Giornale italiano di Dermatologia e Venereologia

Dermatomyositis (DM) is an idiopathic systemic inflammatory disease that is often accompanied by interstitial lung disease (ILD) or internal malignancy. New autoantibodies, anti-clinically amyopathic dermatomyositis 140 (anti-CADM-140) antibody (Ab) and anti-155/140 Ab, as well as anti-aminoacyl-tRNA synthetase (anti-ARS) Ab and anti-Mi-2 Ab, have been discovered and their utility indicated. However, the association between these autoantibodies and the clinical characteristics of DM is not fully understood, and it is unclear whether anti-155/140 Ab is "specific" to DM patients with internal malignancy. Therefore, we analyzed 55 DM patients and 18 non-DM patients with malignancy to evaluate the clinical characteristics, especially skin manifestations, in association with DM-specific autoantibodies detected by immunoprecipitation. Six patients (11%) had anti-CADM-140 Ab, nine (16%) had anti-155/140 Ab, eight (15%) had anti-ARS Ab and six (11%) had anti-Mi-2 Ab. The frequency of DM patients positive for any type of autoantibody was 53%. Among the 20 DM patients with ILD, three (15%) had both anti-CADM-140 Ab and rapidly progressive ILD, and required intensive therapy (P < 0.05). ILD found in anti-ARS Ab-positive patients did not progress rapidly. The prevalence of muscle involvement in patients with anti-CADM-140 Ab was 83%. Among the 18 DM patients with internal malignancy, four (22%) had anti-155/140 Ab, and internal malignancy was found in four cases (44%) of nine anti-155/140 Ab-positive patients. None of the non-DM patients with malignancy were positive for anti-155/140 Ab. In conclusion, the results of the present study indicate that anti-155/140 Ab is specific to DM patients with internal malignancy and that we may be able to predict prognosis of ILD and the presence of malignancy to some extent, suggesting that examination of autoantibodies in DM patients is clinically very useful. However, further investigation is needed because several findings differ from those of previous reports.

Analysis of dermatomyositis‐specific autoantibodies and clinical characteristics in Japanese patients

This study aims to investigate the prevalence and predictive risk factors of malignancy in patients with polymyositis (PM) and dermatomyositis (DM). The medical records of 192 PM/DM patients followed up in a medical center between January 2000 and December 2013 were reviewed. Among the 192 patients, 33 patients (17.2 %) had associated cancer. Both PM and DM are significantly associated with cancer, although the risk of cancer appears to be somewhat higher among patients with DM (23.0 %) than among those with PM (8.9 %). Nasopharyngeal cancer (30.3 %) and breast cancer (18.2 %) comprised the most common malignant diseases associated with PM/DM. Univariate analysis showed that an older age at PM/DM onset, heliotrope rash, Gottron's sign, dysphagia, and low creatine phosphokinase (CPK) level were associated with increased malignancy. Multivariate analysis revealed that independent predictors of malignancy in PM/DM were age >40 years at PM/DM onset (adjusted OR 3.44; 95 % CI 1.08-10.98; p = 0.037) and heliotrope rash (adjusted OR 2.96; 95 % CI 1.04-8.43; p = 0.042). During the follow-up period, 66 (34.4 %) patients died and the overall patient survival rates were 83.1 % at 1 year, 78.9 % at 2 years, 74.2 % at 5 years, and 65.5 % at 10 years. This study demonstrates a high frequency of malignancy (17.2 %) in DM/PM patients. Nasopharyngeal cancer and breast cancer were the most common cancer types in DM/PM patients in our study. Cancer screening should be offered to patients with newly diagnosed DM/PM. Moreover, all patients should be evaluated for the possibility of an underlying malignancy during treatment.

Malignancy in dermatomyositis and polymyositis: analysis of 192 patients

This study compared self-reported incidence and prevalence of dysphagia in inflammatory myopathy diseases with instrumental data from videofluoroscopy in a cohort of 18 patients with inflammatory myopathies (inclusion body myositis, polymyositis, and dermatomyositis). We found a high self-report of incidence of dysphagia and demonstrated that symptoms described by the patients follow a characteristic pattern. We conclude that there is a high incidence of dysphagia in all three of the inflammatory myopathies. Questions about swallowing should routinely be included in inflammatory myopathy patient examinations in order to appropriately refer patients for further investigation of their swallowing function and avoid the complications associated with dysphagia.

Dysphagia in Inflammatory Myopathy: Self-report, Incidence, and Prevalence

Dysphagia has previously been reported in the inflammatory myopathies (IMs): inclusion body myositis (IBM), dermatomyositis (DM), and polymyositis (PM). Patients report coughing, choking, and bolus sticking in the pharynx. Myotomy has been the treatment of choice, with variable success reported. We sought to determine underlying causes of dysphagia in IM patients using instrumental evaluation. Eighteen subjects participated in the study: four with DM, six with PM, and eight with IBM. They underwent simultaneous videofluoroscopy and manometry, yielding 214 swallows for analysis regarding function of the upper esophageal sphincter (UES), swallow initiation, hyolaryngeal excursion, and pharyngeal residue. Penetration and aspiration were also recorded. UES failed to relax in two participants. High incidence of pharyngeal dysphagia was noted; 72% of participants demonstrated abnormalities, including delayed swallow initiation (24%), decreased hyolaryngeal excursion (22%), pyriform residue (17%), and penetration (22%). Dysphagia in IM patients appears to be more due to impaired muscle contraction and reduced hyolaryngeal excursion than the often held belief of failed UES relaxation. The distinction between mechanisms causing patients’ dysphagia should be examined, particularly if CP myotomy is being considered as it may be contraindicated for patients with normal UES relaxation. More studies investigating IM patients pre- and post-myotomy are needed.

Pharyngeal dysphagia in inflammatory muscle diseases resulting from Iimpaired suprahyoid musculature

We retrospectively studied 132 patients with dermatomyositis; 84 had idiopathic, 30 paraneoplastic, 5 juvenile and 13 amyopathic forms of the disease. The commonest features were macular erythema, heliotropic erythema and Gottron's papules. Flagellate erythema occurred in 5% of patients with idiopathic dermatomyositis and correlated with the disease activity. Necrotic lesions were also found in this group of patients but did not always signal malignancy. The prevalence of malignancy was high (23%). Raynaud's phenomenon occurred in 10.6% of patients, also in those with malignancy. Dysphagia, interstitial lung disease and arthralgias affected 20%, 8% and 40% of patients, respectively. Anti-Jo-1 antibodies were found in 5% of patients with idiopathic dermatomyositis and low titre ANA in 1/3 of patients. ANA did not correlate with the disease activity. We confirmed the data from the literature, but no cutaneous sign, constitutional symptom or circulating antibody was found marking a particular subtype of the disease.

Dermatomyositis in 132 Patients with Different Clinical Subtypes: Cutaneous Signs, Constitutional Symptoms and Circulating Antibodies

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Dermatomyositis in 132 Patients with Different Clinical Subtypes: Cutaneous Signs, Constitutional Symptoms and Circulating Antibodies