Anti-mitochondria antibody-related tubulointerstitial nephritis accompanied by severe hypokalemic paralysis

Abstract

A 47-year-old man presented with severe hypokalemic paralysis and respiratory failure. A large amount of potassium was administered along with providing intensive care, and his condition improved. Hypokalemia was attributed to increased urinary potassium excretion. A kidney biopsy was performed to make a definitive histological diagnosis. It revealed acute tubulointerstitial nephritis (TIN). After the diagnosis, prednisolone was administered, and the TIN gradually improved. From the clinical course and laboratory findings, the TIN was presumed to be an autoimmune disorder. Further specific autoantibody tests were positive for anti-mitochondrial antibody (AMA), which has been gaining increasing attention in regard to TIN. In addition, all previous cases of TIN associated with AMA have affected females. The detailed pathogenetic mechanisms are as yet unclear and require further investigation.

Relapsing Tubulointerstitial Nephritis with Antimitochondrial M2 Antibody Accompanied by Pulmonary Involvement

“…Furthermore, pulmonary involvement has not been reported in TIN with AMAs/IgM-positive plasma cells thus far (1)(2)(3)(4)(5)(13)(14)(15). The absence of Sjögren's syndrome, but a definite finding for PBC, TIN with AMA in this case suggested for the first time a possible association between lung involvement and TIN with AMAs.…”

“…The first case of tubulointerstitial nephritis (TIN) with primary biliary cholangitis (PBC) was reported in 1987 (1). Since then, cases of TIN with PBC and/or antimitochondrial antibodies (AMAs) have been reported to have similar clinical features of appearing predominantly in women, high serum IgM levels, AMA positivity, distal renal tubular acidosis, proximal renal tubular dysfunction, and responsiveness to glucocorticoid therapy (1)(2)(3)(4)(5).…”