Acute Interstitial Nephritis and Uveitis with Bone Marrow Granulomas and Anti-IMeutrophil Cytoplasmic Antibodies

Abstract

Acute interstitial nephritis (AIN) and uveitis of unknown etiology developed in a 15-year-old girl. Symptoms and urinary abnormalities improved spontaneously but moderate renal dysfunction and anterior uveitis persisted. A repeat biopsy performed 2 years later showed focal tubulointerstitial changes, while bone marrow examination revealed granulomas. Simultaneously, anti-neutrophil cytoplasmic antibodies (ANCA) were detected, and decreases in T cell population and lymphocyte function were found. These immunological abnormalities normalized in parallel with clinical improvement with corticosteroid therapy, strongly suggesting that in some patients autoimmune disorders contribute to the pathogenesis of the disease.

Tubulointerstitial Nephritis and Uveitis Syndrome

“…1 Interestingly, noncaseating granulomata were found in a minority of renal biopsy specimens, and 6 patients have been reported to have granulomata on examination of bone marrow aspirates. [15][16][17][18][19] Hepatic granulomata have also been reported. [20][21][22] These granulomata were not found to have any fungi or acid-fast bacilli on histologic examination.…”

Bone marrow manifestations of infections and systemic diseases observed in bone marrow trephine biopsy

“… 12 In association with this last disease, an interesting syndrome has been described, characterized by bone marrow granulomas, interstitial nephritis and uveitis. 32–33 …”

Adult onset tubulointerstitial nephritis and uveitis syndrome

“…Several patients with TINU syndrome were found to have serological evidence of auto-antibodies, including antinuclear antibodies, 10 rheumatoid factor, 11 anti-DNA antibodies 12 and cytoplasmic antineutrophil cytoplasmic antibodies (cANCA). 13 The patient in the present case had proximal tubular dysfunctions (tubular glucosuria and proteinuria). A kidney biopsy confirmed acute tubulointerstitial nephritis.…”

“…In some patients with acute interstitial nephritis, no precipitating factor can be identified. Several patients with TINU syndrome were found to have serological evidence of auto‐antibodies, including antinuclear antibodies, 10 rheumatoid factor, 11 anti‐DNA antibodies 12 and cytoplasmic antineutrophil cytoplasmic antibodies (cANCA) 13 …”