Dados do Trabalho

Title

OVERLAP SYNDROME OF SYSTEMIC LUPUS ERYTHEMATOSUS WITH POLYMYOSITIS AND SCLERODERMA.

Background

Connective tissue diseases are autoimmune and inflammatory diseases affecting different organs and systems. They are non-ethnic diseases, affecting mainly women in the ratio of 10:1, in the age group of 15-25 years. Such diseases may manifest clinically in an isolated way or in sequential superposition, with the main collagenoses being systemic lupus erythematosus (SLE), scleroderma, polymyositis, rheumatoid arthritis (RA) and Sjögren's syndrome. The objective of this article is to report the overlap of two other diseases in a patient with systemic lupus erythematosus (SLE).

Case report

A 27-year-old female patient, kitchen assistant, with a previous diagnosis of systemic lupus erythematosus (SLE) 3 years ago: arthritis, skin rash, Raynaud's phenomenon and serositis. Anti-DNA: positive 1/40, anti-SM: positive 480, anti-SSA: 240, anti-SSB: 23, Anticardiolipin IgG: positive, Anti-coagulant lupus: positive, Anti-RNP: 240. Were used Prednisone 20 mg/d and hydroxychloroquine 400 mg/d with lupus remission. Two years ago had cutaneous thickening in the upper limbs and leucomelanodermia in the lower limbs (FIGURE 1). However, one month ago, she started with incapacitating asthenia, proximal muscle weakness of the upper and lower limbs, accompanied by fever and CPK 8.000. Patient was hospitalized and presented with anemia, increased inflammatory tests (PCR: 49), increased transaminases (TGO: 348, TGP: 810), and muscle enzymes (CPK: 8.134, Aldolase: 52). Leptospirosis and Dengue negative. Immunology: Anti-JO1: negative, Anti-Mi2: negative, C3: 58, C4: 8, proteinuria of 1,599 mg/24h. Chest X-ray without changes. Chest tomography showing discrete pericardial effusion, with no other changes. Echocardiogram: absence of pericardial effusion and normal ejection fraction. Magnetic resonance imaging of the right shoulder showed a great deal of muscle inflammation, suggestive of polymyositis. Were performed pulse therapy with methylprednisolone 1g/d for 5 days showing improvement and good evolution.

Conclusion

This case is an overlap syndrome between scleroderma and polymyositis at confluence with lupus. Diagnosis of mixed disease of discarded connective tissue due to patient presenting antibodies specific to lupus. Thus, when faced with a patient who has characteristics of two or more distinct types of autoimmune diseases, must to remember the syndrome of overlap, so it must be treated according to the manifestations that the patient presents.

Área

Systemic Lupus Erythematosus