Diagnostic dilemma of Wegener’s granulomatosis and its effective management: A rare case report and update
https://doi.org/10.4081/cdr.10.10651
Accepted: 27 June 2022
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Granulomatosis with Polyangiitis (GPA) is an uncommon condition belongs to the group of ANCA-associated necrotizing vasculitides. It is characterized by necrotizing granulomatosis of upper and lower respiratory system with coexisting glomerulonephritis. Its signs and symptoms are largely varied due to a wide spectrum of involvement sites. In the present case report, we describe a case of 26-year-old female presented to our centre with history of bilateral ear discharge, earache, and dry cough with a deviated mouth angle to left. Diagnostic work up was done and patient was treated with pulse methylprednisolone, plasmapheresis, and rituximab. Timely diagnosis and prompt initiation of the treatment has prevented the GPA progression and helped the patient to improve quickly. Currently, the patient is doing well and is in remission.
Lakhani DA, Balar AB, Adelanwa A, et al. Granulomatosis with polyangiitis: A case report and brief review of literature. Radiol Case Reports 2021;16:3445-50.
Iijima Y, Kobayashi Y, Uchida Y, et al. A case report of granulomatous polyangiitis complicated by tuberculous lymphadenitis. Medicine (Baltimore) 2018;97:e12430.
Tiwari, Raman A, Gupta A, et al. Granulomatosis with polyangiitis masquerading as renal mass: Case report and literature review. Indian J Nephrol 2021;31:406.
Kitching AR, Anders H-J, Basu N, et al. ANCA-associated vasculitis. Nat Rev Dis Prim 2020;6:1-27.
Arslan S, Işik AÜ, Mungan S, Ural A, Sarı RA. Wegener’s Granulomatosis with an Atypical Presentation as Acute Tonsillitis. ORL 2014;76:57-61.
Falk RJ, Merkel PA, King TA. Granulomatosis with polyangiitis and microscopic polyangiitis: Clinical manifestations and diagnosis. UpToDate. Accessed October 1, 2021. Available from: https://www.uptodate.com/contents/granulomatosis-with-polyangiitis-and-microscopic-polyangiitis-clinical-manifestations-and-diagnosis
Radiopaedia. Granulomatosis with polyangiitis | Radiology Reference Article | Radiopaedia. Accessed October 1, 2021. Available from: https://radiopaedia.org/articles/granulomatosis-with-polyangiitis
Hoffman GS, Kerr GS, Leavitt RY, et al. Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med 1992;116:488-98.
Cardenas-Garcia J, Farmakiotis D, Baldovino B-P, Kim P. Wegener’s granulomatosis in a middle-aged woman presenting with dyspnea, rash, hemoptysis and recurrent eye complaints: A case report. J Med Case Reports 2012;6:1-3.
Bohm M, Gonzalez Fernandez MI, Ozen S, et al. Clinical features of childhood granulomatosis with polyangiitis (wegener’s granulomatosis). Pediatr Rheumatol 2014;12:1-5.
Ananthakrishnan L, Sharma N, Kanne JP. Wegener’s granulomatosis in the chest: High-resolution CT findings. 2012;192:676-82.
Langford CA, Fauci AS. The vasculitis syndromes. Harrison’s Principles of Internal Medicine, 20e. AccessMedicine; McGraw Hill Medical. Accessed October 3, 2021. Available from: https://accessmedicine.mhmedical.com/content.aspx?bookid=2129§ionid=192285458
Sanchez-Masiques J, Ettensohn DB. Alveolar hemorrhage in Wegener's granulomatosis. Am J Med Sci 1989;297:390-3.
Zagelbaum N, Zainab S, Gilani A, et al. Granulomatosis with polyangiitis (GPA) case report outlining the importance of urinalysis in patients presenting with pulmonary cavitary lesions. Pulm Crit Care Med 2016;1:1000119.
Shafiei K, Luther E, Archie M, Gulick J, Fowler MR. Wegener granulomatosis: Case report and brief literature review. J Am Board Fam Pract 2003;16:555-9.
Luqmani RA. State of the art in the treatment of systemic vasculitides. Front Immunol 2014;5:471.
Jones RB, Tervaert JW, Hauser T, et al. Rituximab versus cyclophosphamide in ANCA-associated renal vasculitis. N Engl J Med 2010;363:211-20.
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