Mechanic’s hands and hiker’s feet in antisynthetase syndrome

Objective.To analyze the characteristics, outcomes, and predictive factors of disease-modifying antirheumatic drug (DMARD) use in 48 patients with antisynthetase syndrome [characterized by myositis, interstitial lung disease (ILD), arthritis, Raynaud’s phenomenon (RP), and/or mechanic’s hands] and the presence of anti-histidyl-transfer RNA synthetase (anti-Jo1) autoantibodies.Methods.Forty-eight patients (33 women, 15 men) who were anti-Jo1-positive referred to one center between 1998 and 2008 were analyzed retrospectively.Results.The median age of disease onset was 43 years [interquartile range (IQR) 33–53 yrs]. The median followup was 5 years (IQR 2–8 yrs). At diagnosis, 81% of patients presented with myositis, 80% ILD, 77% arthralgia, 48% RP, and 21% mechanic’s hands. During the followup, 14 patients (29%) had no need for DMARD, while 34 (71%) required DMARD. Patients with mechanic’s hands (p = 0.02) and higher creatine phosphokinase at diagnosis (median 6070 IU/l vs 1121 IU/l; p = 0.002) were more likely to need DMARD. ILD, noted on computed tomography scan by a nonspecific interstitial pneumonia score, was lower in the group of patients with no DMARD need (4 vs 7; p = 0.04). Twenty patients (44%) presented with a pulmonary aggravation (worsening of radiologic score of ILD and/or pulmonary function test results) leading to DMARD use. Nonspecific interstitial pneumonia score (7 vs 5; p = 0.05) and total lung volume (57.5% vs 70%; p = 0.02) values predicted pulmonary aggravation.Conclusion.Our study outlines the burden of chest involvement for the prognosis of antisynthetase syndrome in terms of patients’ requirement for DMARD therapy.

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Mechanic’s hands in a woman with undifferentiated connective tissue disease and interstitial lung disease – anti-PL7 positive antisynthetase syndrome: a case report

Journal of Medical Case Reports ◽

10.1186/s13256-015-0571-2 ◽

2015 ◽

Vol 9 (1) ◽

Cited By ~ 1

Author(s):

Ellen De Langhe ◽

Jan Lenaerts ◽

Xavier Bossuyt ◽

Rene Westhovens ◽

Wim A Wuyts

Keyword(s):

Case Report ◽

Interstitial Lung Disease ◽

Connective Tissue ◽

Lung Disease ◽

Connective Tissue Disease ◽

Undifferentiated Connective Tissue Disease ◽

Antisynthetase Syndrome ◽

Mechanic’S Hands

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The clinical manifestations at the onset of antisynthetase syndrome: A chameleon with multiple faces

Reumatismo ◽

10.4081/reumatismo.2020.1275 ◽

2020 ◽

Vol 72 (2) ◽

pp. 86-92

Author(s):

A.C.C.D. Baccaro ◽

G.L. Behrens Pinto ◽

R.C.S. Carboni ◽

S.K. Shinjo

Keyword(s):

Interstitial Lung Disease ◽

Lung Disease ◽

Clinical Symptoms ◽

Raynaud’S Phenomenon ◽

Clinical Manifestations ◽

Raynaud's Phenomenon ◽

Joint Involvement ◽

Antisynthetase Syndrome ◽

Initial Symptoms ◽

Mechanic’S Hands

The antisynthetase syndrome (ASS) is clinically characterized by fever, myositis, interstitial lung disease, joint involvement, mechanic’s hands, or Raynaud’s phenomenon, and the presence of antisynthetase autoantibodies. These clinical manifestations may not occur simultaneously. Therefore, the aim of this study was to analyze the sequence in which these clinical manifestations can develop at the onset of ASS. This retrospective, single-center cohort study enrolled 55 ASS patients. Their mean age at the onset of ASS symptoms was 42.3±11.8 years. There was a predominance of female patients (75.9%) and white patients (72.7%). At initial presentation, 41.8% of the patients had fever, 43.6% had joint symptoms, 38.2% had myositis, 36.4% had interstitial lung disease, 18.2% had Raynaud’s phenomenon, and 16.4% had mechanic’s hands. Subsequent clinical symptoms emerged at varying time points. In two out of 55 cases, joint, muscle, and lung manifestations developed simultaneously. The median time between the onset of symptoms and the complete ASS clinical manifestation was 19.9 (4.0-60.2) months; whereas, the timeframe between the onset of symptoms and the ASS diagnosis was 29.0 (11.0-63.0) months. The confounding misdiagnoses interfering with the initial diagnosis were polymyositis (52.7%), dermatomyositis (29.1%), nonspecific interstitial pneumopathy (23.6%), rheumatoid arthritis (18.2%), and others (10.9%). Clinical features at the onset of ASS are highly variable. Consequently, confounding factors can lead to significant delays for the final and definitive diagnosis of ASS. Therefore, ASS should be considered a differential diagnosis in patients with initial symptoms of joint, lung, and/or muscle involvements, as well as fever, mechanic’s hands, and/or Raynaud’s phenomenon manifestations.

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Successful treatment of refractory mechanic’s hands with ustekinumab in a patient with the antisynthetase syndrome

Rheumatology ◽

10.1093/rheumatology/kez020 ◽

2019 ◽

Vol 58 (7) ◽

pp. 1307-1308 ◽

Cited By ~ 2

Author(s):

Iago Pinal-Fernandez ◽

Christopher T Kroodsma ◽

Andrew L Mammen

Keyword(s):

Successful Treatment ◽

Antisynthetase Syndrome ◽

Mechanic’S Hands

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Widespread Mechanic's Hands in Antisynthetase Syndrome With Anti-OJ Antibody

The Journal of Rheumatology ◽

10.3899/jrheum.201043 ◽

2021 ◽

pp. jrheum.201043

Author(s):

Koichiro Shinoda ◽

Yasuhito Hamaguchi ◽

Kazuyuki Tobe

Keyword(s):

Antisynthetase Syndrome ◽

Ulnar Side ◽

Radial Side ◽

Mechanic’S Hands

Mechanic's hand is a representative manifestation of antisynthetase syndrome (AS) but is observed in other forms of dermatomyositis1. This is a nonpruritic, hyperkeratotic, and scaly eruption on the ulnar side of the thumb and radial side of other fingers; occasionally the palms, fingertips, and feet are involved. However, a widespread type affecting the whole palm and fingers is extremely rare in anti-OJ antibody.

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The relevance of anti-Jo-1 autoantibodies in patients with definite dermatomyositis

Advances in Rheumatology ◽

10.1186/s42358-021-00171-x ◽

2021 ◽

Vol 61 (1) ◽

Author(s):

Vanessa Posener de Andrade ◽

Fernando Henrique Carlos De Souza ◽

Gustavo Luiz Behrens Pinto ◽

Samuel Katsuyuki Shinjo

Keyword(s):

Death Rate ◽

Retrospective Cohort ◽

Disease Duration ◽

Immunosuppressive Drugs ◽

Joint Involvement ◽

Antisynthetase Syndrome ◽

Tertiary Center ◽

Mechanic’S Hands ◽

Commercial Kit

Abstract Background To assess the prevalence and clinical relevance of anti-Jo-1 autoantibodies in a representative sample of patients with definite dermatomyositis (DM). Methods This retrospective cohort study took place from 2005 to 2020 and assessed 118 adult patients from a tertiary center who were diagnosed with definite DM. A commercial kit was used to detect anti-Jo-1 autoantibodies. Results The presence of anti-Jo-1 autoantibodies was observed in 10 out of 118 (8.5%) patients with definite DM. The following variables were comparable between individuals with and without anti-Jo-1 autoantibodies: age at diagnosis, sex, ethnicity, disease duration, follow-up period, recurrence rate, complete clinical response, death rate, and cancer incidence. There was no difference in clinical features between groups, except for an increased prevalence of “mechanic’s hands,” joint involvement, and lung disease, as well as a reduced occurrence of skin findings in patients positive for anti-Jo-1 autoantibodies. No anti-Jo-1-positive patients went into remission; they required greater use of glucocorticoids and immunosuppressive drugs. Conclusions Anti-Jo-1 positivity was found in 8.5% of patients with definite DM. This autoantibody was associated with an antisynthetase syndrome phenotype and might predict clinical outcomes in patients with definite DM.

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