Contribución del panel de miositis en el estudio de pacientes con enfermedades pulmonares intersticiales y sospecha de enfermedad del tejido conectivo / Contribution of myositis panel in the study of patients with interstitial lung disease and suspicion of connective tissue disease

INTRODUCCIÓN: La manifestación extramuscular de las miopatías inflamatorias idiopáticas (MII) es la enfermedad pulmonar intersticial (EPI) y el diagnóstico se basa en autoanticuerpos séricos. Los nuevos anticuerpos específicos y asociados a MII han ayudado a identificar nuevas entidades clínicas en el espectro de MII. El objetivo de este estudio es evaluar la contribución diagnóstica de un panel de anticuerpos de miositis (PM) en una cohorte de pacientes chilenos con EPI sin una enfermedad del tejido conectivo (ETC) definitiva. MATERIALES Y MÉTODOS: A partir de enero de 2017 se realizó un panel de miositis a 111 pacientes consecutivos con EPI y sospecha de ETC, pero sin un diagnóstico definitivo a través de otra herramienta diagnóstica, en el programa de Pulmón-Reumatológico del Instituto Nacional del Tórax, Santiago, Chile. Se compararon las características basales clínicas y serológicas de los pacientes que se asociaban más frecuentemente a la probabilidad de tener un panel positivo. RESULTADOS: El PM fue positivo en 56 de 111 pacientes. El síndrome antisintetasa (SAS) fue el diagnóstico más frecuente. Los anticuerpos más frecuentes fueron Ro-52, PM / Scl-75 y Ku. Las variables más frecuentes en el grupo PM(+) fueron la presencia del Raynaud, miositis, manos de mecánico, los anticuerpos Ro y La positivos, la presencia de un patrón combinado de neumonía intersticial inespecífica y neumonía organizada en la tomografía computarizada de tórax. CONCLUSIONES: la incorporación del PM nos ha ayudado a mejorar nuestra precisión diagnóstica en pacientes con EPI / ETC. Presentamos elementos clínicos y serológicos que perfeccionan el rendimiento de la prueba.

INTRODUCTION: The most common extramuscular manifestation of the idiopathic inflammatory myopathies (IIM) is interstitial lung disease (ILD) and the diagnosis is based on serum autoantibodies. The new specific and associated antibodies to IIM have helped to identify new clinical entities in the spectrum of IIM. The objective of this study is to evaluate the diagnostic contribution of a myositis antibodies panel (MP) in a cohort of Chilean patients with ILD without a definitive connective tissue disease (CTD). MATERIALS AND METHODS: Starting on January 2017 we performed a MP to 111 consecutive patients with ILD and suspected CTD but without a definitive diagnosis through another diagnostic tools in the Lung-Rheumatological Program at the "Instituto Nacional del Tórax", Santiago, Chile. The clinical and serological baseline characteristics of the patients that were most frequently associated with the probability of having a positive panel were compared. RESULTS: The MP was positive in 56 of 111 patients. Anti synthetase syndrome (ASS) was the most prevalent diagnosis. The most frequent antibodies were Ro-52, PM/Scl-75 and Ku. The most frequent variables in the positive MP group were the presence of Raynaud's phenomenon, myositis, mechanic's hands, positive Ro and La antibodies and the presence of combined pattern of nonspecific interstitial pneumonia and organizing pneumonia in chest computed tomography scan. CONCLUSIONS: The incorporation of the MP has helped us to improve our diagnostic precision of patients with CTD/ILD. We present clinical and serological elements that refine the performance of the test.

Frecuencia de anticuerpos para diagnóstico de enfermedad celíaca en pacientes con enfermedades del tejido conectivo y artropatías inflamatorias / Serum anti endomysial and anti transglutaminase antibodies in patients with connective tissue diseases

Background: The detection of anti-transglutaminase IgA (tTG) and anti-endomysial (EMA) is used for screening of celiac disease (CD) with a sensitivity and specificity of 90 and 99% respectively. There is an association between CD and connective tissue diseases (CTD). Aim: To report the frequency of IgA tTG and EMA in patients with a definite diagnosis of CTD and inflammatory arthropathies (IA). Material and Methods: One hundred forty nine patients, aged 19 to 86 years (133 females) with CTD and IA were studied. tTG were determined by ELISA and EMA by indirect immunofluorescence. Results: Eight participants had at least one positive antibody (5.4%, confidence intervals (CI) = 1.8-9), six had both (4.0% CI = 0.9-7.2) and two had only tTG positive. An intestinal biopsy was performed in four of these participants, finding a marked villous atrophy in three and partial atrophy in one. Conclusions: Five percent of this group of patients with CTD or IA had positive antibodies for CD.

Comparative study of ophthalmological and serological manifestations and the therapeutic response of patients with isolated scleritis and scleritis associated with systemic diseases / Estudo comparativo entre as manifestações oftalmológicas, sorológicas e resposta terapêutica de pacientes com esclerite isolada e esclerite associada a doenças sistêmicas

INTRODUCTION: Scleritis is a rare, progressive and serious disease, the signs of which are inflammation and edema of episcleral and scleral tissues and is greatly associated with systemic rheumatoid diseases. PURPOSE: To perform a prospective and comparative study between ophthalmologic manifestations, serologic findings and therapeutic response of patients with isolated scleritis and scleritis associated with systemic rheumatoid disease. METHODS: Thirty-two outpatients with non-infectious scleritis were studied, from March 2006 to March 2008. The treatment was corticoid eye drops associated with anti-inflammatory agents, followed by systemic corticoids and immunosuppressive drugs if necessary, was considered successful after six months without scleritis recurrence. RESULTS: Fourteen of 32 patients had scleritis associated with systemic rheumatoid disease, of which nine had rheumatoid arthritis, two systemic lupus erythematosus, one Crohn's disease, one Behçet's disease and one gout. There were no difference in relation to involvement and ocular complications, there was predominance of nodular anterior scleritis and scleral thinning was the most frequent complication. The scleritis associated with systemic rheumatoid disease group had 64.3 percent of autoantibodies, versus 27.8 percent among those with isolated scleritis and this difference was statistically significant. In the isolated scleritis group 16.7 percent used anti-inflammatory, 33.3 percent corticosteroids, 27.8 percent corticosteroids with one immunosuppressive drug, 5.5 percent two immunosuppressive drugs, 16.7 percent corticosteroids with two immunosuppressive drugs and 33.3 percent pulse of immunosuppressive drugs, there was remission in 88.9 percent. In the scleritis associated with systemic rheumatoid disease group 7.1 percent used anti-inflammatory, 7.1 percent corticosteroids, 50 percent corticosteroids with one immunosuppressive drug, 7.1 percent two immunosuppressive drugs and 22.2 percent pulse of immunosuppressive drugs, 100 percent had treatment success. CONCLUSION: Prevalence of unilateral nodular scleritis was noted in both groups and higher rates of all the parameters tested were noted in the scleritis associated with systemic rheumatoid disease group. There were no differences between the groups with respect to the use of immunosuppressive drugs and therapeutic response, which was fully satisfactory in the scleritis associated with systemic rheumatoid disease group and satisfactory in the isolated scleritis group.

INTRODUÇÃO: Esclerite é uma doença grave, rara e progressiva, que envolve inflamação e edema dos tecidos episcleral superficial, profundo e escleral e está associada com doenças sistêmicas reumatológicas em muitos casos. OBJETIVOS: Realizar um estudo prospectivo comparativo entre as manifestações oftalmológicas, achados sorológicos e resposta terapêutica de pacientes com esclerite isolada e com esclerite associada a doenças sistêmicas reumatológicas. MÉTODOS: Trinta e dois pacientes com esclerite não infecciosa participaram do estudo, de março de 2006 a março de 2008. O tratamento realizado baseou-se no uso de colírios de corticoides associados aos anti-inflamatórios não-hormonais, seguidos de corticoides sistêmicos e imunossupressores, se necessário. O sucesso do tratamento foi considerado como seis meses sem crises de esclerite. RESULTADOS: Quatorze dos 32 pacientes apresentaram esclerite associada à doença sistêmica, dos quais nove com artrite reumatóide, dois com lúpus eritematoso sistêmico, um com doença de Crohn, um com doença de Behçet e um com gota. Não houve diferenças em relação ao envolvimento ocular e suas complicações, predominando a esclerite anterior nodular e o afinamento escleral, respectivamente. O grupo com esclerite associada a doenças sistêmicas apresentou 64,3 por cento de positividade de autoanticorpos contra 27,8 por cento no grupo com esclerite isolada, sendo tal diferença estatisticamente significante. No grupo com esclerite isolada, 16,7 por cento fez uso de apenas anti-inflamatórios, 33,3 por cento de corticoide sistêmico, 27,8 por cento de corticoide com um imunossupressor, 5,5 por cento dois imunossupressores, 16,7 por cento corticoide com dois imunossupressores e 33,3 por cento pulsoterapia com imunossupressor; sendo que houve sucesso do tratamento em 88,9 por cento. No grupo com esclerite associada à doença sistêmica, 7,1 por cento fez uso de anti-inflamatórios, 7,1 por cento corticoide sistêmico, 50 por cento corticoide com um imunossupressor, 7,1 por cento dois imunossupressores e 22,2 por cento pulsoterapia com imunossupressor; com 100 por cento de sucesso no tratamento nesse grupo. CONCLUSÃO: Em ambos os grupos houve predomínio da esclerite nodular unilateral e o grupo com esclerite associada a doença sistêmica apresentou taxas maiores de todos os autoanticorpos testados. Não houve diferença entre os grupos em relação ao uso de imunossupressores e à resposta terapêutica, a qual foi totalmente satisfatória no grupo com esclerite associada à doença sistêmica e satisfatória no grupo com esclerite isolada.

Anticuerpos antinucleares, imágenes y características obtenidas por inmunofluorescencia: Importancia de los isotipos IgA, IgM e IgG / Antinuclear antibodies, patterns and characteristics obtained by immunofluorescence: The importance of the IgA, IgM and IgG isotypes

La técnica de elección para el screening de anticuerpos antinucleares (ANA) es la inmunofluorescencia indirecta que utiliza como sustrato una línea de células epiteliales de carcinoma de laringe humano (IFI-HEp2), y como antisuero, anti-IgG o anti-Ig totales. Los ANA-IgG son los más importantes para el diagnóstico y monitoreo de las enfermedades del tejido conectivo (ETC), mientras los ANA-IgM son de menor relevancia clínica en estos pacientes. Sin embargo, poco se sabe de los ANA-IgA ya que estos Ac han sido menos investigados. El objetivo de este trabajo fue estudiar la prevalencia de los diferentes isotipos de inmunoglobulinas de anticuerpos antinucleares en los pacientes con ETC y evaluar la conveniencia de utilizar conjugados monovalentes o polivalentes. Se procesaron 100 sueros de pacientes con diversas ETC empleando IFI-HEp2, en los cuales se detectó 38% de ANA-IgA (títulos ≥ 1:80) y 12% de ANA-IgM (títulos ≤ 1:160). En 29 casos se detectó IgA en ausencia de IgM, en 3 casos IgM en ausencia de IgA. En todos los casos los ANA-IgG estuvieron presentes. En 6 sueros se observó un cambio de imagen con conjugado anti-IgA y en 3 con conjugado anti-IgM. Debido a la alta prevalencia de ANA-IgA detectada por IFI-HEp2, se destaca la conveniencia de utilizar conjugado anti-Ig totales en lugar de anti-IgG, mientras se desconozca la relevancia de los ANA-IgA en el diagnóstico, pronóstico y seguimiento de las enfermedades reumáticas sistémicas.

The indirect immunofluorescence with epitelial cell line from human laryngeal carcinoma as substrate (IIF-HEp2) and anti-IgG or anti-total Ig as antisera, is the technique currently used for the detection of antinuclear antibodies. The most important antibodies for the diagnosis and follow-up of connective tissue diseases (CTD) are the IgG-ANA, while the IgM-ANA have no clinical relevance. However the IgA-ANA have not been thoroughly investigated so far. The aim of this work was to study the prevalence of different ANA isotypes of Ig antibodies in CTD patients and to evaluate the convenience of the use of monovalent or polyvalent conjugate. We examined the sera of 100 patients with different CTD by IIF-HEp2 and detected a prevalence of 38% IgA-ANA (titles ≥ 1:80) and 12% IgM-ANA (titles ≤ 1:160). In twenty nine cases we detected IgA-ANA in absence of IgM-ANA, and in 3 cases IgM-ANA in absence of IgA-ANA. In all the cases IgG-ANA were present. In 6 sera a change in the immunofluorescence pattern was observed while using anti-IgA conjugate, whereas in 3 the change was observed with the use of anti-IgM conjugate. Because of the high prevalence of ANA-IgA detected by IIF-HEp2, we emphasize the convenience of employing anti-total Ig in spite of anti-IgG conjugated until the role of ANA-IgA is dilucidated in CTD patients, in order to establish its relevance in the diagnosis, prognosis and follow-up of systemic rheumatic diseases.

role of the clinical immunology laboratory in the diagnosis and monitoring of connective tissue diseases

Connective tissue diseases CTD are a group of autoimmune systemic diseases that can affect any organ-system in the body. The initial clinical presentations of these diseases overlap, not only with each other, but also with a wide range of other rheumatological and non-rheumatological disorders. Due to these reasons, clinicians depend heavily on the use of the clinical immunology laboratory for the diagnosis of CTD. A large number of tests exist in the laboratory for the investigation of CTD and each test can be performed by a number of different methods, each with its own limitations. Consequently, the significance of the results generated not only has to be interpreted in relation to the clinical picture, but also to the method used to generate the results. Moreover, within the laboratory, there is a hierarchical testing system for the investigation of CTD and if this system is used appropriately, in conjunction with the clinical picture, can result in the diagnosis/exclusion of CTD more efficiently and economically. In contrast, random use of the laboratory tests, combined with limited knowledge of the methods used to carry out these tests, can lead to delay or even misdiagnosis, as well as can lead to wastage of resources. In the following review, we have discussed the various tests that are used in the investigation of CTD, as well as the different methods used to carry out these tests, with the hope that such knowledge would lead to a more efficient and economical use of the clinical immunology laboratory in the investigation of CTD

Anti Ro/SSA positive undifferentiated connective tissue disease in a mother with a newborn with complete congenital heart block: a case report.

An association between complete congenital heart block (CCHB) and anti-Ro/SSA antibody is well recognized but has never been reported in Thailand. We report here a 37-year-old female who was admitted because of massive epistaxis secondary to immune thrombocytopenia. She had given birth to a child with CCHB 2 years previously, when she was healthy. Antinuclear antibody and anti-Ro/SSA were positive in her sera, but were negative in her son. The relationship between anti-Ro/SSA antibody and outcome of mothers with infants with CCHB is reviewed.

Silicone breast implants, auto antibodies and rheumatic diseases

Objective: Systematic prospective study in six hundred and nineteen symptomatic woman with breast implants. Methods: To evaluate the symptoms and laboratory findings of patients with silicone breast implants. Results: The majority of the patients in our study presented with the atypical rheumatic syndrome (74,9 percent). A few with so called silicone disease. Various auto antibodies were found sometimes more than one in close to ten percent of the patients analysed. Conclusions: A systemic disease characterized by fatigue, cognitive dysfunction, muscle skeletal pain and the presence of auto antibodies in certain patients were observed in symptomatic brazilian woman with silicone breast implants.

Cellular immune response to retinal S-antigen & interphotoreceptor retinoid binding protein fragments in idiopathic human uveitis.

The role of retinal antigens in idiopathic human uveitis has been studied in 38 patients of uveitis, and 30 patients of systemic connective tissue disease (CTD) and 30 healthy volunteers. Lymphocyte proliferative responses were tested in vitro against native S-antigen, its uveitopathogenic peptides (peptide M, peptide G), yeast histone H3 peptide and uveitopathogenic fragment of interphotoreceptor retinoid binding protein (IRBP: R16) to establish their role in pathogenesis of human uveitis. Seven patients with uveitis, and none among CTD patients and healthy volunteers, responded (stimulation index > 3) to at least one retinal antigen used. One uveitis patient showed response to native S-antigen, peptide M and yeast histone H3. One responded to both S-antigen and peptide M and another responded to both peptide G and R16 peptide. Two responded to S-antigen only, one to peptide M and one to peptide G. In addition, one uveitis patient responded to yeast histone H3 only. These results suggest that retinal antigens may play a role in the etiopathogenesis of a subset of idiopathic human uveitis.

Clinical significance of anti-Sm antibody in systemic lupus erythematosus & related disorders.

A retrospective analysis was undertaken of the clinical and immunological profile of 33 anti-Sm antibody positive (group I) and 243 anti-Sm antibody negative (group II) patients with systemic connective tissue diseases including systemic lupus erythematosus and related disorders (overlap, mixed and undifferentiated connective tissue diseases). The disease patterns were comparable, except for cutaneous vasculitis and hypocomplementaemia (low serum C3 levels). Vasculitis was observed in 55 per cent of group I and 35 per cent of group II subjects (P less than 0.05). Hypocomplementemia was also detected more frequently (86%) in group I as compared to group II (66%) patients (P less than 0.05). No association was found between anti-Sm antibody and renal, neuropsychiatric and cardiopulmonary features. The study thus demonstrated the association of anti-Sm antibody with immune complex mediated vasculitis.

Autoanticuerpos en niños con enfermedades del tejido conjuntivo / Auto antibodies in children with connective tissue disease

El diagnóstico y clasificación de las enfermedades del tejido conjuntivo en los niños se ha basado principalmente en datos clínicos. Sin embargo, la adición de un número importante de hallazgos inmunológicos puede sustentar que en la infancia se presentan diferentes tipos y subgrupos de enfermedades autoinmunes. Los autoanticuerpos con diferentes especificidades definen no solo a distintos padecimientos sino también a diferentes subgrupos de pacientes con enfermedades del tejido conjuntivo. Esta revisión enfatiza que el factor reumatoide y los anticuerpos antinucleares son pruebas útiles que pueden ayudar a definir las características clínicas en niños con padecimientos reumáticos. El factor reumatoide de la clase IgM está presente frecuentemente en el grupo de pacientes con artritis reumatoide poliarticular, mientras que el grupo oligoarticular con uveitis tiene por características presentar una alta prevalencia de anticuerpos antinucleares. Los anticuerpos anti-DNA y anti-Sm son positivos en niños con lupus eritematoso generalizado. Los pacientes con dermatomiositis infantil tienen una menor frecuencia de anticuerpos contra los antígenos Jo-1 y PM-1, al compararlos con el grupo de polidermatomiositis del adulto. Los niños con esclerosis sistémica y síndome de CREST pueden mostrar anticuerpos anti-Scl-70 (topoisomerasa-1) y anticuerpos anti-centrómeto. Los títulos elevados de los anticuerpos anti RNpn (U1) parecen definir no sólo a la enfermedad mixta del tejido conjuntivo del adulto sino también a los niños. Los anticuerpos anti-SS-A (Ro) y anti-SS-B (La) han sido encontrados en pacientes con síndrome de Sjögren primario. Interesante otras anomalías, incluyendo a los anticuerpos antifosfolípidos, anti-proteínas hsp-70 y hsp-90, así como los anticuerpos contra productos contra oncogene-c-myc que han sido reportados en pacientes adultos con diferentes padecimietos del tejido conjuntivo, no han sido estudiados completamente en las enfermedades autoinmunes del niño. En suma los autoanticuerpos constituyen marcadores importantes que pueden ayudar a comprender lo complejo de la autoinmunidad en los niños.

Auto-anticorpos em doenças de tecido conjuntivo / Antibodies in diseases of connective tissue

No presente trabalho foi feita uma revisäo sobre os auto-anticorpos antinucleares e anticitoplasmáticos mais freqüentemente mencionados na literatura médico. Säo descritas as relaçöes entre estes anticorpos e as doenças sistêmicas do tecido conjuntivo objetivando o diangóstico. Também é apresentado um quadro geral focalizando cada auto-anticorpo, seu determinante antigênico (epítopo), o padräo de imunofluorescência pela técnica de IF indireta e outros métodos laboratoriais de pesquisa destes anticorpos e as doenças relacionadas com a presença deles

Antibodies to extractable nuclear antigens in connective tissue disorders in India: prevalence and clinical correlations.

Antibodies to Extractable Nuclear Antigens (ENAs) namely Sm, nRNP, SS-A and SS-B were studied in 397 patients with various connective tissue diseases (CTD), 146 patients with inflammatory polyarthropathies, 16 cases of systemic vasculitides, and 39 normal subjects using counterimmunoelectrophoresis and double immunodiffusion methods. Anti-ENA antibodies were positive in 40.8 percent cases of Systematic lupus erythematosus (SLE) (n = 191), 36.4 percent of overlap CTD (OCTD, n = 44), 27.8 percent of Sjogren's syndrome (n = 18), 10.6 percent of progressive systemic sclerosis (PSS, n = 66) and 2.7 percent of rheumatoid arthritis (n = 111) patients. The correlation of these antibodies with disease features was done. The significant finding was negative association of anti-nRNP antibodies (when present alone) with renal involvement. Anti-Sm antibodies did not correlate with any disease feature. The other associations included correlation of anti-nRNP with pulmonary parenchymal lesions, anti-SS-A with serositis and pulmonary hypertension, and anti-SS-B with myocarditis and recurrent diarrhoea. We conclude that Anti-ENAs may correlate with certain subsets of these diseases but the subject is controversial.

Antinuclear antibodies in Thai patients with connective tissue diseases.

A study of antinuclear antibodies (ANA) among Thai patients with various connective tissue diseases revealed that the prevalence of ANA was similar to that in other countries, but that the ANA patterns showed interesting contrasts in most diseases. Rather than the predominant homogeneous pattern seen elsewhere in systemic lupus erythematosus and rheumatoid arthritis, the speckled pattern was commonest among Thai patients with these two diseases (67.9% and 76.9% respectively). Patients with scleroderma exhibited a much lower percentage of the nucleolar pattern (17%) than reported elsewhere. The discrepancy between our findings and those from other studies may reflect differences in genetics, the environment or the severity of disease.

Complexos imunes circulantes e crioglobulinas / Circulating immune complexes and cryoglobulins

Em virtude de nuances existentes entre crioglobulinas (CRIO) e complexos imunes circulantes (ICC), avaliam-se as inter-relaçöes entre ambos em 160 pacientes; três com crioglobulinemia essencial, 84 com lúpus eritematoso sistêmico (LFS), 32 com vasculite, 11 com síndrome de Sjögren, 13 com doença reumatóide e 17 com nefropatias. Verificou-se que 10% dos portadores de crioglobulinemia essencial obviamente, apresentam crioglobulinas no soro e 90% têm ICC; 22% dos pacientes com LES mostran CRIO enquanto 50% têm ICC; 12,5% dos pacientes com vasculite revelam CRIO e 28% têm ICC; na síndrome de Sjögren, 27% têm crioglobulinas e 70% ICC; na doença reumatóide a CRIO aparece em 27% dos pacientes enquanto os ICC em 70% e nos indivíduos com acometimento renal primário, 25% mostram CRIO e 60% revelam ICC. Embora a freqüência de crioglobulinas seja menor que a de ICC, na maioria das doenças avaliadas (exceto na crioglobulinemia essencial), näo ocorreu associaçäo significante entre CRIO e ICC, exceto no LES (X = 10,11; gl:1; alfa = 0,05. As inter-relaçöes entre CRIO e ICC podem ser devidas à existência de fraçäo crioprecipitável nos ICC, ou pelo comportamento físico-químico das crioglobulinas

Anticorpos antinucleares, anti-SSA (Ro) e anti-SSB (La) nas doenças difusas do tecido conectivo / Antinuclear antibodies, anti-SSA (Ro) and SSB (La) in diffuse connective tissue diseases

O trabalho teve como objetivo determinar a freqüência de anticorpos anti-SSA(Ro) e anti-SSB(La) em DDTC no nosso meio. Foram estudados 52 pacientes portadores de lúpus eritematoso sistêmico (LES), 19 pacientes com síndrome de Sjögren (SS) primária e 13 portadores de SS secundária, além de 170 doentes com outras doenças difusas do tecido conectivo (DDTC), tais como: doença reumatóide (DRe), esclerose sistêmica progressiva (ESP), polimiosite/dermatomiosite (PM/DM), doença mista do tecido conectivo (DMTC), artropatias soronegativas, osteoartrose (OA), vasculites, LE discóide, lúpus droga-induzido e 30 indivíduos normais. Anticorpos (ACs) anti-SSA(Ro) foram encontrados em pacientes portadores de LES (15,4%) e SS primária (68,6%), näo tendo sido observado em nenhuma outra DDTC estudada, exceçäo feita a um caso de SS secundária (SS/LES) Da mesma forma ACs anti-SSB (La) foram observados apenas em pacientes com LES (9,6%) e SS primária (42,1%). A freqüência de ACs anti-SSA (Ro) e anti-SSB (La) foi significantemente maior no grupo de pacientes portadores de SS primária, quando comparada ao grupo de pacientes com LES

Atividade biologica do anticorpo anticentrômero. Capacidade de fixaçäo de complemento / Biological activity of anticentromere antibody. Complement fixation capacity

Anticorpo anticentrômero (AAC) foi detectado em oito de nove pacientes com síndrome de CREST, num estudo de 105 pacientes com doenças difusas do tecido conectivo (DDTC) e 60 normais, por técnica de imunofluorescência indireta em células HEp-2. Quatro destes soros foram avaliados quanto a capacidade de fixaçäo de complemento, pela mesma técnica, porém revelando a reaçäo com anti-Ciq e anti C, marcados com fluoresceína. Todos os soros mostraram positividade da reaçäo apenas com anti-C3. Apesar do ensaio sugerir fixaçäo de complemento pela via alternativa, näo foram observadas alteraçöes do nível sérico do complemento. O estudo seriado de uma paciente tratada com D. penicilamina, demonstrou negativaçäo deste auto anticorpo após seis meses de tratamento. O estudo familiar, deste caso, revelou a presença de AAC em uma das filhas, que apresentava apenas fenômeno de Raynaud. Esses dados sugerem que AAC pode ter um papel de importância relevante no diagnóstico, e possivelmente na monitorizaçäo destes pacientes