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Corticosteroids (1 mg/kg/d of oral prednisone) are a mainstay of therapy for severe disease manifestations Azathioprine (2 mg/kg/d orally) may be an effective steroid-sparing agent Cyclophosphamide (2 mg/kg/d orally) or chlorambucil (01 02 mg/kg/d orally) is indicated for severe ocular and central nervous system complications of Beh et s Both colchicine (06 mg once to three times daily orally) and thalidomide (100 mg/d orally) help ameliorate the mucocutaneous findings of Beh et s TNF inhibition and interferon alfa-2a demonstrates some promise as a therapeutic approach, but large studies are lacking to date
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Calamia KT et al Major vessel involvement in Beh et disease Curr Opin Rheumatol 2005 Jan;17(1):1 8 [PMID: 15604898] Diri E et al Neuro-Beh et s syndrome: differential diagnosis and management Curr Rheumatol Rep 2006 Aug;8(4): 317 22 [PMID: 16839512] Kural-Seyahi E et al The long-term mortality and morbidity of Beh et syndrome: a 2-decade outcome survey of 387 patients followed at a dedicated center Medicine (Baltimore) 2003 Jan;82(1):60 76 [PMID: 12544711]
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PRIMARY ANGIITIS OF THE CENTRAL NERVOUS SYSTEM
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Primary angiitis of the central nervous system is a syndrome with several possible causes that produces small and medium-sized vasculitis limited to the brain and spinal cord Biopsy-proved cases have predominated in men who have a history of weeks to months of headaches, encephalopathy, and multifocal strokes Systemic signs and symptoms are absent, and routine laboratory tests are usually normal MRI of the brain is almost always abnormal, and
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ularis include the vasculitides (particularly polyarteritis nodosa), cholesterol emboli syndrome, thrombocythemia, cryoglobulinemia, cold agglutinin disease, primary hyperoxaluria (due to vascular deposits of calcium oxalate), and disseminated intravascular coagulation
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Franc s C et al Dermatologic manifestations of antiphospholipid syndrome: two hundred consecutive cases Arthritis Rheum 2005 Jun;52(6);1785 93 [PMID: 15934071]
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the spinal fluid often reveals a mild lymphocytosis and a modest increase in protein level Angiograms classically reveal a string of beads pattern produced by alternating segments of arterial narrowing and dilation However, neither the MRI nor the angiogram appearance is specific for vasculitis Indeed, in one study, none of the patients who had biopsy-proved central nervous system vasculitis had an angiogram showing the string of beads, and none of the patients with the classic angiographic findings had a positive brain biopsy for vasculitis Many conditions, including vasospasm, can produce the same angiographic pattern as vasculitis Definitive diagnosis requires a compatible clinical picture; exclusion of infection, neoplasm, or metabolic disorder or drug exposure (eg, cocaine) that can mimic primary angiitis of the central nervous system; and a positive brain biopsy In contrast to biopsy-proved cases, patients with angiographically defined central nervous system vasculopathy are chiefly women who have had an abrupt onset of headaches and stroke (often in the absence of encephalopathy) with normal spinal fluid findings Many patients who fit this clinical profile may have reversible cerebral vasoconstriction rather than true vasculitis Such cases may best be treated with calcium channel blockers (such as nimodipine or verapamil) and possibly short course of corticosteroids Biopsy-proved cases usually improve with prednisone therapy and often require cyclophosphamide In recent years, cases of central nervous system vasculitis associated with cerebral amyloid angiopathy have been reported These cases often respond well to corticosteroids, albeit the long-term natural history remains poorly defined
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Calabrese LH et al Narrative review: reversible cerebral vasoconstriction syndromes Ann Intern Med 2007 Jan 2;146(1): 34 44 [PMID: 17200220]
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SERONEGATIVE SPONDYLOARTHROPATHIES
The seronegative spondyloarthropathies are ankylosing spondylitis, psoriatic arthritis, Reiter s syndrome (also called reactive arthritis), and the arthritis associated with inflammatory bowel disease These disorders are noted for male predominance, onset usually before age 40, inflammatory arthritis of the spine or the large peripheral joints (or both), enthesopathy (inflammation of where ligaments, tendons, and joint capsule insert into bone), uveitis in a significant minority, the absence of autoantibodies in the serum, and a striking association with HLA-B27 Present in only 8% of normal whites and 4% of normal blacks, HLA-B27 is positive in 90% of patients with ankylosing spondylitis and 75% with Reiter s syndrome HLAB27 also occurs in 50% of the psoriatic and inflammatory bowel disease patients who have sacroiliitis Patients with only peripheral arthritis in these latter two syndromes do not show an increase in HLA-B27
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