A case of glomerulopathy showing podocytic infolding in association with Sjögren's syndrome and primary biliary cirrhosis.

A 49-year-old man was admitted to our hospital with mild proteinuria. Prior to admission, he had been diagnosed as having Sjögren's syndrome in association with primary biliary cirrhosis. Examination of a renal biopsy under light microscopy revealed diffuse and global mesangial cell proliferation and a spike and/or bubbling formation of the glomerular basement membrane (GBM), resembling membranoproliferative glomerulonephritis. In contrast, immunofluorescent studies showed marked immunoglobulin and complement depositions in the mesangial areas; however, only faint granular IgG and IgA deposition was observed along the GBM. Interestingly, electron microscopy revealed that a microtubular structure, derived from podocytes, was present in the GBM. We present a case of glomerulopathy showing podocytic infolding in association with Sjögren's syndrome and primary biliary cirrhosis.

A case of henoch-schönlein purpura nephritis in pulmonary tuberculosis.

A 38-year-old man with pulmonary tuberculosis developed purpura over both lower extremities and renal disturbance after starting antituberculosis treatment. A renal biopsy and skin biopsy were performed to diagnose the new clinical manifestations, and leukocytoclastic vasculitis with IgA and C3 deposition were detected. Henoch-Schönlein purpura nephritis (HSPN) was diagnosed on the basis of the clinical and pathologic findings, and prednisolone therapy was added. The skin lesions disappeared in 7 days after starting steroid therapy, and renal function gradually improved. These results suggested that the pathogenesis of HSPN might be the consequence of the deposition of the circulating immune complexes. The treatment of HSPN has been not established yet. We should consider how to use steroid therapy for HSPN and call attention to the recurrences of renal disturbance and pulmonary tuberculosis. It is thus recommended to follow patients with HSPN in tuberculosis for long periods.

[Interstitial pneumonia and nephritis with Sjögren's syndrome: successful treatment with corticosteroid therapy].

A Sixty eight-year-old man complained of shortness of breath on exercise since the spring of 2001. An abnormal lung shadow was pointed out on chest X-ray and progression of renal dysfunction and high gamma globulinemia were detected out on blood examination. He was admitted to the Department of Respiratory Disease in our hospital in March 2003, because of bilateral lower lung interstitial shadow, severe anemia (Hb 7.9 g/dl), and renal dysfunction (S-Cr 1.9 mg/dl). He was found to have hypergammaglobulinemia (IgG 2,997 mg/dl), positive RO/SS-A antigen, high serum KL-6 level (2,050 U/ml), and increased urinary excretion of beta2-microglobulin (beta2MG). Both Gum test and Schirmer test results were positive. Lip biopsy showed cell infiltration to the salivary glands and he was diagnosed as having Sjogren's syndrome. Renal biopsy showed diffuse interstitial cell infiltration and a Trans Bronchoscopic Lung Biopsy (TBLB) showed fibrotic thickness and lymphocyte infiltration in the alveolar septum. Accordingly, he was diagnosed as having Sjögren's syndrome complicated with both interstitial nephritis and interstitial pneumonitis. He was treated by high-dose corticosteroid therapy and anticoagulant heparin. His laboratory data showed that both serum KL-6 and urinary beta2MG were reduced. Chest CT showed remarkable improvement of the interstitial shadow. A second renal biopsy performed at ten weeks after the beginning of treatment showed remarkable improvement of the interstitial cell infiltration. This is a rare case of Sjögren's syndrome complicated with interstitial nephritis and interstitial pneumonitis, treated successfully with high-dose corticosteroid therapy. Both complications showed immediate improvement with high-dose corticosteroid therapy, suggesting that early steroid therapy is effective for both complications.