Connective tissue disease-associated interstitial lung disease.

Connective tissue disease-associated interstitial lung disease (CTD-ILD) represents a group of systemic autoimmune disorders characterized by immune-mediated organ dysfunction. Systemic sclerosis, rheumatoid arthritis, idiopathic inflammatory myositis, and Sjögren's syndrome are the most common CTDs that present with pulmonary involvement, as well as with interstitial pneumonia with autoimmune features. The frequency of CTD-ILD varies according to the type of CTD, but the overall incidence is 15%, causing an important impact on morbidity and mortality. The decision of which CTD patient should be investigated for ILD is unclear for many CTDs. Besides that, the clinical spectrum can range from asymptomatic findings on imaging to respiratory failure and death. A significant proportion of patients will present with a more severe and progressive disease, and, for those, immunosuppression with corticosteroids and cytotoxic medications are the mainstay of pharmacological treatment. In this review, we summarized the approach to diagnosis and treatment of CTD-ILD, highlighting recent advances in therapeutics for the various forms of CTD.

Relative incidence of interstitial lung diseases in Brazil.

OBJECTIVE: To assess the relative frequency of incident cases of interstitial lung diseases (ILDs) in Brazil. METHODS: This was a retrospective survey of new cases of ILD in six referral centers between January of 2013 and January of 2020. The diagnosis of ILD followed the criteria suggested by international bodies or was made through multidisciplinary discussion (MDD). The condition was characterized as unclassifiable ILD when there was no specific final diagnosis following MDD or when there was disagreement between clinical, radiological, or histological data. RESULTS: The sample comprised 1,406 patients (mean age = 61 ± 14 years), and 764 (54%) were female. Of the 747 cases exposed to hypersensitivity pneumonitis (HP)-related antigens, 327 (44%) had a final diagnosis of HP. A family history of ILD was reported in 8% of cases. HRCT findings were indicative of fibrosis in 74% of cases, including honeycombing, in 21%. Relevant autoantibodies were detected in 33% of cases. Transbronchial biopsy was performed in 23% of patients, and surgical lung biopsy, in 17%. The final diagnoses were: connective tissue disease-associated ILD (in 27%), HP (in 23%), idiopathic pulmonary fibrosis (in 14%), unclassifiable ILD (in 10%), and sarcoidosis (in 6%). Diagnoses varied significantly among centers (c2 = 312.4; p < 0.001). CONCLUSIONS: Our findings show that connective tissue disease-associated ILD is the most common ILD in Brazil, followed by HP. These results highlight the need for close collaboration between pulmonologists and rheumatologists, the importance of detailed questioning of patients in regard with potential exposure to antigens, and the need for public health campaigns to stress the importance of avoiding such exposure.

Clinical insights from an unusual Turner syndrome manifestation: Congenital cutis verticis gyrata.

Cutis verticis gyrata (CVG) is classified as primary or secondary according to the absence or presence of underlying soft tissue abnormalities. We report an infant with Turner syndrome (TS) who in addition presented with CVG on the scalp. The skin biopsy revealed a hamartoma-like lesion. We reviewed the clinical and histopathological findings of the 13 reported cases of congenital CVG in patients with TS, including ours. In 11 of them, CVG was localized on the skin of the scalp, mainly on the parietal region, and in two, on the forehead. Clinically, CVG had a flesh-colored aspect, with absent or sparse hair, and was not progressive. CVG was classified as primary in four patients who had skin biopsy and it was attributed to the intrauterine lymphedema of TS. However, histopathology in two of these patients identified dermal hamartoma as a secondary cause of CVG, and in three others, including ours, there were hamartomatous changes. Although further studies are required, previous findings support the proposal that some CVG may instead be dermal hamartomas. This report alerts clinicians to recognize CVG as a low-frequency manifestation of TS, but also to consider the possible co-occurrence of TS in all female infants with CVG.

Myositis-associated Interstitial Lung Disease: Clinical Characteristics and Factors Related to Pulmonary Function Improvement: A Latin-American Multicenter Cohort Study.

BACKGROUND AND OBJECTIVES: ILD patients can be positive to highly specific autoantibodies of connective tissue diseases (CTD). Among them stand out myositis-specific and associated autoantibodies (MSA/MAA). There is limited knowledge about treatment response and prognosis of ILD patients positive to MSA/MAA (MSA/MAA-ILD). Our aim was to describe clinical, radiological and pulmonary function (PF) of MSA/MAA-ILD Latin-American patients and risk factors associated to PF at onset and long term follow up. METHODS: Multicentric retrospective study of MSA/MAA-ILD patients evaluated between 2016 and 2018 in 3 ILD clinics in Latin America. Clinical, functional and tomographic variables were described. Variables associated with poor baseline PF and associated with functional improvement (FI) were analyzed in a multivariate logistic regression model. RESULTS: We included 211 patients, 77.4% female, mean age 57 years old. Most frequent MSA/MAA were Ro-52 and Jo-1. Poor baseline PF was associated to ILD as initial diagnosis and NSIP/OP HRCT pattern. 121 patients were included in the follow up PF analysis: 48.8% remained stable and 33% had a significant FI. In multivariate analysis, OP pattern on HRCT was associated with FI. Systemic symptoms from the beginning and the absence of sclerodactyly showed a trend to be associated with FI. CONCLUSIONS: Worse baseline PF could be related to the absence of extra-thoracic symptoms and "classic" antibodies in CTD (ANA), which causes delay in diagnosis and treatment. In contrast, FI could be related to the presence of extra-thoracic signs that allow timely diagnosis and therapy, and more acute and subacute forms of ILD, such as OP pattern.

Inflammatory myopathies overlapping with systemic sclerosis: a systematic review.

We performed a systematic review of the clinical manifestations and complementary exams of patients with myopathies and systemic sclerosis overlap syndrome (MyoSScOS). Systematic review from January 1976 to November 2021 according PRISMA protocol on three electronic databases: PubMed, Web of Science, and Scopus. Studies were analyzed based on the following eligibility criteria: at least one combination of the terms described in the search strategy appears in the title; written in English, Portuguese, or Spanish; and addresses MyoSScOS. Brief communications, reviews, studies that addressed myopathies in children, congress proceedings, monographs, and dissertations were excluded. Thirty-five articles were selected. MyoSScOS seems to be more common in women. It also commonly affects the esophagus and joints with symmetrical and bilateral muscle involvement, Raynaud's phenomenon, and impairment of forced vital capacity. Concerning SSc, the most common subtype was the diffuse form. Cardiovascular and pulmonary complications are an important cause of death. Anti-centromere, anti-PM/Scl, anti-Scl70, anti-RNA polymerase III, anti-Ku, and anti-RNP were more correlated with this entity, and muscle biopsies may present a more aggressive pattern. Electroneuromyography patterns are quite similar to those found in inflammatory myopathies. The absence of studies with robust methodologies and the large number of case reports and series make more robust statistical analyses such as meta-analyses unfeasible. The characterization of MyoSScOS is important for the formulation of therapeutic measures and specific treatments aiming at better quality of life and prognosis. Greater and better theoretical contributions are necessary to better characterize it.

[Overview of the interstitial lung disease in the Southeast of Mexico]. / Panorama de la enfermedad pulmonar intersticial en el sureste de México.

Background: Interstitial lung disease (ILD) corresponds to a heterogeneous group of pathologies that differ in etiology with common clinical and radiological manifestations. In Latin America and Mexico, reports are scarce and the need for studies to understand the scenario is emphasized. Objective: To analyze a multidimensional profile in patients with interstitial lung disease in Yucatan. Method: This is an observational, prospective, analytic, descriptive study including consecutive patients diagnosed with ILD over a 4-year period. Demographic and clinical data, lung function tests, chest imaging, serum immunological profile, and echocardiographic findings were recorded. Differences between subgroups were analyzed performing a one-way analysis of variance (ANOVA). Results: 110 patients were included. The median age was 60 years and women were most affected. The main cause of ILD was related with connective tissue diseases (CTD). A group subanalysis revealed that Idiopathic pulmonary fibrosis (IPF) was common in males with a history of smoking and an imaging pattern of usual interstitial pneumonia. Lung function tests showed a moderate-to-severe pulmonary restriction (FVC 55%p) and mild hypoxemia (PaO2 79mmHg). Positive antinuclear antibodies are less likely in cases with IPF (20 vs. 65%; p = 0.006). Conclusion: In Southeastern Mexico, ILD occurs in women in their seventh decade of life; the most common cause is related with CTD. Our results support that ILD has a heterogeneous expression and is relevant the need for subsequent studies characterizing each ILD.

Introducción: La enfermedad pulmonar intersticial (EPI) corresponde a un grupo heterogéneo de patologías que difieren en su etiología pero tienen manifestaciones clínicas y radiológicas comunes. En Latinoamérica y México los reportes son escasos, enfatizando la necesidad de estudios que permitan conocer su escenario clínico-epidemiológico. Objetivo: Realizar un análisis multidimensional y contrastado de la EPI en la población de la Península de Yucatán. Método: Estudio observacional, prospectivo, analítico y descriptivo, que incluye la totalidad de pacientes diagnosticados de EPI en un período de 4 años. Se registraron datos demográficos y clínicos, pruebas de función pulmonar, imagenología del tórax, perfil inmunológico y ecocardiografía transtorácica. Se analizaron las diferencias según la etiología mediante análisis de la varianza de una sola vía (ANOVA). Resultados: Se incluyeron 110 pacientes con una mediana de edad de 60 años y predominio del sexo femenino. La causa principal de la EPI fue relacionada con enfermedad del tejido conectivo. El subanálisis de grupos mostró que la fibrosis pulmonar idiopática (FPI) es frecuente en los varones con antecedente de tabaquismo y patrón tomográfico de neumonía intersticial usual. Las pruebas de función pulmonar demostraron restricción pulmonar moderadamente grave (FVC 55%p) e hipoxemia leve (PaO2 79 mmHg). La positividad de anticuerpos antinucleares ocurre en menor proporción en la FPI (20 vs. 65%, p = 0.006). Conclusiones: En el sureste de México, la EPI ocurre en mujeres de la séptima década de la vida y se relaciona con enfermedad del tejido conectivo. Nuestros resultados respaldan que la EPI tiene expresión heterogénea y se requieren estudios subsecuentes sobre cada tipo de enfermedad.

Evaluación reumatológica del paciente con enfermedad pulmonar intersticial / Rheumatological evaluation of the patient with interstitial lung disease

Las Enfermedades del Tejido Conectivo (ETC) comprenden un grupo heterogéneo de patologías multisistémicas de origen autoinmune. La Enfermedad pulmonar intersticial (EPI) asociada a ETC (EPI-ETC) es frecuente y empeora el pronóstico de la ETC. Las EPI-ETC representan aproximadamente 15-30% del total las EPI y se presentan con las mismas formas histopatológicas y radiológicas descritas para las EPI idiopáticas. Esto pone en evidencia la importancia de incorporar en forma rutinaria a reumatología en el comité multidisciplinario para el diagnóstico y manejo de las EPI.

Connective Tissue Diseases (CTD) comprise a heterogeneous group of multisystemic pathologies of autoimmune origin. Interstitial lung disease (ILD) associated with CTD (CTD-ILD) is common and and it worsens the prognosis of CTD. CTD-ILD represent approximately 15-30% of the universe of ILD and have the same histopathological and radiological forms described for idiopathic ILD. This highlights the importance of routinely incorporate a rheumatologist into the multidisciplinary committee for the diagnosis and management of ILD.

Vasculopatia livedoide tratada com rivaroxabana / Livedoid vasculopathy treated with rivaroxaban

A vasculopatia livedoide é uma doença rara caracterizada pela oclusão da microvasculatura da derme, originando lesões maculosas que, posteriormente, podem evoluir para úlceras e cicatrizes atróficas. Como um fenômeno vaso-oclusivo, o tratamento geralmente é realizado com antiplaquetários e fibrinolíticos. O presente relato descreve o caso de uma paciente refratária à terapia convencional, que obteve regressão da doença utilizando a rivaroxabana, um fármaco inibidor seletivo do fator Xa. (AU)

Livedoid vasculopathy is a rare disease characterized by occlusion of the dermis microvasculature, leading to spotted lesions that can later develop into ulcers and atrophic scars. As a vaso- occlusive phenomenon, treatment is usually performed with antiplatelet and fibrinolytic agents. The present report describes the case of a female patient refractory to conventional therapy who presented disease remission using rivaroxaban, a selective factor Xa inhibitor drug. (AU)

Pulmonary Vasoreactivity and Phenotypes in Pulmonary Arterial Hypertension Associated to Connective Tissue Diseases.

BACKGROUND: Pulmonary arterial hypertension (PAH) is a fatal complication in patients with connective tissue disease (CTD). OBJECTIVE: The objective of the study was to study the prognostic value of the acute pulmonary vasoreactivity test with inhaled iloprost and its association with clinical deterioration in a tertiary care academic medical center. METHODS: We conducted a prospective study of patients with CTD and the diagnosis of PAH established by right heart catheterization. Patients were classified into classic responders, partial responders, and non-responders. The association of the pulmonary response and clinical deterioration was analyzed. RESULTS: We enrolled 25 patients (mean age of 47 ± 13.4 years); 88% were female. The most frequent rheumatologic diagnosis was systemic lupus erythematosus, in 16 (64%) patients. Seventy-two percent of patients were classified as non-responders, and 28% were partial responders. Patients with a partial response had lower right atrial pressure values (5.1 ± 3.1 vs. 8.5 ± 3.2, p = 0.01) and greater systolic pulmonary arterial pressure (87.6 ± 8.1 vs. 72.4 ± 16.2, p = 0.02), compared with non-responders. Non-responders had a tendency for a shorter time to clinical deterioration than partial responders (17.8 vs. 41.1 months, p = 0.052). CONCLUSIONS: Patients with a partial response to the acute pulmonary vasodilator test with inhaled iloprost had a longer clinical deterioration-free period than non-responders.

Hipertensión de la arteria pulmonar y enfermedades autoinmunes del tejido conectivo: revisión de la literatura / Pulmonary arterial hypertension and autoimmune connective tissue diseases: literature review

La hipertensión de la arteria pulmonar es una grave complicación que pueden presentar los pacientes con enfermedades autoinmunes del tejido conectivo Exis-ten distintas prevalencias reportadas según cada país. Por otro lado, la sobrevida de estos pacientes reportada al año y a los tres años, va desde 70-82% y 47-53% respectivamente dependiendo de cual es la enfermedad del tejido conectivo aso-ciada. En los últimos años se ha avanzado en la precocidad del diagnóstico y han aparecido nuevas terapias que han demostrado mejores resultados. Sin embargo, la respuesta al tratamiento sigue siendo mejor en pacientes con hipertensión pul-monar idiopática que en aquellas asociadas a enfermedad de tejido conectivo.

Pulmonary artery hypertension is a serious complication that may occur in pa-tients with autoimmune diseases of the connective tissue. There are different prevalence reported by country. On the other hand, the survival of these patients reported at one year and three years, going from 70-82% and 47-53% respec-tively, depending on which is the associated connective tissue disease. In recent years it has made progress in the precocity of diagnosis and new therapies have appearedthat have shown better results. However, the response to treatment is still better in patients with idiopathic pulmonary hypertension than in those associated with connective tissue disease.

Caracterización de pacientes con enfermedades autoinmunes del tejido conectivo e hipertensión de arteria pulmonar atendidos en Hospital San Juan de Dios / Patients characterization with autoimmune diseases of the connective tissue and high blood pressure in the pulmonary artery seen in hospital San Juan de Dios

La hipertensión de la arteria pulmonar (HAP) es una grave complicación que pue-den presentar los pacientes con enfermedades autoinmunes del tejido conectivo de prevalencia variable según cada país. Habitualmente su diagnóstico es tardío lo que influye negativamente en su pronóstico y respuesta terapéutica. En el pre-sente trabajo se ha iniciado una revisión en conjunto entre la Unidad de Hemodi-namia y de Reumatología del Hospital San Juan de Dios, con el propósito de tener una caracterización de los pacientes con HAP y enfermedades autoinmunes del tejido conectivo y poder así elaborar planes de manejo de acuerdo a las caracte-rísticas clínicas de estos pacientes e intentar obtener un diagnostico precoz, para mejorar la sobrevida de los portadores de estas enfermedades.

High blood pressure in the pulmonary artery (HAP) is a serious complication that may occur in patients with autoimmune diseases of the connective tissue of vari-able prevalence according to each country. Diagnosis is usually late which affects negatively their prognosis and therapeutic response. In the present work has been initiated a review in conjunction between the unit of Hemodynamic and Rheumatology of the Hospital San Juan de Dios, with the purpose of having a characterization of patients with PAH and autoimmune diseases of the connective tissue and can thus develop management plans according to the clinical features of these patients and try to get an early diagnosis, to improve the survival of the carriers of these diseases.

Immune diffuse alveolar hemorrhage: Clinical presentation and outcome.

BACKGROUND: Diffuse alveolar haemorrhage (DAH) is a manifestation of several immune and nonimmune diseases. OBJECTIVE: The objective of this study was to characterize the clinical characteristics and short-term outcomes of patients with immune-mediated DAH requiring hospital admission. METHODS: A retrospective study from December 2010 to December 2015, was conducted by analyzing the clinical records of 39 patients with DAH with a proven immunological origin. The diagnosis of individual collagen vascular diseases (CVD) was made according to the criteria of the corresponding societies. RESULTS: Thirty-nine patients were included (median age 44.8 years, range 16-76). The main causes of DAH were ANCA-related vasculitis (74.3%) mainly granulomatosis with polyangiitis (n = 14) and microscopic polyangiitis (n = 13). Thirty patients (76.9%) had hemoptysis. An alveolar airspace filling pattern was found in most of patients (59%). All the patients had a drop in hemoglobin level that ranged from 1.0 to 3.0 g/dL. BAL fluid was macroscopically bloody in 43.6% of patients (n = 17) and showed siderophagic alveolitis on BAL cytology in 100%. All patients received high doses of corticosteroids. Other additional treatments were antibiotics (53,8%, n = 21), intravenous cyclophosphamide (87.2%, n = 34), plasma exchange (35.9%, n = 14); intravenous immunoglobulin (12.8%, n = 5) and rituximab in 5 patients (12.8%). Mortality rate was higher amongst patients who required dialysis (50.0 vs 15.4%, p = 0.045), with SaO2 <90% at admission (50.0 vs 5.3%, p = 0.003) or those who required mechanical ventilation (76.9 vs 6.8%, p = < 0.001). CONCLUSION: DAH may present without hemoptysis and requires an early bronchoscopy to confirm the diagnosis and exclude infection. Other characteristics could be included in the abstract which are relevant to the paper. (relation between mortality, dialysis, ventilation, etc.).

Laparoscopic Antireflux Surgery in Patients with Connective Tissue Diseases.

Different connective tissue diseases (CTDs), such as dermatomyositis, mixed CTD, rheumatoid arthritis, polymyositis, lupus, and Behçet's, may affect the esophagus, impairing its motor function. The muscular atrophy and fibrosis caused by the autoimmune vasculitis and neuronal dysfunction affect the esophageal body and the lower esophageal sphincter, leading to a clinical presentation of dysphagia and gastroesophageal reflux disease (GERD). The belief that the impaired esophageal motility may negatively affect surgical outcome has led to the common recommendation of avoiding laparoscopic antireflux surgery (LARS) for fear of creating or worsening dysphagia. This review focuses on the evaluation of the outcome of LARS in patients with CTD. Specifically, this review shows that the literature on LARS and CTDs is scarce and most studies have a small number of patients and a short follow-up. Furthermore, a subanalysis of the outcome based on the type of CTD or the manometric profile is still elusive. In the setting of these limitations, it appears that results are good and comparable to those of patients with GERD and without a CTD. Morbidity and mortality are insignificant even considering the systemic manifestations of the CTD. LARS should not be denied to patients with CTD and GERD.

An update of neurological manifestations of vasculitides and connective tissue diseases: a literature review / Atualização das manifestações neurológicas das vasculitides e das doenças do tecido conjuntivo: revisão de literatura

Vasculitides comprise a heterogeneous group of autoimmune disorders, occurring as primary or secondary to a broad variety of systemic infectious, malignant or connective tissue diseases. The latter occur more often but their pathogenic mechanisms have not been fully established. Frequent and varied central and peripheral nervous system complications occur in vasculitides and connective tissue diseases. In many cases, the neurological disorders have an atypical clinical course or even an early onset, and the healthcare professionals should be aware of them. The purpose of this brief review was to give an update of the main neurological disorders of common vasculitis and connective tissue diseases, aiming at accurate diagnosis and management, with an emphasis on pathophysiologic mechanisms.

As vasculites são um grupo heterogêneo de doenças autoimunes primárias ou secundárias a uma grande variedade de doenças infecciosas sistêmicas, malignas ou do tecido conjuntivo. Estas últimas são as que ocorrem com mais frequência, porém os mecanismos patogênicos ainda não foram plenamente determinados. Diversas e frequentes complicações do sistema nervoso central e periférico ocorrem nas vasculites e doenças do tecido conjuntivo. Em muitos casos, os distúrbios neurológicos têm evolução clínica atípica ou mesmo início precoce, ao que todos os profissionais de saúde devem estar cientes. O objetivo desta breve revisão foi atualizar os principais distúrbios neurológicos da vasculite comum e das doenças do tecido conjuntivo, visando ao diagnóstico e ao tratamento corretos, com ênfase nos mecanismos fisiopatológicos.

An update of neurological manifestations of vasculitides and connective tissue diseases: a literature review.

Vasculitides comprise a heterogeneous group of autoimmune disorders, occurring as primary or secondary to a broad variety of systemic infectious, malignant or connective tissue diseases. The latter occur more often but their pathogenic mechanisms have not been fully established. Frequent and varied central and peripheral nervous system complications occur in vasculitides and connective tissue diseases. In many cases, the neurological disorders have an atypical clinical course or even an early onset, and the healthcare professionals should be aware of them. The purpose of this brief review was to give an update of the main neurological disorders of common vasculitis and connective tissue diseases, aiming at accurate diagnosis and management, with an emphasis on pathophysiologic mechanisms.

Perfil clínico radiológico de 18 pacientes con síndrome antisintetasas atendidos en Policlínico de Pulmón Reumatológico, Instituto Nacional del Tórax / Clinic and radiologic profile of 18 patients with antisynthetase syndrome, treated in a Rheumatologic Lung Disease Clinic, Instituto Nacional del Torax

Connective Tissue Diseases (CTD) can manifest as Interstitial lung disease (ILD). ILD is a common manifestation of Anti-synthetase syndrome (AS). The main pattern of ILD in AS is nonspecific interstitial pneumonia (NSIP) with or without elements of organizing pneumonia (OP). Other less common forms include usual interstitial pneumonia (UIP) and nonspecific forms. Objectives: Describe radiological and clinical profile of 18 patients with ILD due to AS, evaluated in Instituto Nacional del Torax (INT) between 2013 and 2015. Highlighting the importance of Myositis Panel in patients being evaluated for ILD. Methods: Review of clinical records and lung CT of 76 patients with ILD and suspected AS, seen at INT between august 2013 and July 2015. Results: The diagnosis of AS was made in 18 of 76 patients, with female predominance and mean age of 46.5 years . In most patients the diagnosis of AS and ILD was simultaneous. The most frequent AS antibody was Jo-1, followed by PL-12. Less than half had ANA (+). The predominant radiographic pattern was NSIP / OP. Half of the patients had myositis. Conclusions: AS should be suspected in patients under study for ILD, especially NSIP / OP, and may occur without myositis and with negative ANA. It is essential to have new immunological tests such as a Myositis Panel, which will allow us to diagnose AS with subtle clinical features and negative or inconclusive serology...

Las Enfermedades del tejido conectivo (ETC) se pueden manifestar como Enfermedad Pulmonar Difusa (EPD). El Síndrome Antisintetasas (SAS) con mucha frecuencia presenta EPD. La forma de expresión más frecuente en SAS es la neumonía intersticial no específica (NSIP) con o sin elementos de neumonía en organización (OP). Otras formas menos frecuentes son la neumonía intersticial usual (UIP) y formas inespecíficas. Objetivos: Describir el perfil clínico radiológico de 18 pacientes con EPD secundaria a SAS, atendidos en el Instituto Nacional del Tórax (INT) entre los años 2013 y 2015. Destacar la importancia del Panel de Miositis en pacientes con EPD en estudio. Métodos: Revisión de ficha clínica y TAC pulmonar de 76 pacientes con EPD y sospecha de SAS, atendidos en INT entre agosto 2013 y julio 2015. Resultados: En 18 de 76 pacientes se hizo el diagnóstico de SAS, predominio femenino, edad promedio 46,5 años. En la mayoría el diagnóstico reumatológico y pulmonar fue simultáneo, el anticuerpo antisintetasa más frecuente fue Jo-1, seguido de PL 12. Menos de la mitad tuvo ANA (+). El patrón radiológico predominante fue NSIP/OP. La mitad de los pacientes no presentaron miositis. Conclusiones: El SAS debe ser sospechado en el estudio de pacientes con EPD, especialmente NSIP/OP y se puede presentar sin miositis y ANA (-). Es indispensable contar con nuevos exámenes inmunológicos como el Panel de Miositis, que permite diagnosticar SAS con clínica sutil y serología habitual negativa o no concluyente...

Dermatomyositis: analysis of 109 patients surveyed at the Hospital das Clínicas (HCFMUSP), São Paulo, Brazil.

BACKGROUND: Dermatomyositis affects striated muscles, skin and other organs. OBJECTIVE: To characterize the disease from January 1992 to December 2002, assessing its classification, cutaneous and systemic manifestations, and also laboratory results, therapeutic and prognostic findings compared to those in the literature. METHODS: Data were obtained from medical records of 109 patients who were classified into five groups: 23 juvenile dermatomyositis; 59 primary idiopathic dermatomyositis; 6 amyopathic dermatomyositis; 7 dermatomyositis associated with neoplasms and 14 dermatomyositis associated with other connective tissue diseases. RESULTS: Sixty patients were classified as "definite" diagnosis; 33 as "possible"; four as "probable" and 12 and as amyopathic. The average age at diagnosis was 36 years. Cutaneous manifestations occurred in all patients; the most frequent symptom was loss of proximal muscle strength; the most common pulmonary disorder was interstitial lung disease, and gastritis was the most prevalent digestive manifestation. Tumors were documented in 6.42% of cases. Lactate dehydrogenase was the muscle enzyme most frequently elevated in the majority of cases. Skin biopsies were performed in 68 patients; muscle biopsies in 53; and electroneuromyographies in 58 patients. The most commonly used treatment was corticotherapy and the mortality rate was 14.7%. CONCLUSION: in this sample, the disease appeared in younger individuals, was more frequent in women and the association with cancer was small.