Dermatomyositis: analysis of 109 patients surveyed at the Hospital das Clínicas (HCFMUSP), São Paulo, Brazil

Abstract

BACKGROUNDDermatomyositis affects striated muscles, skin and other organs.OBJECTIVETo characterize the disease from January 1992 to December 2002, assessing its classification, cutaneous and systemic manifestations, and also laboratory results, therapeutic and prognostic findings compared to those in the literature.METHODSData were obtained from medical records of 109 patients who were classified into five groups: 23 juvenile dermatomyositis; 59 primary idiopathic dermatomyositis; 6 amyopathic dermatomyositis; 7 dermatomyositis associated with neoplasms and 14 dermatomyositis associated with other connective tissue diseases.RESULTSSixty patients were classified as "definite" diagnosis; 33 as "possible"; four as "probable" and 12 and as amyopathic. The average age at diagnosis was 36 years. Cutaneous manifestations occurred in all patients; the most frequent symptom was loss of proximal muscle strength; the most common pulmonary disorder was interstitial lung disease, and gastritis was the most prevalent digestive manifestation. Tumors were documented in 6.42% of cases. Lactate dehydrogenase was the muscle enzyme most frequently elevated in the majority of cases. Skin biopsies were performed in 68 patients; muscle biopsies in 53; and electroneuromyographies in 58 patients. The most commonly used treatment was corticotherapy and the mortality rate was 14.7%.CONCLUSIONin this sample, the disease appeared in younger individuals, was more frequent in women and the association with cancer was small.

Clinically amyopathic dermatomyositis: clinical features, response to medications and malignancy‐associated risk factors in a specific tertiary‐care‐centre cohort

“…Although DM is classically associated with photosensitivity, several studies showed that only about 50% of patients are truly photosensitive. [31][32][33] This is similar to our observation, where about one-third of our patients were photosensitive (Table 1). Notably, whereas periungual erythema seemed to be protective against malignancy in our CADM cohort, it has been found to be associated with malignancy in classic DM.…”

“…Intriguingly, whereas previous clinical predictors of malignancy were not confirmed, photosensitivity ( P = 0·03) and periungual erythema ( P = 0·02) were associated with absence of malignancy in our cohort. Although DM is classically associated with photosensitivity, several studies showed that only about 50% of patients are truly photosensitive . This is similar to our observation, where about one‐third of our patients were photosensitive (Table ).…”

Inflammatory Muscle Disease, 15-Year Experience at Tertiary Centers

“…Rheumatoid factor was positive in (10.7%) of patients and this percentage was closed to previous study [53]. Though anti ds DNA and anticardiolipin antibodies were not evaluated in previous studies, it was positive in (10.7%) of our patients may be due to high number of overlap syndrome we had in this study.…”

Syndrome in question

“…The diagnosis of neoplasia may precede (40%), be concurrent (26%) or follow (34%) the clinical manifestations of DM. 2 , 7 , 8…”