IgG4-related tubulointerstitial nephritis: A prospective analysis.
OBJECTIVE
Immunoglobulin-G4 (IgG4)-related tubulo-interstitial nephritis (IgG4TIN) could be the first presentation of IgG4-related systemic disease. Most of the data is from the West or Japan and retrospective, with good patient outcome.


METHODS
This study was carried out from April 2011 to July 2013. We report a prospective follow-up of 11 patients who presented with renal dysfunction and had histological diagnosis of IgG4TIN followed for a minimum period of 1 year or until end-stage renal disease.


RESULTS
IgG4TIN constituted 0.28% of total renal biopsies and 6.5% of all tubulointerstitial nephritis. Patient ages ranged between 21 and 71 years with a male predominance. All the patients had renal dysfunction at presentation with a mean serum creatinine of 5.12 mg/dL. Proteinuria was subnephrotic except when there was coexisting membranous glomerulonephritis (36.4%). The mean 24-h urine protien excretion was 1.8 g. Serum IgG4 levels were elevated in 10 (90.9%) patients. Ten (90.9%) patients had renomegaly and one (9.1%) had focal renal mass. Extra-renal manifestations were present in seven (63.6%). Renal histology showed pattern A in five (45.5%), pattern B in four (36.3%) and pattern C in two (18.1%) patients. All but one patient (90.9%) received immunosuppressive therapy. Four (36.3%) achieved complete remission and three (27.2%) progressed to end stage renal disease. Two patients died due to infections while on steroid therapy. One patient with a mass had end stage renal disease for 12 months and did not improve with steroid therapy, and one (pattern C) had progressive chronic kidney disease on follow-up.


CONCLUSIONS
IgG4TIN in an Indian cohort most often presents with rapidly progressive renal failure and less often has extra-renal organ involvement. On follow-up, patients can experience a more aggressive course with progression to end stage renal disease.
Department of Histopathology, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
Ritambhra
R
Nada
Department of Nephrology, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
Raja
R
Ramachandran
Department of Histopathology, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
Ashwani
A
Kumar
Department of Nephrology, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
Manish
M
Rathi
Department of Pediatrics, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
Amit
A
Rawat
Department of Histopathology, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
Kusum
K
Joshi
Department of Nephrology, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
Harbir Singh
HS
Kohli
Department of Pediatrics, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
Krishan Lal
KL
Gupta
D000328:Adult; D000368:Aged; D001327:Autoimmune Diseases / Q000097:blood / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology*; D015415:Biomarkers / Q000097:blood; D001706:Biopsy; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D015433:Glomerulonephritis, Membranous / Q000175:diagnosis / Q000276:immunology; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood / Q000276:immunology*; D007150:Immunohistochemistry; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007194:India; D007668:Kidney / Q000187:drug effects / Q000276:immunology* / Q000473:pathology; D007676:Kidney Failure, Chronic / Q000175:diagnosis / Q000276:immunology; D008297:Male; D008875:Middle Aged; D009395:Nephritis, Interstitial / Q000097:blood / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology*; D011446:Prospective Studies; D011507:Proteinuria / Q000175:diagnosis / Q000276:immunology; D012074:Remission Induction; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult
D015415:Biomarkers; D007074:Immunoglobulin G; D007166:Immunosuppressive Agents
IgG4; remission; tubulointerstitial nephritis