Background: ANCA-associated vasculitis is a small vessel necrotizing vasculitis with few or no immune deposits, necrotizing glomerulonephritis is very common in the microscopic poliangiitis subset. Systemic scleroderma renal involvement is included neither in the current classification criteria, nor in the definition of CREST-syndrome. The presence of ANCA in patients with SS was first described in 1996, and since that has been reported to be rare and not presenting clinical significance. However during next decade about 50 cases with association of SS and AAV were described, and more recently, additional cases were reported and the clinical features of such association were recognized.
Results: We present here a case, illustrating association of systemic sclerosis and ANCAassociated vasculitis, proven by kidney biopsy findings of sclerosing and necrotizing glomerulonephritris with11% of crescents.
Conclusions: Patients with systemic sclerosis in general, and CREST-syndrome in particular, demonstrating unexplained deterioration of kidney function, should be tested for ANCA and undergo kidney biopsy in search for association with AAV. Immunosuppressive treatment, especially in cases with advanced sclerotic changes by kidney pathology evaluation, should be performed with caution to ensure patient’s survival even in cases of progression to ESRD.
Keywords:
Published on: Nov 12, 2016 Pages: 49-52
Full Text PDF
Full Text HTML
DOI: 10.17352/acn.000015
CrossMark
Publons
Harvard Library HOLLIS
Search IT
Semantic Scholar
Get Citation
Base Search
Scilit
OAI-PMH
ResearchGate
Academic Microsoft
GrowKudos
Universite de Paris
UW Libraries
SJSU King Library
SJSU King Library
NUS Library
McGill
DET KGL BIBLiOTEK
JCU Discovery
Universidad De Lima
WorldCat
VU on WorldCat
PTZ: We're glad you're here. Please click "create a new query" if you are a new visitor to our website and need further information from us.
If you are already a member of our network and need to keep track of any developments regarding a question you have already submitted, click "take me to my Query."