9) We report another case of DM--ILD with negative serology for antisynthetase syndrome that was successfully managed with rituximab with no other immunosuppressive agents.
Gabay, Rituximab in the treatment of antisynthetase syndrome.
Rituximab treatment in a case of antisynthetase syndrome with severe interstitial lung disease and acute respiratory failure.
6] These include Raynaud's phenomenon, peri-ungual swelling or erythema, 'raggedy' cuticles or other features of the antisynthetase syndrome.
The anti-Jo-1 antibody test, although not sensitive, is relatively specific for interstitial lung disease and the antisynthetase syndrome mentioned above.
The antisynthetase syndrome (AS) represents a subset of connective tissue disease (CTD), usually dermatomyositis and polymyositis, in which autoantibodies to specific aminoacyl-transfer RNA synthetases develop and are associated with clinical manifestations of myositis, Raynaud phenomenon, arthritis, fever, mechanic's hands, and interstitial lung disease (ILD).
In summary, the anti-KS AS is one of the more subtle antisynthetase syndromes, with more pronounced respiratory complications and often subtle musculoskeletal manifestations.
is characterized by the presence of antihistidyl tRNA synthetase (Jo-1) antibody, interstitial lung disease (ILD), Raynaud's phenomenon, arthralgia/ arthritis and "mechanic's hands.
Recognition of "mechanic's hands," periungual erythema, abnormal capillary microscopy, and inflammatory arthritis led to new diagnoses including antisynthetase syndrome
, systemic sclerosis, rheumatoid arthritis-associated interstitial lung disease, mixed connective tissue disease, dermatomyositis.
and malignancy: our experience.