DOI: 10.12890/2021_002448
Granulomatosis with Polyangiitis: Recurrence or Treatment Consequences?
Authors
andreiadiegues@gmail.com
Keywords
ANCA-PR3-associated vasculitis, granulomatosis with polyangiitis, intracranial hypertension, lung nodulesAbstract
Granulomatosis with polyangiitis (GPA) is the most common antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). We describe the case of a 38-year-old woman with relapsing GPA who presented with intracranial hypertension, followed by the appearance of cavitated lung nodules despite treatment with azathioprine. Clinical improvement and ANCA titre reduction were observed after rituximab treatment. We report a rare form of GPA relapse and highlight the challenge of following-up patients with GPA, in whom can be hard to distinguish relapse from the consequences of long-term immunosuppression.
References
- Yates M, Watts RA, Bajema IM, Cid MC, Crestani B, Hauser T, et al. EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis. Ann Rheum Dis 2016;75(9):1583–1594.
- Wallace ZS, Miloslavsky EM. Management of ANCA associated vasculitis. BMJ 2020;368March):1–16.
- Geetha D, Jefferson JA. ANCA-associated vasculitis: core curriculum 2020. Am J Kidney Dis 2020;75(1):124–137.
- Arnaoutoglou MA, Xerras CG, Kalevrosoglou IK, Rafailidis VD, Notas KP, Tegos TI. Headache linked to intracranial hypertension and hypertrophic pachymeningitis as the initial and dominant presentation of granulomatosis with polyangiitis. Case report and review of the recent literature. Headache 2018;58(4):589–595.
- Feragalli B, Mantini C, Sperandeo M, Galluzzo M, Belcaro G, Tartaro A, et al. The lung in systemic vasculitis: radiological patterns and differential diagnosis. Br J Radiol 2016;89(1061):20150992.