CASE REPORT |
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Year : 2015 | Volume
: 25
| Issue : 2 | Page : 113-116 |
Immunoglobulin G4-related tubulointerstitial nephritis associated with interstitial pulmonary disease: Report of a case with review of literature
M Saravanan1, S Alexander1, SM Matthai2, A Korula3, S Varughese1, V Tamilarasi1
1 Department of Nephrology, Christian Medical College, Vellore, Tamil Nadu, India 2 Department of Central Electron Microscopic Unit, Christian Medical College, Vellore, Tamil Nadu, India 3 Department of General Pathology, Christian Medical College, Vellore, Tamil Nadu, India
Correspondence Address:
Dr. S Alexander Department of Nephrology, Christian Medical College, Ida Scudder Road, Vellore - 632 004, Tamil Nadu India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0971-4065.136886
Immunoglobulin G4-related disease (IgG4-RD) is an emerging clinicopathological entity. Renal involvement is dominated by tubulointerstitial nephritis (TIN) with IgG4-positive plasma cells and fibrosis. IgG4-RD commonly affects middle-aged to elderly men with accompanying extra-renal lesions such as sialadenitis, lymphadenopathy, or type 1 autoimmune pancreatitis, all of which respond favorably to corticosteroid therapy. The disease burden of IgG4-related kidney disease (IgG4-RKD) in India remains largely underestimated. We report a case of IgG4-RKD manifesting as TIN associated with interstitial pulmonary disease, illustrating typical clinico-pathologic, serologic, immuno-histochemical, and ultrastructural features of this condition. In view of potential amelioration of renal dysfunction with appropriate therapy, the need for awareness of this condition and early diagnosis is highlighted.
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