Tubulointerstitial Nephritis and Primary Biliary Cirrhosis with a T Cell-dominant Profile of Infiltrating Cells and Granulomas in Both Organs

Abstract

A 46-year-old woman was admitted to our hospital for an evaluation of progressive renal insufficiency and elevated liver enzymes. A renal biopsy revealed chronic granulomatous interstitial nephritis. Her laboratory findings indicated primary biliary cirrhosis (PBC), which was confirmed with a liver biopsy. CD4 + T cells and CD8 + T cells with granuloma formation were the predominant cells infiltrating into the interstitium of the kidneys and liver. The etiology of tubulointerstitial nephritis in the present patient was not clear; however, it might have shared the same pathogenesis as PBC due to the relatively close onset, the similar profiles of infiltrating cells and the presence of granulomas.

IgM-Positive Tubulointerstitial Nephritis Associated With Asymptomatic Primary Biliary Cirrhosis

“… 8 There were 16 similar cases, including our own ( Table 2 ). 1 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 All 16 patients were female, and 11 were diagnosed with Fanconi syndrome. Of the 16 patients, 12 did not have osteomalacia.…”

“… 12 2012 46/F NA NA NA 2.3 NA NA 209 252 NA (+) (+) x x Iwakura et al. 13 2013 46/F 20,975 11.1 NA 1.1 38 50 694 190 966 160 143 x x Rasolzadegan et al. 14 2014 28/F NA NA NA 2 185 138 NA 698 NA (−) 19.5 x x Yamaguchi et al.…”

“… 12 2012 40 5 mo NA No Iwakura et al. 13 2013 NU NA NA NA Rasolzadegan et al. 14 2014 NA 2 yr NA No Yamaguchi et al.…”

Tubulointerstitial Nephritis with IgM-Positive Plasma Cells

“…In addition, concomitant Fanconi syndrome has also been reported, [11][12][13][14][15] and in most past reports the renal involvement was histologically diagnosed as TIN. 5,11,[13][14][15][16][17][18][19][20][21] In this paper, we report the novel clinical and morphologic features of 13 patients diagnosed with TIN and IgM-positive plasma cell infiltration, which we term "IgM-positive plasma cell-tubulointerstitial nephritis" (IgMPC-TIN).…”

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

“…In such cases, a high prevalence of elevated serum IgM levels, proximal and distal RTA, and AMA-positivity has been observed [2]. AMA is increasingly being considered as a cause of TIN [2,[8][9][10][11][12][13][14][15][16][17][18][19][20][21], but the details remain to be elucidated. In a few reports of TIN with AMA, patients have had subclinical PBC, indicating that the severity of PBC does not always correlate with that of the TIN [11][12][13].…”