Myositis-related interstitial lung disease and antisynthetase syndrome
. J Bras Pneumol 2011; 37: 100-9.
Clinical Spectrum Time Course in Anti Jo-1 Positive Antisynthetase Syndrome
: Results From an International Retrospective Multicenter Study.
Clinical manifestations of antisynthetase syndrome
positive for anti-alanyl-tRNA synthetase (anti-PL12) antibodies: a retrospective study of 17 cases.
We present a 69-year-old female with antisynthetase syndrome
(ASTS) presenting with myalgia, arthritis, interstitial lung disease, mechanics hands, and positive Jo-1 antibody requiring multiple courses of prolonged immunosuppression with corticosteroids, azathioprine, mycofenolate mofetil, and rituximab.
Rituximab has been reported in previous case reports and case series in the management of ILD related to antisynthetase syndrome
. (2,3,5,6,8) Data of cases with negative antisynthetase serology is sparse.
 These include Raynaud's phenomenon, peri-ungual swelling or erythema, 'raggedy' cuticles or other features of the antisynthetase syndrome
. This syndrome refers to a patient with PM or DM with the typical findings of constitutional symptoms such as fever, polyarthritis, mechanic's hands (Fig.
This fact confers relevant significance on anti-Ro52 as a costimulatory autoantibody, a concept reported in patients with antisynthetase syndrome
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." It is thought to have a negative association with malignancy.
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.
It was also suggested that RTX could be useful for severe ILD in antisynthetase syndrome
[12, 13]; meanwhile, there are only a few case reports in which RTX was used in anti-MDA5-Ab-positive DM with ILD [14-18].
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).
and malignancy: our experience.