Abstract
78. Atypical presentation of anti-signal recognition particle antibody positive myositis with profound extra-muscular features
Highlights
Autoantibodies against signal recognition particle (SRP) were first reported in the serum of a patient with polymyositis
Anti-SRP antibodies are a rare cause of myopathy; they are found in roughly 5% of all inflammatorymyopathies.Based onclinical observations,it isreported that anti-SRP antibodies are associated with the severe and rapidly progressive muscle weakness, marked elevation of creatine kinase (CK) and a necrotising myopathy on histology
Patients with anti-SRP positive myositis tend toshowagood initial responsetohigh dose steroid therapy butthe myositis tends to relapse leading to the requirement of high cumulative doses of steroid
Summary
Hypophosphatasia (HPP) is a rare metabolic bone disease caused by loss-of-function mutations in the gene encoding tissuenonspecific alkaline phosphatase (TNSALP). The patient reported episodes of sudden onset of joint pain and swelling, affecting the knees which was suggestive of a crystal arthritis These features were not typical of psoriatic arthritis, the diagnosis was re-evaluated. This patient does not qualify for this treatment despite symptoms which significantly affect her quality of life Given her personal and family history of psoriasis, it is possible that this patient may have coexisting HPP, CPPD and psoriatic arthritis. It was for this reason that an isotope bone scan was requested to determine the extent and pattern of joint involvement.
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