Cerebral vasculitis in a patient with rheumatoid arthritis

Mrabet, Dalila; Meddeb, N.; Ajlani, H.; Sahli, H.; Sellami, Slaheddine

doi:10.1016/j.jbspin.2006.08.004

Cited by 50 publications

(18 citation statements)

References 9 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Thus, CNS vasculitis in XLP differs from CNS vasculitis occurring in the setting of other inflammatory conditions such as rheumatoid arthritis[16] or systemic lupus erythematosis[17], where it is possible to achieve remissions through the use of methylprednisone and cyclophosphamide or agents targeting B cells or Tumor Necrosis Factor-α. Given its poor outcome, it is critical that XLP CNS vasculitis be recognized promptly and treated aggressively.…”

Section: Resultsmentioning

confidence: 99%

Lymphocytic vasculitis involving the central nervous system occurs in patients with X‐linked lymphoproliferative disease in the absence of Epstein–Barr virus infection

Talaat

Rothman

Cohen

et al. 2009

Pediatric Blood & Cancer

View full text Add to dashboard Cite

X-linked lymphoproliferative disease (XLP) is an immunodeficiency caused by defects in the adaptor molecule SAP. The manifestations of XLP generally occur following Epstein-Barr virus (EBV) infection and include fulminant mononucleosis, hypogammaglobulinemia and lymphoma. In this report, we describe two unrelated patients with fatal T cell-mediated central nervous system vasculitis for whom repeated serologic and molecular testing for EBV was negative. In both patients, clonal T cell populations were observed, but neither demonstrated evidence of lymphoma. Thus, loss of SAP function can lead to dysregulated immune responses characterized by the uncontrolled expansion and activation of T cells independent of EBV infection.

show abstract

Section: Resultsmentioning

confidence: 99%

Lymphocytic vasculitis involving the central nervous system occurs in patients with X‐linked lymphoproliferative disease in the absence of Epstein–Barr virus infection

Talaat

Rothman

Cohen

et al. 2009

Pediatric Blood & Cancer

View full text Add to dashboard Cite

show abstract

“…These processes are exceedingly rare and do occur mostly in association with a disturbed immune function. There are reports of isolated CNS vasculitis following systemic lupus erythematosus [13], rheumatoid arthritis [15], immunization and X-linked lymphoproliferative (XLP) disease [8,23]. In our patients the clinical settings lacked any of these conditions and so the final diagnosis of PACNS was proposed.…”

Section: Discussionmentioning

confidence: 86%

Primary angiitis of the central nervous system: 2 atypical cases

Pagni¹,

Isimbaldi²,

Vergani³

et al. 2012

View full text Add to dashboard Cite

A b s t r a c t The presence of an angiitis process in the central nervous system (CNS) characterizes different groups of conditions: from idiopathic pachymeningitis to lymphoproliferative disorders. In absence of specific infections, inflammatory and neoplastic diseases, the term "PACNS" (Primary Angiitis of the CNS) was proposed to indicate a peculiar vascular inflammation of unknown origin of meningeal vessels extending to the brain or spinal cord parenchyma. We report two cases of PACNS with peculiar and atypical features: the first one with a possible Epstein Barr virus (EBV) relationship, the second one with spinal cord involvement only, treated surgically. We also hypothesize a correlation between EBV chro nic infection and possible subtypes of PACNS stressing the importance of EBER (EBV-encoded RNA) test in the routine examination of brain biopsies suspicious for PACNS.

show abstract

“…Patient may present with headache that was unresponsive to symptomatic treatment developed abruptly, together with gait disorders 22 . Rheumatoid arthritis may develop after treatment of other disease.…”

Section: Atypical Presentationmentioning

confidence: 99%