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Reversible cerebral vasoconstriction syndromes (RCVS)
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- Treatment
- Other
- Epidemiology
- Complications
- 4 major forms of arthritis
- Findings
- Treatment : TNF-alpha blockers
- Reiter’s syndrome
- Common complications : lower extremities: ankles , knees , feet, heels
- Arthritis of inflammatory bowel disease
Reversible cerebral vasoconstriction syndromes (RCVS) (or Call-Fleming Syndrome or Migraine Angiitis [AIM] must be distinguished from classical cerebral angiitis (using CNS imaging) Associated conditions (many) [table] / unlike migraine, no aura, presentation is hyperacute Causes: vasoactive drugs, diet pills, stimulants, some antidepressants, decongestants, illicit drugs (amphetamines, cocaine, ecstasy) Treatment: calcium channel blockers, steroids (mechanisms and treatments being worked out 1/07) Spondylarthropathies AS, psoriasis, Reiter’s and Reactive, IBD spondylitis sacroiliitis enthesopathy asymmetric oligoarthritis Other: inflammatory eye disease, urethritis, and mucocutaneous lesions Labs: all have negative RF Ankylosing spondylitis (AS) (Marie-Stumpell Disease) inflammation and ossification of the joints and ligaments of the spine and of the sacroiliac joints Epidemiology: young people (~24 yrs) / males=females / HLA B27 (90%) / may occur in association with IBD Pathology: chronic, progressive (insidious) inflammatory disease of axial joints (hips, shoulders, sacroiliac) / asymmetrical, oligoarticular (1-4 joints) / inflammation at site of insertion / autoantibodies to joint elements following infection Complications: kyphosis and eventually complete fusion or “bamboo spine” / aortic insufficiency / peripheral joint involvement / pulmonary fibrosis / uveitis (25%) (can lead to glaucoma and blindness) Diagnosis: do sacral XR 1st reveals squaring, syndesmophytes, Presentation: morning stiffness (“gel”) / pace floor at night / improves with exercise / pain may move from one joint to another Treatment: therapeutic goal is to maximize the likelihood that fusion will occur in a straight line physical therapy / avoid smoking (pulmonary compromise) NSAIDS (for symptomatic relief) Anti-TNF-alpha (now in use 2008) methotrexate and sulfasalazine (were tried before TNF-alpha available) surgical procedures to correct some spine and hip deformities may be used in select cases Course: only 6% die from actual disease; most commonly (cervical fracture, heart block, amyloidosis), and more rarely from the restrictive lung disease Psoriatic arthritis (see skin psoriasis) hereditary, 20 to 40 yrs / 7-40% of psoriasis patients get arthritis (may precede skin findings) / also has sporadic form presenting later on in life 4 major forms of arthritis most have peripheral, asymmetric oligoarticular arthritis DIP with nail disease 25% have symmetric polyarthritis similar to RA spondylitis/sacroiliitis less common Findings: DIP swelling, sausage digits [pic] / nail problems (onychodystrophy, onycholysis, nail pitting, and subungual keratosis, onychauxis) [pic] / psoriatic lesions on extensor surfaces Diagnosis: must have skin or nail changes for definitive diagnosis Labs: mildly elevated ESR / hyperuricemia in severe cases Synovial fluid: 2 to 15 WBCs / Radiography: distal interphalangeal erosions or telescoping joints, asymmetric sacroiliitis, isolated axial syndesmophytes Treatment: TNF-alpha blockers slow progression of arthritis and skin complications NSAIDs (indomethacin) and intra-articular steroids (avoid injections through psoriatic plaques) for symptomatic relief / 2nd line: MTX, penicillamine, gold, hydroxychloroquine Reiter’s syndrome (see reactive arthritis) HLA B27 / males, 20-30s / HIV patients Presentation: asymmetric oligoarthritis, (non G-C) urethritis, conjunctivitis, uveitis, characteristic skin and mucous membrane lesions low back pain Onset: 2-4 weeks after inciting GI or GU infection( Chlamydia) Common complications: lower extremities: ankles, knees, feet, heels (enthesitis of Achilles tendon) oligoarticular sausage digits (dactylitis) Other complications: transient conjunctivitis (40%) / may need urgent opthalmological referral (topical or systemic steroids) for (3-5%) disabling iritis, uveitis (can be difficult to treat), corneal ulceration oral ulcers and glans penis (circinate balanitis; 25-40%; painless, red rash) keratoderma blennorrhagicum (mollusk shell skin lesions on palms and soles, may have severe desquamation; similar appearing to papular psoriasis nail changes myocarditis: heart block (<5%), aortic insufficiency Note: many people have single reactive arthritis symptoms without multiple findings Causative organisms: chlamydia trachomatis (decreasing), Neisseria (culture-negative), GI: Shigella, Salmonella, Campylobacter jejuni, Yersinia enterocolitica Labs: elevated ESR and leukocytosis / 0.5 to 75 WBC’s in synovial fluid / bacterial antigens present in joints (chlamydia is dormant) / ANA and RF usu. negative Radiography: asymmetric syndesmophytes along spine (ankylosing spondylitis has symmetric and contiguous) Course: most recover (one to several months), 50% recurrence (varying degrees of disability) Prevention: must take antibiotics (doxycycline) prior to travel / even with HLA B27 – 20% risk of reactive arthritis with proper infection Treatment: symptomatic relief with NSAIDs (2nd line sulfasalazine) and intra-articular steroids / topical steroids for skin complications / prolonged doxycycline may be useful in cases with chlamydia infection Reactive arthritis may follow GI infection (Shigella flexneri, Salmonella species, or Yersinia enterocolitica infections / same joint problems as Reiter’s / extra-articular symptoms tend to be mild / treatment will be similar to Reiter’s (doxy?) HLA B27 Ankylosing Spondylitis (90%) Reiter’s Syndrome (75%) Psoriatic arthritis (20%) / with sacroiliitis/spondylitis (50%) Enteropathic arthritis (IBD) (8%) / with sacroiliitis/spondylitis (50%)
Crohn’s or UC (10-20%) / similar to that of AS spondylitis, sacroiliitis, and peripheral arthritis ( > knee / ankle) peripheral arthritis may correlate with colitis activity (spinal disease does not) antibiotics not effective, but still must rule out septic joints
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