Dados do Trabalho

Title

Systemic Lupus Erythematosus: Severe forms with good prognosis due a early diagnosis

Background

Systemic Lupus Erythematosus (SLE) is an autoimmune disease of unknown cause that affects several organs and systems, with general and specific symptoms of the affected organs. In Brazil, an estimated incidence of SLE is around 8.7 cases per 100,000 people per year, 9 to 10 times more common in women during reproductive age. Genetic and environmental factors may influence its appearance and loss of control of immune regulators, leading to the proliferation and development of autoantibodies, thus causing progressive cellular or tissue damage.

Case report

M.E.F.S, female, 27 years old. The patient was admitted to the hospital with weight loss of 5 kilos, lymphadenomegaly, dyspnea on major efforts and edema of the face and lower limbs. The investigation identified bilateral pleural effusion and pericarditis on chest tomography, nephrotic syndrome with proteinuria of 7.9 grams and anemia with hemoglobin of 10.5. The hypotheses of severe SLE or Lymphoma were raised. An inconclusive lymph node biopsy, Antinuclear Factor and AntiDNA-positive assays were performed. By gravity, pulse therapy with methylprednisolone was started, even without renal biopsy unavailable in the service. After this biopsy, performed then at the reference center, it was observed a diffuse proliferative glomerulonephritis associated to membranous component and multifocal tubular atrophy with fibrosis, initiating pulse therapy with cyclophosphamide. The patient evolved with five of the diagnostic criteria recommended by the American Society of Rheumatology consisting of malar erythema, discoid lesion, oral / nasal ulcers, arthritis, serositis, renal impairment, neurological, hematological, immunological and antinuclear antibodies. Severe lupus is manifested by such changes present in the patient in question, hence the need for immediate treatment even without the certainty of renal dysfunction confirmed by biopsy.

Conclusion

The diagnosis of SLE in its severe forms is still a challenge due to the different signs and symptoms varied and the lack of resources in smaller institutions. The patient's example demonstrates how a diagnosis and early treatment make possible a good prognosis with significant improvement of the clinical picture, since she did not need dialysis or other interventional treatment.