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Death occurred in 10 out of 87 patients (seven with dermatomyositis predominantly dueto respiratory involvement and three with overlap). (Parkodi R et al 2002) .In anotherstudy, the overall mortality rate was 26.7% with infection and malignancy as the maincauses of death (Koh ET et al 1993).In one study consisting of 50 patients with 25 years follow up, cardiovascularchanges were disclosed in 82.3% of PM and 69.7% ofDM patients. Radiological signs ofinterstitial pulmonary fibrosis were noted more frequently in DM (36%) than in PM(23%). (Chwalinska et al1990)E) Final outcome in 84 cases:Grading Frequencies Marie! et al (2001): 77 casesIn percentages In percentagesImproving 4.4Partial recovery 14.4 43Worsened 3.8 17Asymptomatic 54.8 40Expired 9.5 22No final follow up 11.9Keily et al (2003)78 casesIn percentages3255112Table XIV: Comparison of final out come of the current study with the similarstudiesIn a study of 30 cases, Uthrnan E et al in 1996 found following results: The meanfollow-up was 62 months: 23 (77%) had their disease controlled, 3 (10%) patients werelost to follow-up, and 4 (13%) died (no death occurred because of IIM or its treatment).Therapy was discontinued because of remission in 5 (17%) patients. Cumulative survivalrates at 2, 5, and 10 years were 89%, 89%, and 85%, respectivelyH Lilley et al in his analysis of 77 patients with idiopathic inflammatory myopathyfound that partial ( 4 7%) or full (31%) recovery in most cases with no recovery of strength(9%) and death (11%) :ruos Get al found twenty-six patients (51%) achieving completeremission out of 50 cases. ·Relapse rate:Totally 19 patents had relapse in this study .In 12 patients there was increase in serumCPK value earlier than clinical worsening while tapering steroids.In another study consisting 77 cases, short term recurrences of PM/DM (during taperingof therapy) occurred in 36 patients and long term recurrences (after discontinuation oftherapy) in 9 patients. (Marie! et al 2001)33
Follow up:Mean follow up period was 7.8 years is available in 78 patients. During follow up, CPKstarted improving along with clinical improvement in majority of patients (3 8 out of 46patients).F) Prognostic factors:There was no statistically significant correlation between any pf the clinical orinvestigation parameters with the final outcome. However there was positive trend forgood prognosis with early age of onset, early presentation ( less than three months ) andambulation at the time of evaluation.H Lilley et al in his analysis of 77 patients with idiopathic inflammatory myopathyfound that routine statistical tests do not differentiate useful prognostic indices. Hencepolymyositis disability score was devised in. an attempt to gain some prognosticinformation. He found that onset before the age of 50 and duration of symptoms of lessthan 12 months prior to presentation were favourable prognostic features, and treatmentwith regimes other than steroid therapy alone a ·probable favourable indicator. Level ofcreatine kinase (CK) at presentation and histopathological separation of dermatomyositisor polymyositis failed to alter prognosis. In another study, Tymms KE et al, in his studyof 105 cases, found outcome was worse in older patients and in those where weaknessexceeded 4 months before diagnosisAirio et al (2006) in his study to determine prognostic factors in one hundred andseventy-six patients with PM and 72 patients with DM found that except for age in bothgroups and the delay in diagnosis in the PM group, no other individual factor reachedsignificance as a predictor of death. However, cancer had a hazard ratio (HR) of 2.16 fordeath (95% CI: 0.95-4.50) in the DM group and 1.99 (95% CI: 1.01-3.94) in the PMgroup. In another study consisting 77 cases, factors associated with PM/DM remissionwere younger age and shorter duration of clinical manifestations prior to therapyinitiation. Variables associated with poor outcome ofPM/DM were older age, pulmonaryand esophageal involvement, and cancer. (Marie! et al 2001)34
Page 1 and 2: Sree Chitra Tirunal Institute forMe
Page 3 and 4: PROJECT REPORTCLINICAL, ELECTOPHYSI
Page 5 and 6: CONTENTS1. INTRODUCTION2. REVIEW OF
Page 7 and 8: • IdiopathicClassification of the
Page 9 and 10: CLINICAL FEATURESMany inherited myo
Page 11 and 12: PolymyositisEvolution of weakness i
Page 13 and 14: The rate of progression is typicall
Page 15 and 16: Muscle biopsyPathological feature D
Page 17 and 18: controlled trial. These have includ
Page 19 and 20: AGE DISTRIBUTION• < 18yrs|»18-50
Page 21 and 22: Deep tendon reflexes2 40S 30° 201
Page 23 and 24: EMG• Myopathic• Neurogenic• N
Page 25 and 26: Picturel9: Light microscopy: Histop
Page 27 and 28: AIMS AND OBJECTIVES OF THE STUDY1.
Page 29 and 30: Diagnostic Criteria for Inclusion B
Page 31 and 32: L ower r 1m b prox1m . al we akn es
Page 33 and 34: Final diagnosis:DiagnosisFrequencie
Page 35 and 36: Prognostic factors: There was no st
Page 37 and 38: cases. Other common symptoms includ
Page 39: 5 (17%) liM with neoplasia (lymphom
Page 43 and 44: REFERENCESl.Amato AA et al. Inclusi
Page 45 and 46: Clinical, electrophysiological and
Page 47 and 48: 5.4 Not treated6.0Follow-up: 3 mont
Page 49 and 50: 1 0.6 ESR.1. Improved 2 .stationary

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