Individually, Gottron’s papules were seen in 91% (51/56) and heliotrope rash in 73% (36/49). Nailfold capillaroscopy
abnormalities were reported in 26 of 38 patients (68%). Calcinosis was not present in any patient at diagnosis (0/13); however, 18% (8/45) of patients with JDM had calcinosis documented during the course of the disease. Forty-four percent of chronic course patients (7/16) developed calcinosis compared with 4% of monophasic patients (1/21). No patient with polyphasic disease developed calcinosis. Dysphonia was documented in 14 patients and dysphagia in 11 patients at time of diagnosis. Throughout the course of the illness, 21 of 49 patients (43%) in whom there was adequate documentation had dysphonia and/or dysphagia. At presentation, arthritis PI3K inhibitor review was reported in 15 of 43 patients (35%) and NU7441 molecular weight contractures in 17 of 29 (59%). Of those
patients with contractures at onset, only five (29%) also had arthritis. Table 2 outlines the results of common investigations performed in the cohort. CK was the most frequently ordered muscle enzyme investigation (100% of patients) and was abnormal 65% of the time (37/57). Twenty patients had normal CK; four of these had no other enzyme measured and 16 had at least one other enzyme and this was abnormal in all cases. Aldolase was measured in only 10 patients and was abnormal in all. When measured, lactate dehydrogenase (LDH), aspartate aminotransferase (AST) and alanine aminotransferase (ALT) were abnormal 92% (23/25), 88% (29/33) and 58% (29/33) of the time, respectively. Two or more muscle enzymes were elevated in 65% of patients (37/57). Four patients (with only CK measured) had no abnormality
in muscle enzymes. All four demonstrated clinical weakness and supportive evidence of myositis with abnormal MRI, EMG or muscle biopsy. Erythrocyte sedimentation Tau-protein kinase rate (ESR) was elevated in 84% (46/55) of patients. Muscle biopsy was performed in 29 patients and was abnormal in 83% (24/29). EMG was performed on four patients and was abnormal in all patients. Figure 2 outlines the frequency of use of muscle biopsy, EMG and MRI in the diagnostic work-up of patients over the period studied. MRI was performed on a total of 29 patients and demonstrated signs of myositis in 97% (28/29). One patient with normal MRI had treatment with oral steroids prior to the MRI. Antinuclear antibodies (ANA) were tested in 52 patients and titres were abnormal (titre > 1 : 160) in 33 (63%) cases. High titre antibody to extractable nuclear antigen (ENA) was detected in only one patient (1/32, 3%) and was directed toward topoisomerase-I. Table 3 outlines therapy at diagnosis and throughout the disease course of the cohort. Fifty-one percent (29/57) of patients were treated with steroids alone (oral and/or high-dose pulsed methylprednisolone) at diagnosis, of whom 12 (20%) received this as their only treatment throughout their disease course. High-dose pulsed intravenous steroids were used in a total of 47 (82%) patients.