OWN - NLM
DA - 20170517
LR - 20170517
IS - 1536-5964 (Electronic)
IS - 0025-7974 (Linking)
VI - 96
IP - 20
DP - 2017 May
TI - Concurrent IgG4-related tubulointerstitial nephritis and IgG4
myeloperoxidase-anti-neutrophil cytoplasmic antibody positive crescentic
glomerulonephritis: A case report.
PG - e6707
LID - 10.1097/MD.0000000000006707 [doi]
AB - 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.
FAU - Su, Tao
AU - Su T
AD - aRenal Division, Department of Medicine, Peking University First Hospital
bInstitute of Nephrology, Peking University cKey Laboratory of Renal Disease,
Ministry of Health of China dKey Laboratory of CKD Prevention and Treatment,
Ministry of Education of China eDepartment of Pathology, Peking University First
Hospital fPeking-Tsinghua Center for Life Sciences, Beijing, China.
FAU - Yang, Li
AU - Yang L
FAU - Cui, Zhao
AU - Cui Z
FAU - Wang, Su-Xia
AU - Wang SX
FAU - Zhao, Ming-Hui
AU - Zhao MH
LA - eng
PT - Journal Article
PL - United States
TA - Medicine (Baltimore)
JT - Medicine
JID - 2985248R
EDAT- 2017/05/18 06:00
MHDA- 2017/05/18 06:00
CRDT- 2017/05/18 06:00
AID - 10.1097/MD.0000000000006707 [doi]
AID - 00005792-201705190-00004 [pii]
PST - ppublish
SO - Medicine (Baltimore). 2017 May;96(20):e6707. doi: 10.1097/MD.0000000000006707.