The prevalence of symptomatic osteonecrosis is 2-3% in APS patients, but up to 20% of PAPS may have asymptomatic osteonecrosis. Several factors have been associated with this disorder (Table 7.21), although the pathogenic mechanisms still remain partially elucidated. The most predominant hypotheses are included in Table 7.22.
Table 7.21. Etiologic factors associated with osteonecrosis. Trauma
Hematologic disorders: Sickle cell disease, Thalassemias, DIC, Polycythemia, Hemophilia, Clotting disorders. Inherited thrombophilic factors: Protein C deficiency, Protein S deficiency, Antithrombin deficiency, Factor V Leiden, Homo-cysteinemia, Dysfibrinogenemia, decrease Tissue plasminogen activator, increase Plasminogen activator inhibitor. Acquired thrombophilic factors: aPL, Nephrotic syndrome, Smoking, Alcohol, Pregnancy, Estrogens, Obesity, Diabetes mellitus, Cushing syndrome, Corticosteroids, Malignancies, Hepatic failure, Hyperlipidemia.
Connective tissue diseases (CTD): SLE, APS, RA, Systemic vascu-litis, Systemic sclerosis.
Cytotoxic agents: vincristine, vinblastine, cisplatin, bleomycine, methotrexate, cyclophosphamide, 5-fluorouracil. Infections: HIV, meningococcemia.
Metabolic conditions: Gaucher disease, Hyperparathyroidism, Hyperlipidemia, Hemodialysis, Renal transplant, Diabetes Mel-litus, Gout.
Gastrointestinal diseases: Pancreatitis, Inflammatory bowel disease. Others: radiation therapy, Legg-Calve-Perthes disease, dysbaric osteonecrosis, Fabry disease.
Table 7.22. Pathogenic mechanisms associated with osteonecrosis.
Mechanical vascular interruption (trauma, fractures) Injury to or pressure on a vessel wall (vasculitis, infection, radiation, Gaucher disease)
Vascular embolism (fat, nitrogen bubbles, sickle cells) Thrombosis
Small vessel vasulitis or thrombotic microvasculopathy associated with aPL have been suggested as the pathogenetic mechanisms in autoimmune diseases, even in the absence of corticosteroid administration.
Osteonecrosis can be entirely asymptomatic or it can be associated with pain and/or limitation of the movement in the affected joints. The most susceptible sites for osteonecrosis are the bones with single blood terminal supply such as the femoral head (the most vulnerable), the talus, the humerus head, or the carpal bones.
Early diagnosis is crucial in selecting the appropriate treatment options. Radiographic findings in early stages are unremarkable. In advanced disease, flattening, subchondral radiolucent lines (crescent sign), and collapse may be present. MRI is recognized as the most sensitive tool for the early recognition of osteonecrosis, having more than 95% overall sensitivity.
Treatment strategies primarily depend on the location, size and stage of the lesion. Conservative therapy, used in early stages, includes nonsteroidal agents or other analgesics for pain relief, steroid tapering, weightbearing avoidance, bed rest, or even immobilization for some cases. However, conservative therapies usually fail to prevent the progression of osteonecrosis. Thus, various prophylactic surgical procedures have been proposed in order to suppress the evolution of further degenerative changes (e.g., core decompression with or without bone grafting, osteotomy). Total joint arthroplasty is recommended for the late stage, which is characterized by necrotic subchondral bone, articular cartilage collapse, and secondary osteoarthrosis.
The pathogenesis of sensorineural hearing loss (SNHL) is still considered idiopathic in most cases.
Evidence now exists that inner-ear pathology is frequently associated with immune dysfunction, including the presence of aCL in the sera of these patients. In fact, early corticoster-oid and immunosupressor treatment has been associated with high improvement of symptoms.
Since the internal auditory artery is an end artery, disturbed circulation of the inner ear has long been suggested as the cause of sudden SNHL. Because microthrombosis associated with aPL is known to affect either dermal or retinal vascula-ture, a similar involvement of the cochlear vessels could be envisioned, causing sudden SNHL.
Sudden deafness, severe hearing loss of acute onset is usually unilateral, but may also occur bilaterally as well.
If suspected, the use of anticoagulant therapy in these patients may be considered.
Ocular vaso-occlusive disease is a common finding in APS. aPL damage is predominant in the posterior segment as retinal or choroidal vaso-occlusive diseases and can be arterial, venous, or both. Visual aPL-related symptoms are shown in Table 7.23.
Anterior segment symptoms are less common, these include telangiectasias at conjunctival vessels, keratitis limbal or filamentary, and neovascular.
Table 7.23. aPL-related visual symptoms.
Decrease vision Transient blurring Amaurosix fugax Transient diplopia Field loss Photopsy
Differential diagnosis should consider diabetic retinopathy, sarcoid, and hematological neoplasia, as well as stroke and accelerated artheriosclerosis, and of course systemic rheumatic diseases such as SLE, Sjogren's Syndorme or sclero-derma, and some infections.
Prompt recognition and anticlotting therapy (aspirin and long-term oral anticoagulation) limit organ damage and usually achieve sustained improvement.
Amigo MC (2006) Kidney disease in antiphospholipid syndrome.
Rheum Dis Clin North Am 32(3):509-522 Amigo MC, Garcia-Torres R, Robles M, Bochicchio T, Reyes PA (1992) Renal involvement in primary antiphospholipid syndrome. J Rheumatol 19(8):1181-1185 Asherson RA, Cervera R (2007) Pulmonary hypertension, antiphos-pholipid antibodies, and syndromes. Clin Rev Allergy Immunol 32(2):153-158
Asherson RA, Khamashta MA, Gil A, Vazquez JJ, Chan O, Baguley E, Hughes GR (1989a) Cerebrovascular disease and antiphos-pholipid antibodies in systemic lupus erythematosus, lupus-like disease, and the primary antiphospholipid syndrome. Am J Med 86(4):391-399
Asherson RA, Khamashta MA, Baguley E, Oakley CM, Rowell NR, Hughes GRV (1989b) Myocardial infarction and antiphos-pholipid antibodies in SLE and related disorders. Q J Med 73(272):1103-1115 Asherson RA, Higenbottam TW, Dinh Xuan AT, Khamashta MA, Hughes GRV (1990) Pulmonary hypertension in a lupus clinic: experience with twenty-four patients. J Rheumatol 17(10):1292-1298
Asherson RA, Cervera R, de Groot PG, Erkan D, Boffa MC, Piette JC et al (2003) Catastrophic antiphospholipid syndrome: international consensus statement on classification criteria and treatment guidelines. Lupus 12(7):530-534 Asherson RA, Frances C, Iaccarino L, Khamashta MA, Malacarne F, Piette JC et al (2006) The antiphospholipid antibody syndrome: diagnosis, skin manifestations and current therapy. Clin Exp Rheumatol 24(1 Suppl 40):S46-S51 Branch DW, Khamashta MA (2003) Antiphospholipid syndrome: obstetric diagnosis, management and controversies. Obstet Gynecol 101(6):1333-1344 Cabral AR, Cabiedes J, Alarcon-Segovia D (1990) Hemolytic anemia related to an IgM autoantibody to phosphatidylcholine that binds in vitro to stored and to bromelain-treated human eryth-rocytes. J Autoimmun 3(6):773-787 Castanon C, Amigo MC, Banales JL, Nava A, Reyes PA (1995) Ocular vaso-occlusive disease in primary antiphospholipid syndrome. Ophthalmology 102:256-262 Cervera R, Khamashta MA, Font J, Reyes PA, Vianna JL, Lopez-Soto A et al (1991) High prevalence of significant heart valve lesions in patients with the 'primary' antiphospholipid syndrome. Lupus 1(1):43-47 Cuadrado MJ, Mujic F, Munoz E, Khamashta MA, Hughes GRV (1997) Thrombocytopenia in the antiphospholipid syndrome. Ann Rheum Dis 56(3):194-196 Cuadrado MJ, Khamashta MA, Ballesteros A, Godfrey T, Simon MJ, Hughes GRV (2000) Can neurologic manifestations of Hughes (antiphospholipid) syndrome be distinguished from multiple sclerosis? Analysis of 27 patients and review of the literature. Medicine (Baltimore) 79(1):57-68 Derksen RH, Khamashta MA, Branch DW (2004) Management of the obstetric antiphospholipid syndrome. Arthritis Rheum 50(4):1028-1039
Espinosa G, Bucciarelli S, Cervera R, Lozano M, Reverter JC, de la Red G et al (2004) Thrombotic microangiopathic haemo-lytic anaemia and antiphospholipid antibodies. Ann Rheum Dis 63(6):730-736
Espinosa G, Bucciarelli S, Asherson RA, Cervera R (2008) Morbidity and mortality in the catastrophic antiphospholipid syndrome: pathophysiology, causes of death, and prognostic factors. Semin Thromb Hemost 34(3):290-294 Herranz MT, Rivier G, Khamashta MA, Blaser KU, Hughes GRV (1994) Association between antiphospholipid antibodies and epilepsy in patients with systemic lupus erythematosus. Arthritis Rheum 37(4):568-571 Hughes GR (2003) Migraine, memory loss, and "multiple sclerosis "Neurological features of the antiphospholipid (Hughes') syndrome. Postgrad Med J 79(928):81-83 Nesher G, Ilany J, Rosenmann D, Abraham AS (1997) Valvular dysfunction in antiphospholipid syndrome: prevalence, clinical features, and treatment. Semin Arthritis Rheum 27:27-35 Khamashta MA (2000) Hughes syndrome: antiphospholipid syndrome. Springer, London, p 474 Khamashta MA, Cervera R, Asherson RA, Font J, Gil A, Coltart DJ et al (1990) Association of antibodies against phospholipids with heart valve disease in systemic lupus erythematosus. Lancet 335(8705):1541-1544 Mclntyre JA, Wagenknecht DR (2003) Antiphospholipid antibodies and renal transplantation: a risk assessment. Lupus 12(7):555-559 Meroni PL, Peyvandi F, Foco L, Bernardinelli L, Fetiveau R, Mannucci PM et al (2007) Anti-beta 2 glycoprotein I antibodies and the risk of myocardial infarction in young premenopausal women. J Thromb Haemost 5(12):2421-2428 Paran D, Chapman J, Korczyn AD, Elkayam O, Hilkevich O, Groozman GB et al (2006) Evoked potential studies in the antiphospho-lipid syndrome: differential diagnosis from multiple sclerosis. Ann Rheum Dis 65(4):525-528 Ruiz-Irastorza G, Khamashta MA (2007) Antiphospholipid syndrome in pregnancy. Rheum Dis Clin North Am 33(2):287-297 Sangle SR, D'Cruz DP, Jan W, Karim MY, Khamashta MA, Abbs IC et al (2003) Renal artery stenosis in the antiphospho-lipid (Hughes) syndrome and hypertension. Ann Rheum Dis 62(10):999-1002
Sangle S, D'Cruz DP, Khamashta MA, Hughes GR (2004) Antiphospholipid antibodies, systemic lupus erythematosus, and nontraumatic metatarsal fractures. Ann Rheum Dis 63(10):1241-1243 Sangle SR, D'Cruz DP, Abbs IC, Khamashta MA, Hughes GR (2005) Renal artery stenosis in hypertensive patients with antiphospho-lipid (Hughes) syndrome: outcome following anticoagulation. Rheumatology (Oxford) 44(3):372-377 Sanna G, Bertolaccini ML, Cuadrado MJ, Laing H, Khamashta MA, Mathieu A et al (2003) Neuropsychiatric manifestations in systemic lupus erythematosus: prevalence and association with antiphospholipid antibodies. J Rheumatol 30(5):985-992
Shoenfeld Y, Lev S, Blatt I, Blank M, Font J, von Landenberg P et al
(2004) Features associated with epilepsy in the antiphospholipid syndrome. J Rheumatol 31(7):1344-1348
Stone S, Khamashta MA, Poston L (2001) Placentation, antiphospholipid syndrome and pregnancy outcome. Lupus 10(2):67-74 Stone S, Hunt BJ, Khamashta MA, Bewley SJ, Nelson-Piercy C
(2005) Primary antiphospholipid syndrome in pregnancy: an analysis of outcome in a cohort of 33 women treated with a rigorous protocol. J Thromb Haemost 3(2):243-245
Suvajac G, Stojanovich L, Milenkovich S (2007) Ocular manifestations in antiphospholipid syndrome. Autoimmun Rev 6(6):409-414 Tektonidou MG, Malagari K, Vlachoyiannopoulos PG, Kelekis DA, Moutsopoulos HM (2003) Asymptomatic avascular necrosis in patients with primary antiphospholipid syndrome in the absence of corticosteroid use: a prospective study by magnetic resonance imaging. Arthritis Rheum 48(3):732-736 Tektonidou MG, Varsou N, Kotoulas G, Antoniou A, Moutsopou-los HM (2006) Cognitive deficits in patients with antiphos-pholipid syndrome: association with clinical, laboratory, and brain magnetic resonance imaging findings. Arch Intern Med 166(20):2278-2284 Toubi E, Ben-David J, Kessel A, Podoshin L, Golan TD (1997) Autoimmune aberration in sudden sensorineural hearing loss: association with anti-cardiolipin antibodies. Lupus 6:540-542 Uthman IW, Khamashta MA (2006) Livedo racemosa: a striking dermatological sign for the antiphospholipid syndrome. J Rheu-matol 33(12):2379-2382 Uthman I, Godeau B, Taher A, Khamashta M (2008) The hemato-logic manifestations of the antiphospholipid syndrome. Blood Rev 22(4):187-194 Yehudai D, Shoenfeld Y, Toubi E (2006) The autoimmune characteristics of progressive or sudden sensorineural hearing loss. Auto-immunity 39(2):153-158
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