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PubMedDate not supplied

Polymyositis-dermatomyositis--a 25-year follow-up of 50 patients (analysis of clinical symptoms and signs and results of laboratory tests).

Clinical symptoms and signs and the frequency of abnormalities in the results of enzymatic, electromyographic, histological and serological tests were analysed in 50 patients, including 17 with polymyositis (PM) and 33 with dermatomyositis (DM), groups I and II according to Bohan's classification and followed-up for 25 years. Weakness of the proximal muscles of the extremities was present in both groups in all patients and in a high proportion of cases weakness involved also the distal muscles. Dysphagia was more frequent in DM patients (54.5%) than in PM (17.6%). In DM erythema and facial oedema as well as Gottron's sign were observed more frequently. In 11.8% of PM patients and 15.1% of DM patients deposition of calcium salts in subcutaneous tissue developed. Signs of vasculitis were found in 39.4% of DM cases and 17.6% of PM cases. In one-third of PM and in only 3 DM cases pitting oedema of the distal parts of the extremities was noted. Cardiovascular changes were disclosed in 82.3% of PM and 69.7% of DM patients. On the other hand, radiological signs of interstitial pulmonary fibrosis were noted more frequently in DM (36%) than in PM (23%). Increased serum activity of CPK, AspAT and ALAT was present with similar frequency in both groups from 71% to 81% of cases. EMG showed evidence of primary muscular changes in all PM patients and 69.6% of DM patients. Histological examination confirmed the diagnosis in the studied patients. Antinuclear antibodies were found relatively rarely, from 2% to 24% of PM/DM patients.(ABSTRACT TRUNCATED AT 250 WORDS)

Antibodies, Antinuclear↗
PubMedDate not supplied

Polymyositis-dermatomyositis:25 years of follow-up of 50 patients disease course, treatment, prognostic factors.

Therapeutic results were analysed in 50 patients, including 17 with polymyositis (PM) and 30 with dermatomyositis (DM). All patients were treated with prednisone (Encorton, Polfa) and 47% of patients with PM and 73% with DM required combined treatment with prednisone and cytostatic agents, mostly with cyclophosphamide (Endoxan G.D.R.). Long-term treatment monitored with the clinical status led to remissions persisting after treatment discontinuation in 17.6% of PM patients and 24.2% of DM patients. In 64.7% of PM patients and 57.6% of DM patients regression of the clinical signs of the disease was achieved but these patients required further treatment. No information was obtained on 5 cases. Four DM patients died. The cumulative 17-year survival rate of PM patients was 100%, and that of DM patients was 78.8% . In 45 follow-up cases, the mortality was 8.9%. Side effects of the combined treatment included most frequently mucosal candidiasis (22.2%), transient leucopenia (14.8%) and recurrent respiratory tract infections (11.1%). In 5 cases liver biopsy was done and histological examination disclosed moderate fatty, glycogen and vacuolar degeneration of the hepatocytes. The statistical analysis of prognostic factors such as the diagnosis of PM or DM, sex, age at disease onset, disease duration till diagnosis establishing and beginning of treatment, fever, dysphagia, circulatory and respiratory changes, leucocytosis nad ESR at the beginning of the disease showed that sex (female), older age at disease onset and respiratory system changes may be accepted as poor prognosis factors.

Adult↗
PubMed1990

Dermatomyositis and malignancy. A multicenter cooperative study.

A retrospective analysis of 118 cases of dermatomyositis (DM) collected in 22 departments of dermatology over a 5-year period is presented. 34 cases (28%) were associated with a cancer that was diagnosed more than 1 year prior to (7 cases), concomitantly (22 cases) or more than 1 year after (5 cases) the occurrence of DM. There was a clear correlation between the evolution of DM and a cancer paraneoplastic evolution in 8 cases only (22%). In most of the cases, no extensive diagnostic procedures were necessary for identifying the tumors. 58% of the patients with cancer-associated DM died 20 (8-29) months after the diagnosis of DM. This multicenter study based on a short period of time confirms and extends previous observations based on retrospective single-center analyses.

Dermatomyositis↗
PubMedDate not supplied

[Late diagnosis of juvenile dermatomyositis with spontaneous pneumothorax, pneumomediastinum, and subcutaneous emphysema].

The authors describe a 11 year-old girl that presented eczematous scabies with acute oligoarthritis that subsided in one day. Fourteen months later, she developed a classical juvenile dermatomyositis with spontaneous pneumothorax, pneumomediastinum, subcutaneous emphysema; pulmonary infection due to Pneumocystis carinii had been diagnosed and was attributable to immunosuppressive therapy. Despite the late diagnosis, the rapid disease activity and the multiple complications, clinical and laboratory controls were achieved by the corticotherapy and by an early diagnosis and treatment of complications.

Adolescent↗
PubMed1990

Incidence of polymyositis-dermatomyositis: a 20-year study of hospital diagnosed cases in Allegheny County, PA 1963-1982.

The incidence of hospital diagnosed polymyositis-dermatomyositis (PM-DM) among residents of Pittsburgh and Allegheny County, PA was determined from 1963 to 1982. Medical records of all Allegheny County hospitals were searched for patients diagnosed PM-DM, and each accepted case was reviewed and verified by a physician, according to specified criteria. Direct age adjusted incidence rates were computed for race and sex, as new cases/million population/year, with the 1970 Allegheny County population as the standard. One hundred seventy-seven cases were identified during this 20-year survey for a total annual incidence of 5.5/million population. Incidence more than tripled during 1973-1982 compared to the first decade of study, with the greatest increase occurring in black women. Overall, the female to male incidence ratio was 2.2:1, but during the childbearing years (ages 15-44) this ratio increased to greater than 5:1 in both races. Detection of PM-DM is increasing, as shown by temporal trends. Although our series has significantly more adult PM and less DM and overlap cases than other published studies, annual incidence is similar to that reported in other locales.

Age Factors↗
PubMedDate not supplied

Autosomal recessive inheritance of polymicrogyria and dermatomyositis with paracrystalline inclusions.

A 7-year-old mentally retarded girl died following subacute dermatomyositis. Muscle biopsies supported the clinical diagnosis and revealed paracrystalline inclusions on EM. The brain autopsy showed cerebral and cerebellar polymicrogyria. The clinico-pathological findings in this child are related to similar previously reported data in her older sister. The possibility of a new autosomal recessive syndrome involving both fetal brain development and childhood immunological function is discussed.

Atrophy↗
PubMedDate not supplied

[Pulmonary fibrosis during a case of dermatomyositis (author's transl)].

The authors describe the case of a pulmonary interstitial fibrosis, the discovery of which preceeded the skin and muscular manifestations of dermatomyositis which was confirmed by a muscular biopsy. The aetiopathogenesis of the pulmonary disorder is not well known, nevertheless an immunological mechanism demonstrated in the experimental myositis is suggested but has yet to be proved.

Adrenal Cortex Hormones↗
PubMed1990

Prognostic factors and predictive signs of malignancy in adult dermatomyositis. A study of 32 cases.

Dermatomyositis (DM) is an inflammatory myopathy with a severe prognosis mainly determined by its association with malignancy. We present the results of a study of 32 adults with DM. Our aim was to define predictive signs of cancer and establish prognostic factors according to patient survival. Thirteen (41%) of the 32 patients had DM associated with malignancy. After 7 years of follow-up, the overall mortality rate was 52%. Mortality was higher for patients with malignancy. Cutaneous necrosis and an elevated erythrocyte sedimentation rate appeared as potential markers of associated malignancy. Classic signs of poor prognosis, such as age and association with cancer were found. Interestingly, extensive cutaneous lesions on the trunk and an elevated erythrocyte sedimentation rate were also more frequent among the patients who died. Our results demonstrate that selected groups of patients with DM, in part defined by cutaneous signs, have a poor prognosis.

Adult↗
PubMedDate not supplied

Autoantibodies in juvenile dermatomyositis.

Fourteen patients with juvenile dermatomyositis (JDM) have been investigated for the presence of several serum autoantibodies: antinuclear (ANA), anti-single-stranded and double-stranded DNA, anti-histones, anti-Sm, anti-ribonucleoprotein, anti-SSA/SSB, anti-PM-1, anti-Jo-1, anti-mitochondrial, anti-smooth muscle, anti-gastric parietal cells, anti-cardiolipin (ACA) antibodies and rheumatoid factor. Patients were negative for all autoantibodies except for ANA and ACA. ANA were detected in 50% of the patients when tested on rat liver, but the percentage of positivity rose to 86% when HEp-2 cells were used as substrate. This finding suggests that HEp-2 cells represent a more sensitive substrate than rat liver for the detection of ANA in JDM. Three patients were positive for ACA; two of these presented vascular complications, thus suggesting a possible relationship between ACA and vascular involvement in JDM.

Antibodies, Antinuclear↗
PubMed1989

[Dramatic effects of high-dose intravenous gammaglobulin in each patient with intractable dermatomyositis and polymyositis].

A high-dose intravenous gamma-globulin (IVGG) therapy was given to two children; one with intractable dermatomyositis (case 1) and the other with polymyositis (case 2). Case 1, a 3-year-6-month-old girl, only poorly responded to the standard oral prednisolone therapy and a pulse therapy with high-dose methyl-prednisolone. This patient showed a complete remission after a course IVGG therapy, 100 mg/kg/day iv. for 5 days, which has been maintained for 6 months up to the present. Case 2, a 13-year-6-month-old girl, also showed little improvement after the oral prednisolone therapy, and transient improvement during plasma exchange therapy. This patient showed fairly good response after three repeated courses of IVGG therapy, but not complete remission.

Adolescent↗
PubMed1989

Adjunctive use of hydroxychloroquine in childhood dermatomyositis.

We examined retrospectively the use of hydroxychloroquine in 9 patients with childhood dermatomyositis (DM). These patients had an incomplete response to corticosteroid therapy or had disease exacerbation when attempting to taper corticosteroids. They received hydroxychloroquine in a mean dosage of 3.6 mg/kg (range 2-5 mg/kg) while maintaining corticosteroid therapy. Significant improvement was noted at 3 months of therapy in rash (p less than 0.0001), proximal (p = 0.0004) and abdominal muscle strength (p = 0.0001) and at 6 months of therapy in reduced prednisone dosage (p less than 0.02). These data suggest hydroxychloroquine may be helpful in some patients with childhood DM, particularly those with prominent cutaneous disease or significant corticosteroid toxicity.

Adolescent↗
PubMedDate not supplied

[Dermatomyositis. Clinico-histologic study of its skin manifestations].

The clinical and pathological findings in five patients with dermatomyositis are described. The diagnostic value of the cutaneous lesions in the disease, indicating the histological patterns of each cutaneous sign is stressed. Based in our experience and in the reviewed literature, we consider that Gottron's papules and poikilodermatomyositis present histological aspects that allow us to confirm the diagnosis suspected by clinical findings. Likewise we review some special difficult aspects of the disease such as infantile forms, association with neoplasias and overlap syndromes.

Adult↗
PubMed1989

Thyroid plasmacytoma with dermatomyositis and palmar fasciitis.

A 74-year-old woman with a primary thyroid plasmacytoma was studied. Clinical signs included a mass in the neck, as well as muscle and skin involvement due to dermatomyositis. In her serum a monoclonal IgG lambda was present. Immunohistochemistry showed an intracellular monoclonal IgG lambda component. After thyroid surgery and radiation, clinical improvement and fall of M protein were noted. On longterm followup, only palmar fasciitis persisted.

Aged↗
PubMed1989

Mechanism of the damaging effect of dermatomyositis mononuclear cells on cultured human skin fibroblasts.

We further studied the mechanism of action of peripheral mononuclear cells (MNC) from patients with dermatomyositis (DM) to strikingly suppress fibroblast proliferation in vitro. Attachment of MNC to fibroblasts was closely related to the mechanism. Most of attached MNC were activated T lymphocytes. Conditioned medium alone did not show any effects on fibroblasts. T8 positive cells and monocytes were essential to elicit the effect. Study of 51Cr release demonstrated that DM MNC augmented cytolysis of fibroblasts. It might be concluded that DM MNC possess T lymphocyte mediated cytotoxicity on fibroblasts and this causes the inhibition of fibroblast proliferation.

Adolescent↗
PubMed1989

[A case of dermatomyositis which rapidly developed to respiratory failure in the presence of pneumomediastinum].

A 49-year-old woman was admitted in February 1987, with a six-month history of joint pain, maculopapular and erythematous rash, proximal muscle weakness and a two-month history of skin ulceration and dyspnea on exertion. Physical examination showed Gottron's papules on her fingers and a faint heliotrope rash. Biopsy of erythematous skin lesions on the shoulder and the back of the hand revealed perivascular inflammatory cell infiltration and tiny ulcerative lesions of the cutaneous tissue. Biopsy of the right quadriceps muscle showed type II fiber atrophy and slight perivascular lymphocytic infiltrate, whereas serum CPK level was within normal range. Chest X-ray film showed granular infiltrates in both lower lung fields. Based on the current findings the case was diagnosed as dermatomyositis associated with interstitial pneumonia. The administration of prednisolone, 30 mg/day resulted in the improvement of the skin lesions and muscle weakness, while the intensity of lung infiltrates was little affected. Three months after steroid therapy, the patient was readmitted because of increasing dyspnea on exertion and multiple skin ulcers. Chest X-ray revealed a small amount of gas in the mediastinum, in addition to slight deterioration of interstitial lung disease. In spite of various treatments for pneumomediastinum, including bed rest, administration of analgesics and oxygen inhalation, it developed rapidly, complicated severe subcutaneous emphysema and right-sided pneumothorax. Although high-dose prednisolone therapy and mediastinal drainage were performed, the pneumomediastinum was not resolved and she died from respiratory failure. At autopsy, predominant histological features of the lungs were acute interstitial pneumonia with hyaline membrane and edematous granulation formation in the alveoli.(ABSTRACT TRUNCATED AT 250 WORDS)

Dermatomyositis↗
PubMed1989

[Plasma exchange in dermatology: apropos of a case of dermatomyositis].

We present a 45 years old male with dermatomyositis refractory to eight months of standard treatment who responded to plasma exchange. The efficacy of the procedure is estimated from clinical follow-up (increase in strength) and serum creatine-kinase activity (decrease from 3,380 to 44 u/ml.) after five sessions of plasma exchange. The therapeutic indications for PE are reviewed in dermatological diseases with emphasis on cases where the response to steroids or cytotoxic drugs is inadequate.

Dermatomyositis↗
PubMed1978

[The myocardiopathies of dermatomyositis and periarteritis nodosa].

Myocardial involvement was indicated by isolated findings (especially on the ECG) without corresponding functional disorders in one third of cases of dermatomyositis. Rapidly fatal heart failure occurred rarely in this disease, and was noteworthy for a QS complex in the central precordial leads, and for the presence of intracardiac thromboses which could give rise to emboli. Terminal cardiac failure was the rule in cases of periarteritis nodosa, which is a much rarer disease; this occurrence was secondary to hypertension. The most characteristic lesions were nodular coronary arteritis and disseminated myocarditis due to the arteriolar lesions.

Cardiomyopathies↗