title: Immunoglobulin G4-related tubulointerstitial nephritis associated with interstitial pulmonary disease: Report of a case with review of literature.
abstract: 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.
authors.affiliation: Department of Nephrology, Christian Medical College, Vellore, Tamil Nadu, India.
authors.forename: M
authors.initials: M
authors.lastname: Saravanan
authors.affiliation: Department of Nephrology, Christian Medical College, Vellore, Tamil Nadu, India.
authors.forename: S
authors.initials: S
authors.lastname: Alexander
authors.affiliation: Department of Central Electron Microscopic Unit, Christian Medical College, Vellore, Tamil Nadu, India.
authors.forename: S M
authors.initials: SM
authors.lastname: Matthai
authors.affiliation: Department of General Pathology, Christian Medical College, Vellore, Tamil Nadu, India.
authors.forename: A
authors.initials: A
authors.lastname: Korula
authors.affiliation: Department of Nephrology, Christian Medical College, Vellore, Tamil Nadu, India.
authors.forename: S
authors.initials: S
authors.lastname: Varughese
authors.affiliation: Department of Nephrology, Christian Medical College, Vellore, Tamil Nadu, India.
authors.forename: V
authors.initials: V
authors.lastname: Tamilarasi
keywords: Corticosteroid therapy; immunoglobulin G4-related disease; immunoglobulin G4-related kidney disease; tubulointerstitial nephritis
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