parámetros clínicos y de laboratorio generales, y específicos de éstas patologías realizándose el estudio neumológico consistente en: espirometría, curva de flujo de volumen, volúmenes pulmonares por espirometría, volúmenes pulmonares por el método del lavado de nitrógeno, capacidad de difusión de monóxido de carbono, vólumen alveolar, gases en sangre arterial y presiones bucales máximas. Se realizaron métodos de diagnóstico por imágen consistente en: radiografía de torax frente y perfíl, radiografía de ambas manos con placa industrial, tránsito esofágico y tomografía computada de alta resolución de tórax [TCAR]. Los datos obtenidos fueron registrados en una base de datos dBASE III+ y procesados mediante el programa EPI INFO. Se obtuvieron valores referentes al sexo, edad, talla, tabaquismo y existencia de lupus eritematoso sistémico [LES], esclerosis sistémica progresiva [ESP], dermatomiositis, polimiositis [DM], Síndrome de Sjogren, Test de RNP, actividad clínica, disnea, estertores crepitantes, fenómeno de Raynaud, dolor anginoso, debilidad muscular, capacidad vital forzada [FVC], vólumen espiratorio forzado [FEV1], relación FEV1/FVC, flujo espiratorio forzado al 25 por ciento [FEV25], flujo espiratorio forzado al 50 por ciento [FEV50], flujo espiratorio forzado al 75 por ciento [FEV75], flujo respiratorio forzado entre 25 y 75 por ciento [FEV25-75], flujo espiratorio máximo [FEmax], máxima ventilación voluntaria [MVV], capacidad vital lenta [SVC], capacidad inspiratoria [IC], volúmen de reserva espiratoria [ERV], capacidad residual funcional [FRC], volúmen residual [RV], capacidad pulmonar total [TLC], relación RV/TLC, capacidad de difusión [DLCO] relación [DLCO=VA] y IH aterial Pa02,PaC02, gradiente Aa02, presión respiratoria máxima PImax, y los hallazgos radiológicos. Se obtuvieron de este estudio las siguientes conclusiones: 1. Se obsevó alta incidencia de alteraciones pulmonares en ambas afecciones EMTC y Síndrome de Superposición. 2. Pareciera observarse un mayor compromiso de la función pulmonar en la EMTC que en el Síndrome de Superposición, sin relación con el tiempo de evolución... (TRUNCADO)