ANCA-Associated Vasculitis in Patient with CREST-Syndrome - Case Report
Journal Title: Archives of Clinical Nephrology - Year 2016, Vol 2, Issue 1
Abstract
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 ANCA-associated 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.
Authors and Affiliations
Zakharova EV, Makarova TA, Stolyarevich ES
Keywords
Related Articles
- EP ID EP344881
- DOI 10.17352/acn.000015
- Views 100
- Downloads 0
Some Aspects of the Evaluation of Brush Border Enzymuria Originating from Proximal Renal Tubules as Screening Test in Patients with Psoriatic Arthritis
Aim: To compare diagnostic values and laboratory variables of alanine - aminopeptidase (microsomale AAP), γ-glutamyl transferase (γ−GT),β2-microglobuline (β2-M), C Reactive Protein (CRP) and index for disease activity (P...
Wunderlich syndrome; Spontaneous Atraumatic Rupture of the kidney: A case report
Atraumatic spontaneous retroperitoneal haemorrhage is a distinct clinical entity with potentially life-threatening complications. The commonest aetologies include ruptured aortic or visceral aneurysm, spontaneous rupture...
The Necessity of Nephrology Consultation for Critically Ill Patients with Acute Renal Failure
Background: Acute renal failure (ARF) continues to be a challenging problem in critically ill patients. We reviewed the nephrology consultations in our ICU to assess the necessity of those consultations and if there are...
Phenazopyridine abuse presenting with acute kidney injury, hemolytic anaemia and jaundice
Phenazopyridine, an azo dye, is commonly used to relieve dysuria caused by bladder irritation or infection. We report the case of a 64-year-old lady who presented with unexplained sub-acute onset hemolytic anaemia follow...
An End-Stage Renal Disease Patient with Invasive Fungal Rhinosinusitis
Mucormycosis is one of the invasive fungal infections particularly in immunocompromised patients with impaired host defense. It is characterized by fungal rhino sinusitis with invasion of adjustment structures including...