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2015 ; 94
(32
): e1366
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Rituximab for the Treatment of IgG4-Related Tubulointerstitial Nephritis: Case
Report and Review of the Literature
#MMPMID26266393
McMahon BA
; Novick T
; Scheel PJ
; Bagnasco S
; Atta MG
Medicine (Baltimore)
2015[Aug]; 94
(32
): e1366
PMID26266393
show ga
Immunoglobulin type gamma 4 (IgG4)-related disease is a relatively newly
described clinical entity characterized by a distinctive histopathological
appearance, increased numbers of IgG4 positive plasma cells and often, but not
always, elevated serum IgG4 concentrations. The most common renal manifestation
of IgG4-related disease is tubulointerstitial nephritis marked with proteinuria,
hematuria, decreased kidney function, hypocomplementemia, and radiologic
abnormalities. Renal biopsy characteristics include dense lymphoplasmacytic
tubulointerstitial nephritis that stains for IgG4, storiform fibrosis, and immune
complex deposition in the interstitium and along tubule basement membranes.
Treatment traditionally consists of prolonged glucocorticoids but cases
refractory to glucocorticoids have been reported.We report a case of a
58-year-old Caucasian man who presented with fatigue, 50 pound weight loss,
dyspnea, lymphadenopathy, and nephromegaly. The patient was first misdiagnosed as
chronic interstitial nephritis secondary to renal sarcoid and was treated with
repeated doses of prednisone. On his third relapse, he underwent a repeat renal
biopsy and a diagnosis of IgG4-tubulointerstitial nephritis was confirmed. He was
refractory to treatment with prednisone. The patient received Rituximab and had
prompt sustained improvement in renal function. At 1 year post Rituximab
treatment, his serum creatinine remains at baseline and imaging study revealed
reduction in his kidney size.This is the first case report using Rituximab as a
steroid sparing option for refractory IgG4-tubulointerstitial nephritis. More
information is needed on the long-term effects of using of B-cell depleting
agents for glucocorticoid resistant IgG4-tubulointerstitial nephritis.
|Antibodies, Monoclonal, Murine-Derived/*therapeutic use
[MESH]