[Recent Developments in Myositis Syndromes]. / Aktuelle Entwicklungen bei Myositis-Syndromen.

Idiopathic inflammatory myopathies (IIM) are a rare and clinically polymorphic and heterogenous group of myositis syndromes. Myositis is part of a systemic autoimmune disorder with various extramuscular manifestations affecting skin, lungs, joints, esophagus and other organ systems. Most myositis patients have autoantibodies against non organspecific antigens. More than 20 different autoantibodies are known. They are associated with different clinical phenotypes of adult or juvenile dermatomyositis or myositis-overlap syndromes and different genetic markers. Pure polymyositis, if not a monosymptomatic manifestation of a systemic disorder, so far has no marker antibody and is an exclusion diagnosis. Sporadic inclusion body myositis (sIBM) has no extramuscular manifestations, cN1A antibodies are directed against muscle-derived antigen and are not highly specific for IBM.Myositis syndromes differ in histopathology. Patients with anti-synthetase-syndrome frequently have necrotizing perifascicular myositis with myonuclear actin inclusions.New classification criteria have been developed by the European (EULAR) and American (ACR) rheumatology societies and different outcome measurements for clinical studies are now available.There is still a lack of controlled therapeutic trials. However there is good consensus that glucocorticosteroids (GC) are necessary and effective to treat active myositis usually in combination with methotrexate or azathioprine. Rituximab is effective in GC-resistant myositis as well as high dose i. v. immune globulin (IVIG) in certain conditions.

Vasoactive Therapy in Systemic Sclerosis: Real-life Therapeutic Practice in More Than 3000 Patients.

OBJECTIVE: Vasculopathy is a key factor in the pathophysiology of systemic sclerosis (SSc) and the main cause for Raynaud phenomenon (RP), digital ulcers (DU), and/or pulmonary arterial hypertension (PAH). It is so far unknown how patients with SSc are treated with vasoactive agents in daily practice. To determine to which extent patients with SSc were treated with different vasoactive agents, we used data from the German Network for Systemic Scleroderma registry. METHODS: The data of 3248 patients with SSc were analyzed. RESULTS: Patients were treated with vasoactive drugs in 61.1% of cases (1984/3248). Of these, 47.6% received calcium channel inhibitors, followed by 34.2% treated with angiotensin-converting enzyme (ACE) inhibitors, 21.1% treated with intravenous (IV) prostanoids, 10.1% with pentoxifylline, 8.8% with angiotensin 1 receptor antagonists (AT1RA), 8.7% with endothelin 1 receptor antagonists (ET1RA), 4.1% with phosphodiesterase type 5 (PDE5) inhibitors, and 5.3% with others. Patients with RP received vasoactive therapy in 63.3% of cases, with DU in 70.1%, and with PAH in 78.2% of cases. Logistic regression analysis revealed that patients with PAH were significantly more often treated with PDE5 inhibitors and ET1RA, and those with DU with ET1RA and IV prostanoids. In addition, 41.8% of patients were treated with ACE inhibitors and/or AT1RA. Patients registered after 2009 received significantly more often ET1RA, AT1RA, and IV prostanoids compared with patients registered prior to 2005. CONCLUSION: These data clearly indicate that many patients with SSc do not yet receive sufficient vasoactive therapy. Further, in recent years, a marked change of treatment regimens can be observed.

Disease progression in systemic sclerosis-overlap syndrome is significantly different from limited and diffuse cutaneous systemic sclerosis.

BACKGROUND: Systemic sclerosis (SSc)-overlap syndromes are a very heterogeneous and remarkable subgroup of SSc-patients, who present at least two connective tissue diseases (CTD) at the same time, usually with a specific autoantibody status. OBJECTIVES: To determine whether patients, classified as overlap syndromes, show a disease course different from patients with limited SSc (lcSSc) or diffuse cutaneous SSc (dcSSc). METHODS: The data of 3240 prospectively included patients, registered in the database of the German Network for Systemic Scleroderma and followed between 2003 and 2013, were analysed. RESULTS: Among 3240 registered patients, 10% were diagnosed as SSc-overlap syndrome. Of these, 82.5% were female. SSc-overlap patients had a mean age of 48±1.2 years and carried significantly more often 'other antibodies' (68.0%; p<0.0001), including anti-U1RNP, -PmScl, -Ro, -La, as well as anti-Jo-1 and -Ku antibodies. These patients developed musculoskeletal involvement earlier and more frequently (62.5%) than patients diagnosed as lcSSc (32.2%) or dcSSc (43.3%) (p<0.0001). The onset of lung fibrosis and heart involvement in SSc-overlap patients was significantly earlier than in patients with lcSSc and occurred later than in patients with dcSSc. Oesophagus, kidney and PH progression was similar to lcSSc patients, whereas dcSSc patients had a significantly earlier onset. CONCLUSIONS: These data support the concept that SSc-overlap syndromes should be regarded as a separate SSc subset, distinct from lcSSc and dcSSc, due to a different progression of the disease, different proportional distribution of specific autoantibodies, and of different organ involvement.

Frequency of disease-associated and other nuclear autoantibodies in patients of the German Network for Systemic Scleroderma: correlation with characteristic clinical features.

INTRODUCTION: In the present study, we analysed in detail nuclear autoantibodies and their associations in systemic sclerosis (SSc) patients included in the German Network for Systemic Scleroderma Registry. METHODS: Sera of 863 patients were analysed according to a standardised protocol including immunofluorescence, immunoprecipitation, line immunoassay and immunodiffusion. RESULTS: Antinuclear antibodies (ANA) were detected in 94.2% of patients. In 81.6%, at least one of the autoantibodies highly associated with SSc or with overlap syndromes with scleroderma features was detected, that is, anti-centromere (35.9%) or anti-topoisomerase I (30.1%), followed in markedly lower frequency by antibodies to PM-Scl (4.9%), U1-ribonucleoprotein (U1-RNP) (4.8%), RNA polymerases (RNAPs) (3.8%), fibrillarin (1.4%), Ku (1.2%), aminoacyl-transfer RNA synthetases (0.5%), To (0.2%) and U11-RNP (0.1%). We found that the simultaneous presence of SSc-associated autoantibodies was rare (1.6%). Furthermore, additional autoantibodies were detected in 55.4% of the patients with SSc, of which anti-Ro/anti-La, anti-mitochondrial and anti-p25/p23 antibodies were most frequent. The coexistence of SSc-associated and other autoantibodies was common (43% of patients). SSc-associated autoantibodies disclosed characteristic associations with clinical features of patients, some of which were previously not acknowledged. CONCLUSIONS: This study shows that five autoantigens (that is, centromere, topoisomerase I, PM-Scl, U1-RNP and RNAP) detected more than 95% of the known SSc-associated antibody responses in ANA-positive SSc patients and characterise around 79% of all SSc patients in a central European cohort. These data confirm and extend previous data underlining the central role of the determination of ANAs in defining the diagnosis, subset allocation and prognosis of SSc patients.

High frequency of corticosteroid and immunosuppressive therapy in patients with systemic sclerosis despite limited evidence for efficacy.

INTRODUCTION: In systemic sclerosis (SSc) little evidence for the effectiveness of anti-inflammatory and immunosuppressive therapy exists. The objective of this study was to determine the extent to which SSc patients are treated with corticosteroids and immunosuppressive agents. METHODS: Data on duration and dosage of corticosteroids and on the type of immunosuppressive agent were analyzed from 1,729 patients who were registered in the German Network for Systemic Scleroderma (DNSS). RESULTS: A total 41.3% of all registered SSc patients was treated with corticosteroids. Corticosteroid use was reported in 49.1% of patients with diffuse cutaneous SSc and 31.3% of patients with limited cutaneous SSc (P < 0.0001). Among patients with overlap disease characteristics, 63.5% received corticosteroids (P < 0.0001 vs. limited cutaneous SSc). A total 16.1% of the patients received corticosteroids with a daily dose >or= 15 mg prednisone equivalent. Immunosuppressive therapy was prescribed in 35.8% of patients. Again, among those patients with overlap symptoms, a much higher proportion (64.1%) was treated with immunosuppressive agents, compared with 46.4% of those with diffuse cutaneous SSc sclerosis and 22.2% of those with limited cutaneous SSc (P < 0.0001). The most commonly prescribed drugs were methotrexate (30.5%), cyclophosphamide (22.2%), azathioprine (21.8%) and (hydroxy)chloroquine (7.2%). The use of these compounds varied significantly between medical subspecialties. CONCLUSIONS: Despite limited evidence for the effectiveness of corticosteroids and immunosuppressive agents in SSc, these potentially harmful drugs are frequently prescribed to patients with all forms of SSc. Therefore, this study indicates the need to develop and communicate adequate treatment recommendations.

Clinical and immunogenetic features of patients with autoantibodies to asparaginyl-transfer RNA synthetase.

OBJECTIVE: We have previously described anti-KS autoantibodies and provided evidence that they are directed against asparaginyl-transfer RNA (tRNA) synthetase (AsnRS). The aim of the present study was to identify patients with anti-AsnRS autoantibodies and elucidate the clinical significance of this sixth antisynthetase antibody. In particular, we studied whether it was associated with the syndrome of myositis (polymyositis or dermatomyositis [DM]), interstitial lung disease (ILD), arthritis, and other features that had been previously associated with the 5 other anti-aminoacyl-tRNA synthetase autoantibodies. METHODS: More than 2,500 sera from patients with connective tissue disease (including myositis and ILD) and controls were examined for anti-AsnRS autoantibodies by immunoprecipitation (IP). Positive and control sera were tested for the ability to inhibit AsnRS by preincubation of the enzyme source with the serum. The HLA class II (DRB1, DQA1, DQB1, DPB1) alleles were identified from restriction fragment length polymorphism of polymerase chain reaction-amplified genomic DNA. RESULTS: Anti-AsnRS antibodies were identified in the sera of 8 patients (5 Japanese, 1 American, 1 German, and 1 Korean) by IP of the same distinctive set of tRNA and protein that differed from those precipitated by the other 5 antisynthetases, and these antibodies showed specific inhibition of AsnRS activity. Two of these patients had DM, but 7 of 8 (88%) had ILD. Four patients (50%) had arthritis, and 1 had Raynaud's phenomenon. This antisynthetase was very rare among myositis patients (present in 0% of Japanese myositis patients), but it was found in 3% of Japanese ILD patients. Thus, most patients with anti-AsnRS had chronic ILD with or without features of connective tissue disease. Interestingly, all 4 Japanese patients tested had DR2 (DRB1*1501/1502), compared with 33% of healthy controls. CONCLUSION: These results indicate that anti-AsnRS autoantibodies, like anti-alanyl-tRNA synthetase autoantibodies, have a stronger association with ILD than with myositis and may be associated with the DR2 phenotype.

Diagnosis and prognosis of early rheumatoid arthritis, with special emphasis on laboratory analysis.

Diagnosis of rheumatoid arthritis (RA) is mainly based on clinical criteria of symmetric polyarthritis of the hands and feet, with morning stiffness lasting usually more than 1 h. Autoantibodies typical for RA, i.e., rheumatoid factors and anti-cyclic citrullinated peptide, and measurements of inflammation add more specific information, especially for early diagnosis, where clinical presentation may be oligosymptomatic involving only a few joints. These laboratory parameters are also relevant for prognosis of disease persistence, functional impairment and radiological progression.

Healthcare and burden of disease in psoriatic arthritis. A comparison with rheumatoid arthritis and ankylosing spondylitis.

OBJECTIVE: To compare quality of life and treatment among patients with psoriatic arthritis (PsA), rheumatoid arthritis (RA), and ankylosing spondylitis (AS) treated by German rheumatologists. METHODS: Data for outpatients with PsA (n = 1863), RA (n = 9627), or AS (n = 1378) enrolled in the national database of the German collaborative arthritis centers in the year 2002 were analyzed. Among those with PsA, 2 subgroups with predominantly peripheral arthritis (n = 1612) and predominantly axial disease (n = 251) were distinguished. RESULTS: We found a high burden of illness in patients with PsA treated by rheumatologists. Among the 2 subgroups, those with axial PsA had worse outcomes (pain, function) than those with peripheral PsA. However, compared with RA and AS, physician ratings of disease activity and severity were lower in PsA. Concerning access to rheumatology care, there were similarities between AS and axial PsA, with very long disease duration at first visit (mean of about 6 yrs), versus RA and peripheral PsA, with shorter duration (1.6 and 2.5 yrs, respectively). A majority (84%) of patients with PsA were treated with disease modifying antirheumatic drugs. Thirty percent of the patients with PsA currently were under therapy with glucocorticoids, mainly (89%) with a dose < 7.5 mg. CONCLUSION: Patients with PsA seen in rheumatologic care have a burden of illness comparable to that of patients with RA or AS.

[The clinical spectrum of dermatomyositis]. / Das klinische Spektrum der Dermatomyositis.

Dermatomyositis is characterized by marked clinical heterogeneity. Many classification schemes are available, based on internal organ involvement, presence of circulating autoantibodies and additional symptoms characteristic for other connective tissue diseases. Viral infections and underlying malignancies are possible mechanisms to trigger this autoimmune disease. Intensive clinical investigations to detect such malignancies are therefore required. The prognosis of the individual patient depends on the involvement of internal organs and the characterization of circulating autoantibodies. There are patients with rapidly developing subsets of dermatomyositis and those with slowly progressive disease. Therefore treatment has to be adjusted for the disease severity. Sometimes high dosages of corticosteroids in combination with immunosuppressive agents or immunoglobulins are required.

Seasonal influence on the onset of idiopathic inflammatory myopathies in serologically defined groups.

OBJECTIVE: To assess possible seasonal patterns in the onset of polymyositis (PM) and dermatomyositis (DM). METHODS: The study group comprised 503 patients who met the criteria for probable or definite PM or DM and for whom detailed data on the time of myositis onset were available. Statistical analyses were performed using a Poisson model that assessed associations of ethnicity, sex, autoantibody presence, and month of onset of muscle weakness. RESULTS: There were no significant seasonal patterns of disease onset in myositis patients as a whole or in the total PM or DM populations. Significant seasonal associations were present, however, in the serologically defined groups. In the 131 patients with antisynthetase autoantibodies who were categorized as non-black, myositis onset peaked in March-April (P = 0.03). Among the antisynthetase-positive patients, the association was predominantly in those with PM (n = 85; P = 0.05) and in men (n = 51; P = 0.042). Patients with anti-signal recognition particle autoantibodies, however, did not have a significant seasonal onset, which is in contrast to previous findings. Patients without myositis-specific autoantibodies showed a significant peak in summer, with myositis onset in June-July (n = 252; P = 0.03); this seasonal association was significant in women (n = 182; P = 0.005), whereas there was no seasonal pattern in men (P = 0.9). CONCLUSION: These findings, in conjunction with other data, suggest that diverse environmental agents, acting upon different immunogenetic backgrounds, result in distinct immune responses and clinical syndromes in the idiopathic inflammatory myopathies. Our results emphasize the importance of considering more homogeneous disease groups, based on clinicopathologic features, immune responses, ethnicity, and sex, when attempting to decipher the pathogeneses of autoimmune disorders.

Development of a CENP-A/CENP-B-specific immune response in a patient with systemic sclerosis.

Antibodies directed against an epitope motif on CENP-A have been shown to cross-react with mimotopes on other autoantigens and on Epstein-Barr nuclear antigen 1 (EBNA-1), suggesting a molecular mimicry. We describe here the gradual development of an anticentromere immune response in a patient with systemic sclerosis, which started from an antihistone response and was not mediated by molecular mimicry. Via an epitope on histone H3, the antibody response spread to a homologous epitope in the H3 homology domain of CENP-A. This was followed by an intramolecular epitope spreading to N-terminal peptides of CENP-A containing the known epitope motif G-P-X(1)-R-X(2). From there it spread to corresponding epitopes on CENP-B and to mimotopes of the major CENP-A epitope motif on other autoantigens including EBNA-1. Whether the D-penicillamine treatment received by this patient was involved in the triggering of this cascade remains a matter of speculation.

Autoantibodies directed to novel components of the PM/Scl complex, the human exosome.

The autoantigenic polymyositis/scleroderma (PM/Scl) complex was recently shown to be the human homologue of the yeast exosome, which is an RNA-processing complex. Our aim was to assess whether, in addition to targeting the known autoantigens PM/Scl-100 and PM/Scl-75, autoantibodies also target recently identified components of the PM/Scl complex. The prevalence of autoantibodies directed to six novel human exosome components (hRrp4p, hRrp40p, hRrp41p, hRrp42p, hRrp46p, hCsl4p) was determined in sera from patients with idiopathic inflammatory myopathy (n = 48), scleroderma (n = 11), or the PM/Scl overlap syndrome (n = 10). The sera were analyzed by enzyme-linked immunosorbent assays and western blotting using the affinity-purified recombinant proteins. Our results show that each human exosome component is recognized by autoantibodies. The hRrp4p and hRrp42p components were most frequently targeted. The presence of autoantibodies directed to the novel components of the human exosome was correlated with the presence of the anti-PM/Scl-100 autoantibody in the sera of patients with idiopathic inflammatory myopathy (IIM), as was previously found for the anti-PM/Scl-75 autoantibody. Other clear associations between autoantibody activities were not found. These results further support the conception that the autoimmune response may initially be directed to PM/Scl-100, whereas intermolecular epitope spreading may have caused the autoantibody response directed to the associated components.

Artrite psoriásica: manifestações clínicas e associações com o lócus B do sistema HLA / Psoriatic arthritis: clinical aspects and associations with HLA-B loci

Introdução - A artrite psoriásica (AP) é doença freqüente no consultório reumatológico, eventualmente com dificuldades diagnósticas pela ausência de acomentimento cutâneo. O diagnóstico tem auxílio na classificação proposta por Moll & Wright e, em alguns casos, na associação com antígenos HLA classe I. Objetivos - Verificar concomitância ou naõ de início do quadro cutaneo/articular, observar a prevalêncai de fator reumatóide (FR) na doença e identificar antígenos HLA-B associados a subtipos específicos de AP. Material e Métodos - Avaliação retrospectiva de 168 pacientes consectivos com com AP, caucasianos, 109 homens e 59 mulheres, com idade média de 47 mais ou menos 12 anos (20-78 anos). Diagnóstico feito pela classificação de Mol & Wright modificada. FR foi analisado por nefelometria. Tipagem do HLA classe I, lócus B, foi realizada em duplicata em 122 pacientes e 94 controles caucasianos por microlinfocitotoxicidade (terasaki et al.). Resultados - A média de idade no início da doença foi de 33 mais ou menos 16 anos. O comprometimento cutâneo precedeu o articular em 94 pacientes (66 por cento), foi simultâneo em 15 (10 por cento) e em 34 pacientes (24 por cento) as manifestaões articulares precederam os achados de pele. A história familiar para psoríase foi positiva em 30 pacientes (18 por cento). Fator reumatóide IgM foi encotnrado em 20 casos (12 por cento), apenas 5 deles com idade superior a 60 anos (p=0,04). As formas poliarticular assimétrica ou simétrica foram as mais frequentes (36 e 31 por cento, respectivaemtne). HLA-B27 foi encontrado em 30 por cento dos casos, B27 em 26 por cento e B13 em 20 por cento, frequências significativas quando comparadas com o grupo controle. Conclusões - 1) A classificação vigente de Moll & Wright é insuficiente para enquadrar de forma adequada todos os pacientes com AP; 2) O comprometimento cutâneo precedeu o articular na maioria dos casos (66 por cento); 3) As formas poliarticular assimétrica e simétrica foram as mais frequentes (67 por cneto); 4) A presença do fator reumatóide em 12 por cento dos casos ocorreu de forma independente da idade dos pacientes; 5) HLA-B13, B17 e B27 foram os antígenos mais frequentemtne encontrados em associação com as formas poliarticulares, com o HLA-B27 associando-se à espondilite

Estudo clínico de pacientes portadoras de anticorpos anti-SS-A/Ro e/ou anti-SS-B/La / Clinical study of patients bearing of anti-SS-A/Ro and/or anti-SS-B/La antibodies

É feito estudo retrospectivo de 84 pacientes escolhidos aleatoriamente, 82 do sexo feminino e dois do masculino, brasileiros e com diferentes doenças do tecido conjuntivo, nos quais é pesquisada a presença e a freqüência de anticorpos contra antígenos Ro/SS-A e La/SS-B e sintomatologia clínica. Dos 84 soros pesquisados, 20 foram positivos às pesquisas desses anticorpos. Eram 14(70 por cento) com diagnóstico de lúpus eritematoso sistêmico (LES):11 com SS-A, três com SS-A e nenhum com apenas SS-B. Três (15 por cento) tinham diagnóstico de doença mista do tecido conjuntivo (DMTC): um com anti-SS-A, um com anti-SS-B, e um com ambos. Três (15 por cento) eram portadores de síndrome de Sjögren (SS) primária, todos com ambos os anticorpos. Fez-se estudo clínico desses pacientes quanto à secura de mucosas: xeroftalmia, xerostomia e secura vulvar. Nove (45 por cento) dos pacientes com anticorpos positivos säo sintomáticos: seis lúpicas, um com DMTC e dois com SS primaria. Conclui-se pela inexistência de sintomas relativos à secura de mucosas em mais da metade dos pacientes com doença do tecido conjuntivo que säo portadores de anticorpos ant-SS-A ou SS-B

Clínica e diagnóstico precoce da esclerose sistêmica: estado atual do tema

A esclerose sistêmica é doença reumatológica que envolve pele e órgäo viscerais, com quadro clínico dominado por processos de fibrose, oclusäo vascular e isquemia. Três formas clínicas säo reconhecidas: uma forma cutânea localizada (lineaer, em placas), uma difusa (com acometimento do tronco e rápida progressäo visceral) e uma forma limitada, com fenômenos englobados sob a denominaçäo CREST (calcinose, Raynaud, esôfago, esclerodactilia e teleangiectasias). O diagnóstico é baseado em critérios testados pela Associaçäo Reumatológica Americana, com sensibilidade acima de 90% e especificidade de 99%: esclerodermia proximal, esclerodactilia, lesöes cicatriciais de polpas digitais e fibrose pulmonar bibasilar. O diagnóstico precoce é difícil, mas de importância para o estabelecimento de terapêutica visando evitar o surgimento de complicaçöes tardias. Baseia-se em provas sorológicas, com demonstraçäo de auto-anticorpos por técnicas imunológicas, na microscopia capilar de pregas ungueais e na demonstracäo de alteraçöes da motricidade esofágica pela manometria ou cintilografia quantitativa

Doença mista do tecido conjuntivo: estudo clínico e laboratorial em 15 casos / Mixed connective tissue disease: clinical and laboratory study of 15 cases

Os autores fazem um estudo retrospectivo de 15 pacientes com diagnóstico de doença mista do tecido conjuntivo. Säo pacientes brasileiras, todas do sexo feminino, de 22 a 56 anos. Os sinais e sintomas mais freqüentes em nossa casuística säo: poliartrite 14(93%), dedos "em salsicha" 11(73%), fenômeno de Raynaud 11 (73%), rigidez matinal 7 (47%), febre 7 (47%), fraqueza muscular 8 (53%), acometimento digestivo (epigastralgia, pirose, gastrite, úlcera oral e perianal, peritonite, disfagia) 7 (47%), acometimento pleuropulmonar (dor torácica, dispnéia e derrame pleural) 6 (40%), rash cutâneo 5 (33%), vasculite 5 (33%), acometimento cardíaco (precordialgia, taquicardia, pericardite, derrame pericárdico e insuficiência cardíaca congestiva 5 33%). Os achados laboratoriais mostraram: FAN positivo em 100% dos casos, com padräo predominantemente pontilhado 13 (87%). AntiDNA nativo presente em 4 pacientes (27%), anti-RNP positivo em todos os soros testados. O fator reumatóide IgM foi positivo em 4(27%), Sm 5/15 (33%), SSA 1/14 (7%), SSB 2/15 (13%). IgG aumentada em 14/14 (100%). IgM aumentada em 9/14 (64%), IgA aumentada em 5.14 (36%). C3 reduzido em 7/14 (50%), C4 reduzido em 2/14 (14%)

Pesquisa de anticorpos anti DNA nativo pelos métodos de radioimunoensaio e da Crithidia luciliae: estudo comparativo

Anticorpos contra DNA de dupla hélice säo achado característico em pacientes com lúpus eritematoso sistêmico (Les. Várias técnicas säo descritas para demonstrar anticorpos antiDNA nativo. No Brasil, essa pesquisa vem sendo habitualmente realizada utilizando-se como substrato a Crithidia luciliae, que tem cinetoplasto rico em DNA nativo. A pesquisa por radioimunoensaio de Farr näo tem sido feita em nosso meio. Foram examinados soros de 82 pacientes de Belo Horizonte com diagnóstico preestabelecido: 65 com LES, 14 com doença mista de tecido conjuntivo (DMTC),dois com síndrome de Sjögren primária (SS) e um com esclerose sistêmica progressiva (ESP); a idade de 32 meses a 70 anos. Neste estudo, empregaram-se as duas técnicas, analisanbdo comparativamente sua sensibilidade e especificidade. Os testes foram executados no Instituto de Pesquisas Reumatológicas de Aachen (alemanha). Os resultados obtidos foram analisados como concordantes (positivo ou negtivo nas duas técnicas) e discordantes (positivo em uma e negativo em outra técnica). Oitenta e quatro por cento dos resultados säo concordantes e os discordantes säo distribuidos em 8,5% positivos ao radioimunoensaio. Dos 33 resultados positivos por um e/ou outro método, 29 pacientes (88%) têm diagnóstico de LES, e os outros quatro pacientes (12%) têm DMTC. Esses dados demonstram alta especificidade para o LES das duas técnicas e sensibilidade estatisticamente iguais em doenças do tecido conjuntivo