1/7. Tubulointerstitial nephritis and uveitis syndrome with autoantibody directed to renal tubular cells.The pathogenesis of tubulointerstitial nephritis and uveitis (TINU) syndrome remains unknown, but T cell-mediated immune response has been postulated to play a role. On the other hand, TINU syndrome is characterized by hypergammaglobulinemia and high serum immunoglobulin g (IgG) levels, suggesting an involvement of humoral immunity. We describe a case of TINU syndrome in a 13-year-old girl with multiple tubular dysfunctions including renal glucosuria, tubular proteinuria, phosphaturia, uricosuria, and concentrating and acidifying defect. IgG antibody from her serum was reactive against 125-kDa human kidney protein. Immunofluorescence study using mouse kidney revealed that the antibody was against cortical renal tubular cells. The antibody disappeared as the renal symptoms resolved. We suggest that IgG antibody may contribute to tubular dysfunction in some patients with TINU syndrome.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
2/7. Acute eosinophilic interstitial nephritis and renal failure with bone marrow-lymph node granulomas and anterior uveitis. A new syndrome.We describe two patients with a unique granulomatous syndrome who presented with renal failure secondary to diffuse eosinophilic interstitial nephritis. Both had bilateral anterior uveitis, bone marrow granulomas, hypergammaglobulinemia and an increased sedimentation rate. One patient had lymph node granulomas and an immunoglobulin g (IgG) rheumatoid factor. An extensive investigation for an etiologic agent was unrewarding, and neither patient could be placed into any existing diagnostic category. Over a period of 2 years both patients have experienced improved renal function and dissolution of their bone marrow granulomas.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
3/7. Tubulointerstitial nephritis with uveitis: clinico-pathological and immunological study.A 10-year-old boy was evaluated for fever, weight loss, uveitis, normocytic, normochromic anemia, renal insufficiency, and hypergammaglobulinemia of 8 weeks' duration. Infectious and neoplastic causes of fever were excluded. A renal biopsy performed in the 4th week of disease revealed diffuse plasmocytic interstitial nephritis. No treatment was prescribed and the patient was transferred to another hospital. Because clinical symptoms and renal insufficiency were still present, in the 8th week of disease a second biopsy was performed, which showed lympho-monocytic interstitial nephritis. At the same time, phenotypic analysis of peripheral blood mononuclear cells was carried out, revealing a significantly decreased number of CD3( ), CD4( ), and CD3( )/CD8( ) cells, increased non-T CD3(-)/CD8( ) and CD56( ) NK cells, and decreased "naive" (CD45RA( )/CD4( )) and memory (CD45RO( )/CD8( )) T lymphocytes. A 6-month course of oral prednisone was prescribed. Clinical symptoms and laboratory findings quickly returned to normal values. After 13 days of corticosteroid therapy, a second phenotypic analysis of peripheral blood mononuclear cells was performed, which revealed normalization of CD3( ), CD4( ), and CD3( )/CD8( ) cells as well as proportions of non-T CD8( ) and CD56( ) NK lymphocytes, "naive" and memory cells. This case shows spontaneous evolution of tubulointerstitial infiltrates from plasmacytic to lympho-monocytic, profound disturbances of the immunological system, and the beneficial effect of corticosteroids on both the clinical course and immunological disturbances.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
4/7. Acute interstitial nephritis with polyclonal B cell infiltration and development of mantle cell lymphoma.A 69-year-old man with proteinuria, hematuria and abnormal renal and hepatic function developed acute renal failure. His clinical and laboratory examinations showed splenomegaly, edema, anemia and polyclonal hypergammaglobulinemia with high IgG levels. Renal biopsy showed interstitial nephritis with predominant B cell and plasma cell infiltration without monoclonality. After corticosteroid treatment, the patient showed remission in clinical symptoms and laboratory findings. However, one year later, he developed mantle cell lymphoma (MCL) with typical lymphocyte markers of cyclin d1 and CD5. When evaluated retrospectively, this patient appeared to have multicentric Castleman's disease initially and developed MCL later.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
5/7. A case of tubulointerstitial nephritis and uveitis syndrome with severe immunologic dysregulation.Idiopathic tubulointerstitial nephritis and uveitis (TINU) syndrome is an uncommon condition, characterized by acute tubulointerstitial nephritis (tin) with a favorable course and uveitis with a chronic relapsing course. The pathogenesis remains unclear, but a lymphocyte-mediated immune mechanism has been suggested. A 9-year-old boy was evaluated for fatigue and 2 kg of weight loss. Renal glucosuria, elevated urine beta(2)-microglobulin (MG), progressive renal dysfunction, polyclonal hypergammaglobulinemia, various autoantibodies and abnormal lymphocyte phenotypes were found. A renal biopsy revealed acute tin. After 2 months of treatment with prednisolone, renal function and polyclonal hypergammaglobulinemia were normalized. While tapering prednisolone, anterior uveitis developed, which was improved with topical steroid. But abnormal lymphocyte phenotypes and autoantibodies persisted on low-dose prednisolone. uveitis became aggravated, and urine beta(2)-MG increased again. The second renal biopsy (7 months later) was normal except for minimal focal interstitial fibrosis. uveitis was not responsive to systemic steroids, but improved with additional cyclosporin. Abnormal lymphocyte phenotypes improved, and most autoantibodies disappeared. We report a rare case of idiopathic TINU syndrome with severe immunologic dysregulation, which correlated with the clinicopathological and biochemical parameters. The information about lymphocyte phenotypes and autoantibodies may provide more insight into the pathophysiology and the clinical course of uveitis in this rare disorder.- - - - - - - - - - ranking = 2keywords = hypergammaglobulinemia (Clic here for more details about this article) |
6/7. A case of Mikulicz's disease complicated with interstitial nephritis successfully treated by high-dose corticosteroid.A 40-year-old woman who had bilateral swelling in the eyelids and submandibular region was admitted. Clinical findings suggested she had primary sjogren's syndrome. Laboratory data showed glucosuria, positive CRP (0.50 mg/dl), liver dysfunction (AST 53 U/l, ALT 101 U/l, gamma-GTP 241 U/l, ALP 914 U/l, LAP 496 U/l), hyperglycemia, hypergammaglobulinemia (IgG 3450 mg/dl, IgA 91 mg/dl, IgM 80 mg/dl), hypocomplementemia (C3 73 mg/dl, C4 2 mg/dl, CH50 < 19.0 U/ml), renal tubular dysfunction (urine N-acetyl-beta-D: -glucosaminidase 8.6 U/l, urine (beta2)-microglobulin 83 microg/l), and urinary concentration defect. ammonium chloride loading test was normal. gallium-67 scintigram indicated abnormal uptake in bilateral lacrimal glands, submandibular glands, and kidneys. A diagnosis of Mikulicz's disease and interstitial nephritis was made, since biopsy specimens of her lacrimal gland and minor salivary gland showed diffuse infiltration of lymphocytes. Renal biopsy specimens also showed severe interstitial infiltration of lymphocytes. Symptoms and laboratory data normalized in response to methylprednisolone pulse therapy and prednisolone 60 mg/day. This case of Mikulicz's disease complicated with interstitial nephritis was successfully treated by high-dose corticosteroid.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
7/7. Acute interstitial nephritis with symmetric enlargement of the lacrymal and salivary glands and systemic lymphadenopathy.We describe a 40-year-old man who developed symmetric enlargement of the lacrymal and salivary glands and systemic lymphadenopathy. Laboratory findings included eosinophilia, polyclonal hypergammaglobulinemia, elevated circulating immune complexes, hypocomplementemia, and renal insufficiency. The lymph node pathology was atypical of angioimmunoblastic lymphadenopathy with disproteinemia (AILD), in that it lacked a prominence of arborizing small vessel proliferation. Despite extensive examinations, the cause of the atypical AILD process has not been identified. However, a flow-cytometric analysis of immunophenotypes of lymphoid cells from the lymph node revealed a predominance of activated helper/inducer T cells, indicating a hyperimmune state. Acute interstitial nephritis was secondary to the renal propagation of the atypical AILD process. Thus, atypical AILD can be a new cause of acute interstitial nephritis. A course of combined steroids and immunosuppressive treatment resolved those pathologic disorders for 5 years.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |