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.
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