Necrotizing arteritis and spinal subarachnoid hemorrhage in Sjögren syndrome

Abstract

A 37-year-old woman with primary Sjögren syndrome developed mixed cryoglobulinemia and systemic vasculitis. Subarachnoid hemorrhage occurred as a result of necrotizing anterior spinal arteritis. Although rarely seen in mixed cryoglobulinemia, central nervous system complications have recently been documented in Sjögren syndrome. The patient's serum contained antibodies to the Ro(SSA) cytoplasmic antigen, and these antibodies were concentrated in the cryoglobulin fraction. Anti-Ro(SSA) antibodies are associated with the occurrence of vasculitis in patients with Sjögren syndrome, which suggests that the spinal arteritis and subarachnoid hemorrhage in this patient may have been directly related to the underlying connective tissue disorder.

Evidence of an immunopathogenic basis for central nervous system disease in primary Sjögren's syndrome

“…Our preliminary observations on postmortem and biopsy specimens of brain tissue from patients with CNS-SS indicate that there may be a cerebral vasculopathy (Alexander et al: unpublished observations), and we have described an SS patient with necrotizing spinal arteritis (7). Other investigators have documented the presence of IVD in SS patients with active nervous system disease (1,12,13).…”

“…Central nervous system (CNS) complications of primary SS (CNS-SS) are becoming recognized increasingly. A spectrum of CNS manifestations has been observed, including focal neurologic deficits, psychiatric disturbances, cognitive dysfunction, seizure of movement disorders, recurrent aseptic meningoencephalitis (AME), and acute and chronic myelopathy (5)(6)(7)(8)(9).…”

Sjögren's syndrome presenting as ischemic stroke.

“…The most common mechanism of PNS and CNS involvement during SS is due to vasculitis of small vessels. Necrotizing vasculitis 24 - 25 involving the entire neuroaxis has been described in two autopsied cases of SS. T-and B-lymphocytic perivascular leptomeningeal and intraparenchymal infiltration is described in a brain biopsy in a case of reversible dementia associated with SS.…”

Cerebral vasculopathy associated with collateralization resembling moya moya phenomenon and with anti‐Ro/SS‐A and anti–La/SS‐B antibodies

“…During the course of our studies on SLE and SS patients who are positive for anti-Ro/SS-A, we have seen, in addition to the patient described here, 3 anti-ROBS-A antibody positive patients who have developed vasculitis involving medium-to-large-sized arteries. In only 1 of these cases, however, was there histologic confirmation of the vasculitis (11). In 1 SS patient, the vasculitis involved the anterior spinal artery and resulted in a fatal subarachnoid hemorrhage (11).…”

“…In only 1 of these cases, however, was there histologic confirmation of the vasculitis (11). In 1 SS patient, the vasculitis involved the anterior spinal artery and resulted in a fatal subarachnoid hemorrhage (11). Another patient, who had both SLE and SS, developed Takayasu arteritis.…”