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PubMed1989

[Plasma exchange in dermatomyositis. A retrospective study of 21 cases].

We have reviewed the results obtained in 21 dermatomyositis patients who were treated with plasma exchanges (PE) in 8 french centres between 1980 and 1986. Patients and methods. Seven of the 21 patients studied were male and 14 were female; 16 were children under 15 years of age. The disease was initially acute in 17 cases, subacute in 3 cases and chronic in 1 case. Plasma exchanges were performed as first-line therapy in 13 patients and after failure of the usual treatments in 18 patients. The decision to use PE therapy was prompted by an increase in muscle weakness in 14 of these 18 patients and by a lack of improvement despite treatment in the remaining 4 patients. PE therapy was started 17 months on average after the beginning of treatment. At the time 10 patients were under systemic corticosteroid therapy (prednisone or prednisolone greater than 1 mg/kg/day in 7 cases); 7 patients were receiving immunosuppressants jointly with corticosteroids (prednisone or prednisolone greater than 1 mg/kg/day in 5 cases); and 1 patient had systemic corticosteroids (3 mg/kg/day), methotrexate and antilymphocyte serum. In 3 patients, 2 of whom had severe muscle weakness. PE's were performed from the start. Our 21 patients underwent a total of 234 plasma exchanges. Each patient had a mean series of 11 +/- 6 PE's spread over 11 +/- 3 weeks. In addition to PE therapy, 12 patients received corticosteroids (greater than 1 mg/kd/day in 7 cases) and 8 had corticosteroids (greater than 1 mg/kd/day in 5 cases) together with immunosuppressants. One patient who was put on TE therapy from the start received no other treatment. It must be noted that in 12 patients the introduction of PE was accompanied by another therapeutic change.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1989

Predisposing factors in polymyositis-dermatomyositis: results of a nationwide survey.

Utilizing case-control methods, we surveyed 322 persons who experienced the onset of polymyositis-dermatomyositis (PM-DM) during calendar years 1985 and 1986, in an effort both to test specific hypotheses, and to generate new hypotheses regarding antecedent risk associations for the disease. Data obtained by recall of events during the 12 months before disease onset in cases, and an identical time period in sex matched sibling controls, suggest that important questions for further study include the roles of antecedent heavy muscular exertion and emotional stress as risk factors for PM-DM. Furthermore, it appears that vaccinations, toxic exposures and symptoms of allergic phenomena, throat infection, and upper respiratory infection are either neutral or are negatively associated with risk of PM-DM, findings which also raise important new etiologic questions, or perhaps lay old ones to rest. Malignancy was infrequently noted.

Adult↗
PubMed1985

Retinopathy of dermatomyositis.

A recent case of childhood dermatomyositis with striking retinopathy is reported. The patient's vision was initially at the 20/80 to 20/100 level with associated macular edema and extensive cotton-wool infarction of the retina. Visual recovery to the level of 20/20 in both eyes was achieved following treatment with high-dose corticosteroids.

Child, Preschool↗
PubMedDate not supplied

[The heart in dermatomyositis and polymyositis ].

UNLABELLED: We studied eleven consecutive patients: eight with Dermatomyositis (DM) and three with Polymyositis (PM) from the cardiological point of view through non invasive methods. Nine patients (82%) had some kind of cardiopulmonary complications as shown by any of the used methods. SYMPTOMS: eight (73%) referred some kind of cardiopulmonary symptoms, mainly dyspnea; Physical examination; in seven (64%) was abnormal, detecting increased second pulmonary sound in four (36%), findings of mitral valve prolapse (MVP) in two (18%) and in two (18%) S3 gallop; Electrocardiogram: in seven (64%) was abnormal; six (55%) had some kind of heart enlargement corresponding four (36%) to right atrial or ventricular hypertrophy (RAH & RVH) and two (18%) to left ventricular hypertrophy (LVH), three (27%) had incomplete or complete right bundle branch block, one (9%) had bifascicular block and one (9%) left anterior hemiblock. Two (18%) had sinus tachycardia and two (18%) atrial premature contractions; d) chest ray: six (55%) were abnormal, among them, three (27%) had pulmonary fibrosis, three (27%) had RAH and/or RVH, two (18%) had LVH and one (9%) pericardial effusion; e) Echocardiogram: was abnormal in eight (73%), corresponding three (27%) to RVH, three to MVP which has been considered rare, in two (18%) congestive cardiomyopathy, in two (18%) pericardial effusion and in one (9%) type "A" paradoxical septal movement.

Adult↗
PubMed1988

[Polymyositis and dermatomyositis in childhood].

Authors report clinical and laboratory findings, treatment and evolution of six girls and three boys aged between 20 months and 13 years, diagnosed of juvenile polymyositis-dermatomyositis in the last seven years. Presenting symptoms were asthenia and proximal muscle weakness; in 3 cases characteristic skin lesions were associated. All were treated initially with prednisone p.o. (1-2 mg/kg/day) response being favourable in seven. Two patients with chronic evolution were treated with methotrexate and IV bolus of methylprednisolone.

Adolescent↗
PubMed1988

Relationship between serum creatine kinase level and corticosteroid therapy in polymyositis-dermatomyositis.

Few guidelines exist for the use of corticosteroid therapy in polymyositis-dermatomyositis (PM-DM). We retrospectively examined the relationship between serum creatine kinase (CK), muscle strength and the dosage and method of administration of prednisone in 30 patients with PM-DM observed monthly for a minimum of one year. Forty-two corticosteroid treated episodes of proximal muscle weakness associated with CK elevations formed the final study group. Each patient course was designated as having a good or poor biochemical and clinical outcome based on predetermined criteria. Adherence to 3 principles predicted a favorable biochemical and clinical outcome in the treatment of myositis: (1) administration of an adequate initial (loading) corticosteroid dose; (2) continuation of the initial dose until or after the time that the serum CK had become normal; and (3) a slow corticosteroid taper rate. Achievement of a CK within the low normal range predicted a prolonged biochemical remission, and a rise of CK within the normal range signalled a subsequent biochemical and clinical relapse. Tapering the corticosteroid dose when the CK was elevated frequently resulted in a further increase in CK. These observations allowed us to develop practical guidelines for the management of PM-DM.

Adolescent↗
PubMed1988

Coexistence of Paget's disease and dermatomyositis.

The occurrence of Paget's disease and dermatomyositis in a single patient has not been previously reported. We present 2 patients with this combination of disorders and consider why these diseases might coexist.

Dermatomyositis↗
PubMed1988

[Toxoplasma infection in dermatomyositis].

A 31-year-old woman with long-standing amyopathic dermatomyositis developed a maculopapular rash resembling erythema multiforme in some areas, accompanied by lymphadenopathy and malaise. An acute infection with Toxoplasma gondii was found to be the cause of clinical symptoms that mimicked an exacerbation of the underlying condition.

Adult↗
PubMed1988

[Value of cyclosporin A in dermatomyositis in a child].

Cyclosporin A was used in a 3 1/2 year-old child with dermatomyositis exhibiting severe junctional impairment and intolerance to steroids. After a few weeks, marked clinical improvement was observed allowing a reduction of the doses of steroids. After 9 months, the clinical status was satisfactory: the calcifications had lost their inflammatory characteristics. No side effect of cyclosporin A was observed at the dose used (4.4 mg/kg/d) which maintained effective blood concentrations between 80 and 220 ng/ml.

Child, Preschool↗
PubMed1988

Acute cricopharyngeal obstruction in dermatomyositis.

We report a case of a 44-year-old woman with dermatomyositis complicated by acute total cricopharyngeal obstruction resistant to corticosteroid therapy. This condition was successfully resolved by cricopharyngeal myotomy.

Adult↗
PubMed1987

Damaging effect of peripheral mononuclear cells of dermatomyositis on cultured human skin fibroblasts.

When human skin fibroblasts were incubated with mononuclear cells (MNC) from 6 patients with dermatomyositis (DM), striking attachment of MNC around fibroblasts was observed. Simultaneously, cell number and 3H-thymidine incorporation of fibroblasts after 4 days of incubation were significantly decreased. MNC of normal subjects, of patients with systemic lupus erythematosus, myasthenia gravis, rheumatoid arthritis, and other miscellaneous diseases with skin manifestations did not show this effect. MNC of patients with DM did not adhere to KYM-1 cells (human rhabdomyosarcoma cell-line) and did not inhibit cell proliferation of these cells. Our study strongly suggests that cell mediated injury of fibroblasts might play an important role in the pathogenesis of DM.

Adult↗
PubMed1987

[Dermatomyositis in children: clinical course of 9 cases].

Eight cases of dermatomyositis and one of polymyositis were studies among five male and four female patients. All had criteria definitive of the disease. All nine patients were give prednisone; six of them responded favorably to treatment and after discontinuing still remain asymptomatic. Three male patients have had a worse response to therapy, two of them deceased and the third still remains symptomatic, but very slowly improving after fourteen months with steroids and cyclophosphamide. These three patients have had chronic cutaneous vasculitic ulcers and this is a sign of bad prognosis. Both deceased patients exhibited important visceral complications (digestive and pulmonary). A more aggressive therapy has been suggested in these forms of worse clinical evolutions, including use of immunosuppressors.

Child↗
PubMed1987

Interstitial lung disease in dermatomyositis: clinicopathological study.

We studied 14 cases of polymyositis-dermatomyositis (PM-DM) and found roentgenographic evidence of interstitial lung disease in 9 patients with DM (64.3%). Serum anti-Jo-1 antibody was demonstrated in only one case. Histologically, interstitial pneumonitis and fibrosis were noted in all cases. Deposition of immunoglobulins along the alveolar interstitium was not found in any case. Response to corticosteroid therapy was poor, and 6 cases (67%) succumbed to respiratory failure and died. We conclude that interstitial lung disease in DM occurs more frequently than previously reported and may be an important prognostic factor.

Adult↗
PubMed1987

Human muscle-derived, tissue specific, myocytotoxic T cell lines in dermatomyositis.

Mononuclear cells were isolated from the inflamed muscle tissue of a patient suffering from dermatomyositis (DM). These were expanded in long-term culture and maintained in the presence of IL-2 containing culture medium. Two cell lines were established, one of the helper/inducer (OKT4+) and the other of the suppressor/cytotoxic phenotype (OKT8+). The OKT4+ cell line exhibited a non HLA-restricted, tissue-specific, myocytotoxic effect on rat muscle cell culture. Its lymphoproliferative response to human muscle antigen was HLA-restricted. The OKT8+ cell line exhibited a non HLA-restricted, tissue-specific response to muscle antigens and no myocytotoxic activity in in vitro rat muscle cell culture. It is likely that clones of OKT4+ lymphocytes in patients suffering from DM are associated with the pathogenesis of the disease--they probably mediate the diffuse damage to skeletal muscle through their myocytotoxic activity.

Aged↗
PubMed1986

[Study of the circulating lymphocytes populations in systemic scleroderma, chronic discoid lupus erythematosus and dermatomyositis].

The authors studied the peripheral blood lymphocytes in any autoimmune diseases. In scleroderma the T-lymphocytes and ratio T4/T8 are decreased and B-lymphocytes are normal. In the LEDC the T-lymphocytes are decreased and B-lymphocytes are increased. In dermatomyositis the B-lymphocytes are increased and T-lymphocytes are decreased, the ratio T4/T8 is normal.

Chronic Disease↗
PubMed1986

Serum enzyme, myoglobin and muscle strength relationships in polymyositis and dermatomyositis.

Strength of the quadriceps and hamstring groups was biomechanically assessed in terms of isometric torque production in 14 patients with inflammatory myopathy. Eleven had polymyositis and 3, dermatomyositis. Determinations of serum creatine kinase, lactate dehydrogenase, transaminase, and myoglobin were simultaneously obtained over an average period of observation of 1.8 years. In certain individual patients, there were significant correlations between laboratory indices and strength during the entire course of illness. In others, this was not the case. In the total group of patients, absolute values of the laboratory indices did not correlate well with strength except in the case of serum myoglobin, where there a significant inverse relationship. Logarithmic transformations of the laboratory data increased the inverse correlations. High strength and low myoglobin were related to high prednisone dose. Since laboratory guides are not always related to disease activity, quantitative assessment of muscle strength is necessary.

Adult↗