| ObjectiveExplore the clinical features of patients with polymyositis (PM) retrospectively ,to provide evidence for clinical diagnosis,treatment and prognostic evaluation.MethodsThe 40 patients of this research were all diagnosised as PM or necrotic myopathy by muscle biopsy combined with clinical manifestation from 2000.3 through 2007.10. They were followed up by telephone or return visiting. 4 patients,who were initially diagnosed as necrotic myopathy ,were interpreted as progressive myodystrophy. 6 patients ,with the possible diagnosis of PM,were lost. Clinical data of other 30 cases was obtained. We analyzed the diagnosis, treatment and prognosis of this 30 cases .Statistic analysis:All available datas have been analyzed by SPSS 13.0 (p>0.05) .Results1 .Clinical ManifestationsIn 30 PM patients,the average age at diagnosis was (43±17) years (range, 17～77 years), and 10 were men(M:F=1:2). Disease duration was 1 month～10 years before biopsy ,with 23 cases >6 months (76.6%) . The follow up time were 0.5～7.5 years.At the onset, proximal limb muscles weakness was in majority (23 cases),whilefever (2 cases) ,myalgia (2 cases) ,arthralgia (1 case), dysphagia (1 case) and atonia masticatoria (1 case) were in the minority. Whereas, in the whole disease progression, proximal limb muscle weakness was in all, neck muscle weakness and dysphagia (18 cases) were common,and malignancy was found in none until the last follow-up.2.Blood Tests 2.1 Serum CK LevelThe CK level rised in 29 patients, 145～15306IU/L (normal value 38～174 IU/L) . The CK level was >10000IU/L in 3 cases , 5000～10000IU/L in 11 cases, 1000～5000IU/L in 14 cases, < 1000IU/L in 2 cases. It is defined as "significant decline" when it declines at lest 1/3 . The CK level presents significant decline in 25 patients who were treated standardly,20 whthin 1 month,23 whthin 2 months and all of 25 whthin 6 months.2.2 Autoimmune Serologic MarkersThe ESR of 16 patients elevated. ANA and Rheumatoid factor of 4 patients were positive eachly.3. Muscle Biopsy FindingsThere are inflammatory cellular infiltration in 19cases(63.3%) and no in 11cases(36.7%).There are different variations in fiber size. Necrotic fibers, regenerating fibers and phagocytes can be seen in all cases. Interstitial connective tissue is increased in 25 cases(83.3%).Fibers with centrally placed nuclei are increased in 6cases(20%). All do not presence cluster atrophy or necrosis.4.Treatment and prognosis15 patients just accepted prednisolone (1 mg/kg/d, Max≤60mg/d), and the other 15 patients were in combination with other drugs: intravenous methyl- prednisolone pulses, Methotrexate (MTX), Cyclophosphamide (CyC), IVIG. All patients taked some measures to avoid side effects, such as protecting gastric mucosa, supplying potassium and calcium. The power of at least one main involved muscle group increasing no less than 1 grade is significant. Symptoms of 25 patients were improved. Another 2 patients who had not accepted the standardized treatment were not improved. 4 cases relapsed,with reccurrence reasons of sudden treatment- stop in 1, too fast PDN reduction in 1, maintenance dose-stop in 2. 3 cases died.In 25 patients with standardized treatments, 19 symptoms were improved significantly (76%) in 2 months , of whom the group treated with PDN alone and the one also added other drugs have the similar efficacy.In three months 21 patients' symptoms were improved significantly (84%). 25 patients 'symptoms were all improved significantly. There was no obvious difference between the two groups with or without inflammatory cells.Side effects: PDN: central obesity(24 cases) , gastric discomfort (2 cases).MTX and CyC : nausea and vomiting (1 case) .No femoral head necrosis, myelosuppression, liver function demagement and other severe side effects. No patient stopped treatment for side effects. 15 patients treated with PDN alone with 10 patients also added other drugs had the similar side effects.Conclusion1 .Endomysial inflammation with invasion is not the necessary condition for the diagnosis of PM. Patients who were primary diagnosed as necrotizing myopathy (PM could not be excluded) by clinical and pathological findings can be diagnosed more definitely by following up their disease progression and efficacy.2. The study indicates that PM patients onseting before 17 years old are rare,and patients before this age with necrotizing myopathy had the high possibility of myodystrophy.3. Serum muscle enzymes monitor is of great significance to PM diagnosis, assessment of curative effect and PDN reduction.4. In the primary treatment ,PND should be adequate (1mg/kg/d, Max≤60 mg/d) , and the method of PDN-gradual-reducing can keep efficacy and prevent replase effectively. If a patient has a poor response to therapy with corticosteroid alone or has severe clinical manifestations, the introduction of intravenous methylprednisolone pulses, immunosuppressive agents or IVIG is suggested,which will probablely produce good efficacy. Our research indicates that Applying measures to prevent side effects is Effective.By regular treatment,PM patients can have good prognosis. |
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