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.jpg): Failed to open stream: No such file or directory in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 117 Medicine+(Baltimore)
2017 ; 96
(20
): e6707
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Concurrent IgG4-related tubulointerstitial nephritis and IgG4
myeloperoxidase-anti-neutrophil cytoplasmic antibody positive crescentic
glomerulonephritis: A case report
#MMPMID28514287
Su T
; Yang L
; Cui Z
; Wang SX
; Zhao MH
Medicine (Baltimore)
2017[May]; 96
(20
): e6707
PMID28514287
show ga
RATIONALE: IgG4-related disease (IgG4-RD) is a newly recognized systemic disease.
The typical pathological finding in the kidney is abundant IgG4-positive plasma
cell infiltration with characteristic storiform fibrosis in the interstitium.
Antibodies of the IgG4 subclass have been linked to certain autoimmune diseases
including antiproteinase 3 (PR3) anti-neutrophil cytoplasmic antibody (ANCA) of
the IgG4 subclass. Here, we report a rare case of kidney injury with concurrent
typical IgG4-related tubulointerstitial nephritis and IgG4 subclass of
myeloperoxidase (MPO) ANCA-positive necrotizing crescentic glomerulonephritis.
PATIENT CONCERNS: A 42-year-old Chinese man presented with repeated epigastric
pain, sausage-shaped pancreas observed morphologically in computed tomography,
effectiveness of prednisone therapy and was diagnosed with autoimmune
pancreatitis. He subsequently developed acute kidney injury. DIAGNOSES: The
patient had an elevated serum IgG4, eosinophilia, and positive MPO-ANCA of
IgG4-dominant subclass. Renal biopsy revealed necrotizing crescentic nephritis
and typical IgG4-related tubulointerstitial nephritis. INTERVENTIONS: The patient
was treated with a combination of corticosteroids and cyclophosphamide, and a
course of rituximab was later added to deplete peripheral B cells. OUTCOMES: The
patient responded well and his renal function improved. LESSONS: This is the
first case report of an IgG4-RD with concurrent IgG4-related tubulointerstitial
nephritis and IgG4 MPO-ANCA-associated necrotizing crescentic glomerulonephritis.
It raises the difficulty in differentiation diagnosis of the two separate
diseases that is worthy of further study.