Poliangiítis microscópica: reporte de un caso con enfermedad renal, pulmonar y neurológica, y revisión de la literatura / Microscopic Polyangiitis: a case report with renal, pulmonary and neurological involvement and review of the literature

Informamos el caso de un hombre de 79 años con hemorragia alveolar, glomerulonefritis necrosante y polineuropatía por poliangiítis microscópica, una enfermedad autoinmune, caracterizada por vasculitis sistémica, predominantemente de vasos de pequeño calibre. Se revisan los aspectos clínicos y terapéuticos de la enfermedad.

We report the case of a 79 years-old male, with alveolar hemorrhage, necrotizing glomerulonephritis and polyneuropathy due to microscopic polyangiitis, an autoimmune disease, characterized by systemic vasculitis, predominantly of small vessels. We review the clinical and therapeutic aspects of the disease.

A Case of Microscopic Polyangiitis with Pulmonary Hemorrhage and Rapidly Progressive Glomerulonephritis

Microscopic Polyangiitis(MPA) belongs to a spectrum of systemic vasculits, and particularly antineutrophil cytoplasmic autoantibodies(ANCA)-associated small-vessel vasculitis which is characterized by involvement of the lung and kidney. The diagnosis of MPA is often difficult to make, and delayed because of the variability of the clinical presentation. Renal biopsies have a very important diagnostic and prognostic value in MPA. We experienced a case of microscopic polyangiits which was confirmed by renal biopsy and positive serum perinuclear ANCA, associated with alveolar hemorrhage and gastrointestinal bleeding. We began methylprednisolone pulse therapy, combined with a low dose of cyclophosphamide and plasmapheresis therapy. ACE inhibitor and Ca channel blocker were used when proteinuria and hypertention developed. On admission, the patient's lab findings showed BUN 117 mg/dL, Cr 2.3 mg/dL, while on the 60th hospital day BUN/Cr values fell to 20.8 mg/dL / 1.6 mg/dL and though proteinuria persisted, the patient's condition was tolerable and is currently under observation on an out-patient basis. The last lab values were BUN 26 mg/dL / Cr 1.6 mg/dL.

A case of prophylthiouracil associated ANCA positive acute glomerulonephritis / 대한내과학회지

Vasculitis is a rare complication of antithyroid drugs. Recently it was reported in association with ANCA. In most cases, the ANCA was specific for myeloperoxidase. The glomerulonephritis was key clinical manifestation in majority of cases. When antithyroid drugs were discontinued, clinical disease generally improved and ANCA titers were tended to fall. We experienced a 44 year old woman who presented with gross hematuria and generalized edema, after she had been treated with prophylthiouracil for 6 week for thyrotoxicosis. Renal biopsy was done, which showed mesangial proliferative glomerulonephritis. Indirect immunoflurorescence staining showed highly positive perinuclear pattern of ANCA in her serum. Prophylthiouracil associated ANCA positive glomerulonephritis was suspected. After cessation of prophylthiouracil and administration of prednisolone, renal function recovered gradually and ANCA titers were reduced. We hereby report a case of prophylthiouracil associated ANCA positive glomerulonephritis with a brief review of literature.