Delayed Diagnosis of Proteinase 3- Anti-Neutrophil Cytoplasmic Antibody Associated Vasculitis, A Case Report and Review of Literature Delayed Diagnosis of Proteinase 3- Anti-neutrophil cytoplasmic antibody Associated Vasculitis.
Journal of Pediatric Nephrology,
Vol. 9 No. 4 (2021),
10 November 2021
Anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis is a type of systemic autoimmune disease with blood vessels swelling and inflammation. Wegener's Granulomatosis (WG) is closely associated with antineutrophil cytoplasmic autoantibodies, particularly those directed to proteinase 3 (PR3). An 18-year-old boy with granulomatosis with polyangiitis (GPA) who was diagnosed 6 months back with scleritis and sinusitis at the onset of the disease. During his initial visits to Ear Nose Throat and ophthalmology departments lab tests was not performed on time due to lack of typical symptoms of kidney involvement. Half a year later, lab tests showed PR3-ANCA (Proteinase 3) positive and advanced renal dysfunction, and was finally diagnosed as sclerotic renal failure with fibrotic crescents based on renal biopsy. Scleritis may be the earliest manifestation in systemic vasculitis and is sometimes hard to diagnose at the onset. Delayed diagnosis and treatment will lead to irreversible renal dysfunction.
- Granulomatosis with Polyangiitis
- Delayed Diagnosis
- Sclerotic glomerulonephritis
How to Cite
2.Kallenberg C. Pathogenesis of PR3-ANCA associated vasculitis. J Autoimmun. 2008;30(1-2):29-36.
3.Kallenberg CG, Stegeman CA, Abdulahad WH, Heeringa P. Pathogenesis of ANCA-associated vasculitis: new possibilities for intervention. Am J Kidney Dis. 2013;62(6):1176-87.
4.Harper L, Savage C. ANCA-associated renal vasculitis at the end of the twentieth century—a disease of older patients. Rheumatology. 2005;44(4):495-501.
5.Hauer HA, Bajema IM, Van Houwelingen HC, Ferrario F, Noël L-H, Waldherr R, et al. Renal histology in ANCA-associated vasculitis: differences between diagnostic and serologic subgroups. Kidney international. 2002;61(1):80-9.
6.Smith JR, Mackensen F, Rosenbaum JT. Therapy insight: scleritis and its relationship to systemic autoimmune disease. Nat Clin Pract Rheumatol. 2007;3(4):219-26.
7.Csernok E, Moosig F. Current and emerging techniques for ANCA detection in vasculitis. Nature Reviews Rheumatology. 2014;10(8):494.
8.Shang W, Ning Y, Xu X, Li M, Guo S, Han M, et al. Incidence of cancer in ANCA-associated vasculitis: a meta-analysis of observational studies. PloS one. 2015;10(5).
- Abstract Viewed: 201 times
- PDF Downloaded: 123 times