[2005] [FRI0012] SPINAL SUBARACHNOID HEMORRHAGE IN
POLYARTERITIS NODOSA
K.D. Torralba1, S. Metyas 1 , P. Colletti
2 , F.P. Quismorio Jr 1 1Department of Medicine,
Division of Rheumatology, 2Department of Radiology, University of
Southern California-Los Angeles County Medical Center, Los Angeles, United
States
Background: Spinal cord involvement in association with classic
polyarteritis nodosa (PAN) is a rare but serious manifestation usually
associated with significant mortality. Majority (at least 80%) of cases of PAN
with spinal subarachnoid hemorrhage had fatal outcomes.
Objectives: We now describe a case of polyarteritis nodosa presenting
with spinal subarachnoid hemorrhage successfully treated with corticosteroids
and cyclophosphamide.
Methods: A retrospective review of a case of PAN with spinal involvement
treated at our institution was done.
Results: Case Report: A 37-year-old female developed pain and swelling of
the wrists, knees and ankles. She was diagnosed with rheumatoid arthritis and
was treated with intramuscular ACTH. Three months later, she developed fever, a
10lb weight loss, headaches, urinary and fecal retention, paresthesia and
weakness in her lower and upper extremities. When seen, she was febrile,
hypertensive, had cervical lymphadenopathy, synovitis and mononeuritis
multiplex. CBC showed anemia, leukocytosis and thrombocytosis. ESR, CRP,
cryoglobulin and rheumatoid factor were all elevated. Screening for hepatitis B
and C were negative. Urinalysis showed proteinuria with granular casts. Urine
collection for protein was 2g/24h. Serum creatinine rose to 2.5mg/dl. Anti-MPO
and pANCA were positive. Lumbar tap yielded bloody cerebrospinal fluid. Thoracic
spine MRI revealed an extramedullary intradural high signal intensity at T5-10
suggestive of subarachnoid hemorrhage. Cerebral and spinal angiograms did not
reveal any vascular abnormalities. MRA of the abdomen revealed aneurysms
involving the kidneys and the superior mesenteric, hepatic and splenic arteries.
A diagnosis of PAN was made and therapy with high dose corticosteroids and oral
cyclophosphamide was started. A repeat MRI after 2 weeks showed diminution of
the spinal lesions. By seven months, the patient had fully recovered with normal
neurologic findings and renal function.
Conclusion: Spinal cord findings, although rare, may be part of the
presenting picture of PAN. Myelopathy, subdural/epidural hematoma and
subarachnoid hemorrhage involving multiple cord levels have been reported. It is
associated with significant mortality due to widespread necrotizing vasculitis
and partly due to a delay in establishing the diagnosis. Neuropathological
changes include vasculitis of the spinal cord arteries with ischemia, aneurysmal
formation and rupture with hemorrhage. Early diagnosis and prompt therapy with
corticosteroids and cyclophosphamide can effect a favorable outcome of spinal
cord involvement in PAN.
References: 1. Travers RL, Allison DJ, Brettle RP, Hughes GR.
Polyarteritis nodosa: a clinical and angiographic analysis of 17 cases. Semin
Arthritis Rheum 1979;8:184-99.
2. Ford RG, Siekert RG. Central nervous system manifestations of periarteritis
nodosa. Neurology 1965;15:114-29.
3. Haft H, Finneson BE, Cramer H, Fiol R. Periarteritis nodosa as a source of
subarachnoid hemorrhage and spinal cord compression. J Neurosurg 1957;14:608-16.
4. Iaconetta G, Benvenuti D, Lamaida E, Gallicchio B, Signorelli F, Maiuri F.
Cerebral hemorrhagic complication in polyarteritis nodosa. Acta Neurologica
1994;16:64-9.
Vasculitis
Citation: Ann Rheum Dis 2005;64(Suppl III):261