Systemic lupus erythematosus and antineutrophilic cytoplasmic antibody-associated vasculitis overlap syndrome complicated by subarachnoid hemorrhage: case-based review
- 131 Downloads
Systemic lupus erythematosus (SLE) and antineutrophil cytoplasmic antibody-associated vasculitis (AAV) overlap syndrome is an inflammatory disorder with a mixed presentation that is characterized by clinical features of both SLE and AAV. Although renal disease predominates, any organ system in the body may be affected. Neurologic manifestation in patients with SLE–AAV overlap syndrome is rare and has only been previously documented as cerebral ischemia. We report a patient with SLE–AAV overlap syndrome diagnosed based on clinical, serologic and biopsy-proven histologic findings who presented with subarachnoid hemorrhage (SAH) secondary to ruptured right anterior cerebral artery aneurysm. To the authors’ knowledge, this is the first reported case of SLE–AAV overlap syndrome diagnosed in a patient with a SAH due to an intracranial aneurysm. Neurologic involvement in patients with SLE–AAV overlap syndrome is uncommon and has not been well-studied. Clinicians who encounter patients with neurologic signs that present with symptoms and a serologic profile that correspond to both SLE and AAV criteria, should consider the association between SLE–AAV overlap syndrome and a hemorrhagic stroke, specifically SAH.
KeywordsSubarachnoid hemorrhage Lupus ANCA-associated vasculitis Overlap syndrome
All authors assisted in data extraction, data analysis and literature review, and wrote significant sections of the paper.
No funding was received for this study.
Compliance with ethical standards
Conflict of interest
The authors declare that they have no conflict of interest.
Written informed consent was obtained from the patient for publication of this case study.
- 3.Jarrot PA, Chiche L, Hervier B, Daniel L, Vuiblet V, Bardin N, Bertin D, Terrier B, Amoura Z, Andres E, Rondeau E, Hamidou M, Pennaforte JL, Halfon P, Daugas E, Dussol B, Puechal X, Kaplanski G, Jourde-Chiche N (2016) Systemic lupus erythematosus and antineutrophil cytoplasmic antibody-associated vasculitis overlap syndrome in patients with biopsy-proven glomerulonephritis. Medicine (Baltimore) 95(22):e3748. https://doi.org/10.1097/MD.0000000000003748 CrossRefGoogle Scholar
- 4.Pendergraft WF 3rd, Cortazar FB, Wenger J, Murphy AP, Rhee EP, Laliberte KA, Niles JL (2014) Long-term maintenance therapy using rituximab-induced continuous B-cell depletion in patients with ANCA vasculitis. Clin J Am Soc Nephrol 9(4):736–744. https://doi.org/10.2215/CJN.07340713 CrossRefPubMedPubMedCentralGoogle Scholar
- 6.Pagnoux C, Mahr A, Cohen P, Guillevin L (2005) Presentation and outcome of gastrointestinal involvement in systemic necrotizing vasculitides: analysis of 62 patients with polyarteritis nodosa, microscopic polyangiitis, Wegener granulomatosis, Churg–Strauss syndrome, or rheumatoid arthritis-associated vasculitis. Medicine (Baltimore) 84(2):115–128CrossRefGoogle Scholar
- 7.Bougea A, Anagnostou E, Spandideas N, Triantafyllou N, Kararizou E (2015) An update of neurological manifestations of vasculitides and connective tissue diseases: a literature review. Einstein (Sao Paulo) 13(4):627–635. https://doi.org/10.1590/S1679-45082015RW3308 CrossRefPubMedCentralGoogle Scholar
- 15.Fauzi AR, Kong NC, Chua MK, Jeyabalan V, Idris MN, Azizah R (2004) Antibodies in systemic lupus antineutrophil cytoplasmic erythematosus: prevalence, disease activity correlations and organ system associations. Med J Malays 59(3):372–377Google Scholar
- 17.Nasr SH, D’Agati VD, Park H-R, Sterman PL, Goyzueta JD, Dressler RM, Hazlett SM, Pursell RN, Caputo C, Markowitz GS (2008) Necrotizing and crescentic lupus nephritis with antineutrophil cytoplasmic antibody seropositivity. Clin J Am Soc Nephrol 3(3):682–690. https://doi.org/10.2215/cjn.04391007 CrossRefPubMedPubMedCentralGoogle Scholar