RESUMO
We describe a case of IgA nephropathy (IgAN) followed by pulmonary hemorrhage associated with Henoch-Schöenlein purpura (HSP) in an adult female. The patient had a history of renal insufficiency and persistent hematuria and proteinuria, without any extra-renal involvement. She was diagnosed with IgAN 7 years before the onset of HSP and had received immunosuppressive therapy for 6 years. One year after discontinuing oral prednisolone and mizoribine, she suffered a pulmonary hemorrhage. She presented with exacerbated urinary findings, and palpable purpura, resulting in the diagnosis of HSP. Intravenous pulse methylprednisolone followed by oral prednisolone (1 mg/kg/day) and a monthly intravenous cyclophosphamide pulse resolved the pulmonary hemorrhage. In a review of 36 HSP patients complicated with pulmonary hemorrhage, 27.8 % of the patients perished [Rajagopala et al., Semin Arthritis Rheum 42:391-400, 1]. While the most efficient therapeutic strategies for these patients have yet to be determined, we speculate that an aggressive therapy of pulse methylprednisolone combined with immunosuppression agents is likely to bring about the best outcome in cases with pathological conditions similar to our patient's. On the other hand, discontinuance of immunosuppressive therapy might have resulted in the aggravation of the disease, hence we should examine patients carefully not to miss the cue.
RESUMO
It has been reported that glomerulosclerosis with IgA deposition is likely to be complicated with alcoholic liver cirrhosis. On the other hand, it is said that complications of nephrotic syndrome or rapidly progressive glomerulonephritis (RPGN) are relatively rare. We experienced two patients with alcoholic liver cirrhosis complicated with RPGN syndrome who had obtained favorable outcomes through the use of steroids and immune system suppressors. Case 1 was a 55-year-old male. He was being treated for alcoholic liver cirrhosis, but as bloody urine was noticed macroscopically, his renal function rapidly decreased. Specimens from a renal biopsy showed endocapillary proliferative lesions accompanying necrotic lesions. Granular deposition of IgA (IgA1) and C3 was seen along the capillary walls and in the mesangial areas. After the combined treatments of bilateral palatotonsillectomy, three courses of steroid semi-pulse therapy and post-therapy with steroids and mizoribin (MZR)were started, his hematuria and proteinuria disappeared and renal function improved markedly. Case 2 was a 37-year-old male with alcoholic liver cirrhosis complicated with hepatic encephalopathy. Although he was being treated at another hospital, nephritic syndrome occurred with rapidly worsening renal function and massive ascites. After continuous drainage of the ascites, we performed a renal biopsy. Mild proliferative lesions and notable wrinkling, thickening and doubling of the basal membrane were seen. Crescent formations were found in about half of the glomeruli. The fluorescent antibody technique showed positive pictures of IgA (IgA1) and C3. When three courses of steroid semi-pulse therapy and post therapy with steroids and MZR were combined, his proteinuria and serum Cre level decreased and stagnated ascites markedly decreased. The two cases were diagnosed as having secondary IgA nephropathy induced by the deposition of the IgA1 derived mainly from the intestinal tract, which had increased in the blood due to alcoholic liver cirrhosis. Active use of immune system suppressor therapy was effective.
