Osteonecrosis and Antiphospholipid Syndrome

Abstract

Osteonecrosis (ON), or avascular necrosis of bone, has been related to decreased blood flow to the bone. Many local and systemic factors have been implicated in the pathogenesis of ON, involving corticosteroid therapy, systemic lupus erythematosus (SLE), hemoglobinopathies, alcohol abuse, Caisson disease, Gaucher disease, and hypercoagulability states. We describe the case of a previously healthy young male with no history of corticosteroid therapy, who developed ON initially on the femoral head, and later on the humeral head with high levels of anticardiolipin antibodies (aCL), beta-2-glycoprotein 1 antibodies and positive lupus anticoagulant. The association between primary antiphospholipid syndrome (PAPS) and ON is controversial and few cases without other risk factors have been described. A review of ON pathogenesis and its relation with thrombotic microangiopathy because of PAPS is presented.

Bone Infarctions in a Patient With Systemic Lupus Erythematosus and Anti-Cardiolipin Antibodies

“…1 AVN is a well-known but rare complication in patients with systemic lupus erythematosus (SLE), usually affecting weight-bearing bones. [2][3][4][5] We illustrate an additional, dramatic case of bone infarctions in a patient with SLE and anticardiolipin antibodies (aCL) and add to the discussion of the role of aCL.…”

Aseptic necrosis at multiple localisations in a lupus patient with lymphoma

“…In our case, the patient had no presence of antiphospholipid antibodies [anti-cardiolipin antibody (aCL), lupus anticoagulant and anti-β2-glycoprotein-I]. There are some case reports and studies describing the strong association between primary and secondary APS and ON (6, 7). However, no difference in the prevalence of aCL in patients with or without ON were found previously (8, 9).…”

The Pathogenesis of Nontraumatic Osteonecrosis

“…Thrombotic occlusion of the microcirculation can occur from hereditary thrombophilia, impaired fibrinolysis or antiphospholipid antibodies [16]. Additional causes include environmental or acquired/preexisting conditions, such as hyperlipidemia, hypersensitivity reactions, thromboplastin release during pregnancy, malignant tumors, and inflammatory bowel disease all may contribute additional risk to individuals with an underlying genetic predisposition to form microvascular thrombi [16, 21, 26, 29, 32, 34, 50, 72, 73, 75, 78, 92–94]. The role of sickle cell disease and other hemoglobinopathies in promoting ON of the femoral head has been well documented and also seems to act through the final pathway of intravascular coagulation [12–14, 95–98].…”

“…As previously discussed, thrombophilic disorders caused by both heritable diseases and genetic anomalies such as SNPs play a major role in the etiology and progression of ON due to genetic alterations in key components in the coagulation cascade including Protein C, Protein S, PAI-1, and a number of other factors [12–14, 16, 27, 30, 33–35, 72, 78, 93, 95–98]. Chen et al evaluated two Taiwanese pedigrees with familial autosomal dominant osteonecrosis of the femoral head.…”

“…Nontraumatic conditions associated with ON are numerous [1, 2, 6, 7]. Some of the well accepted and common associations include corticosteroid use [8, 9], alcohol abuse [10], systemic lupus erythematous [11], hemoglobinopathies including sickle cell anemia [12–14], Legg-Calve Perthes disease [15], and exposure to radiation [16] or cytotoxic agents [17]. Other less common associations include Gaucher's disease [11, 18, 19], dysbarisms [4], HIV [20], hyperlipidemia [21], pancreatitis, and gout [22].…”