Musculoskeletal manifestations of the antiphospholipid syndrome

Abstract

The scope of clinical and laboratory manifestations of the antiphospholipid syndrome (APS) has increased dramatically since its discovery in 1983, where any organ system can be involved. Musculoskeletal complications are consistently reported in APS patients, not only causing morbidity and mortality, but also affecting their quality of life. We reviewed all English papers on APS involvement in the musculoskeletal system using Google Scholar and Pubmed; all reports are summarized in a table in this review. The spectrum of manifestations includes arthralgia/arthritis, avascular necrosis of bone, bone marrow necrosis, complex regional pain syndrome type-1, muscle infarction, non-traumatic fractures, and osteoporosis. Some of these manifestations were reported in good quality studies, some of which showed an association between aPL-positivity and the occurrence of these manifestations, while others were merely described in case reports.

Isolated osteonecrosis revealing primary antiphospholipid syndrome

“…The musculoskeletal manifestations reported during APS included: arthritis/arthritis, bone narrow necrosis, muscle infarction, complex regional pain syndrome type-1, non-traumatic fractures, avascular necrosis of bone, and osteoporosis. 4,6,9 Orthopedic involvement associated with APS is still under-recognized and not well documented feature of this syndrome. 6,7,10 It is therefore described as "novel" and "unusual" by the majority of authors.…”

“…18 Bone infarction associated with primary APS can be uni-, bilateral, or multiple (involving three or more bone anatomic sites). 4,7,8,12,17,19 Several sites of avascular osteonecrosis have been reported as sporadic cases during this syndrome: 4 vertebrae, 16 flat bones of the feet and wrists, 15 femurs, 7,8,15,17 tibia, 8,17 humerus, 7 and ribs. 20 The revealing forms of the disease remain exceptional.…”

“…7,12,19 Localized pain is the main symptom of osteonecrosis but this infarction can remain totally asymptomatic and would be incidentally discovered on imaging. 4,7,21 MRI is the gold standard for the detection of bone infarctions associated with primary APS. 21 The treatment is in most cases conservative, based on hydration, effective anticoagulation, and systemic corticosteroids.…”

“…Its incidence is estimated at 5/100,000 person-years and its prevalence at 40-50/100,000 people. 2,3 The clinical manifestations of this syndrome are highly polymorphic and all tissues and organs can be affected [2][3][4] making its diagnosis a real challenge for clinicians. 5 The orthopedic complications associated with primary APS are very rare and little known.…”

Antiphospholipid (Hughes) syndrome: insights for orthopedics

“…Grossly, musculoskeletal complications of APS 2 may be classified into three categories: pain/inflammation: arthralgia, complex regional pain syndrome type-1 (reflex sympathetic dystrophy), arthritis; necrosis: avascular necrosis of bone, bone marrow necrosis, muscle infarction; fragility: osteoporosis, non-traumatic fractures. …”