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At least 469 records · Page 26Linked to original sources

Dermatomyositis in five Shetland sheepdogs in the United Kingdom.

Five cases of dermatomyositis in four Shetland sheepdog puppies and one adult bitch are described. The dogs all had well-defined patches of scaling, crusting and alopecia over the muzzle, periorbital skin and distal limbs, and the tail, perineum and pinnae were affected in some of them. The affected puppies were all sired by the same stud dog. The affected adult bitch was unrelated to the puppies. Three of the four dogs tested had high serum creatine kinase concentrations and electromyographic abnormalities were detected in three of the four dogs tested. The histological changes observed in the skin of four of the dogs strongly supported the diagnosis of dermatomyositis, and in the fifth dog they were compatible with this diagnosis. Two of the puppies were euthanised shortly after being diagnosed. In the other two puppies and the adult the disease remains stable and non-progressive 15 to 18 months after diagnosis. The sire of the four affected puppies has been used extensively because it was considered to be genetically clear of collie eye anomaly.

Animals↗

Dermatomyositis associated with membranous nephropathy in a 43-year-old female.

A 43-year-old woman was admitted to our hospital for myalgic pain and erythema of the face and thighs. She had been diagnosed with dermatomyositis at another hospital 8 years earlier and proteinuria had developed suddenly. Renal histological examination revealed membranous nephropathy. Membranous nephropathy associated with dermatomyositis is very rare. To date, malignant disease has not been detected in our patient, but the serum level of CA125, a tumor marker, has been elevated to 1,097 U/ml. The increased CA125 level is related to the degree of proteinuria and after prednisolone and cyclosporine therapy, proteinuria and the CA125 level gradually decreased.

Adult↗

The cutaneous histology of dermatomyositis in collie dogs.

Cutaneous lesions of 36 collie dogs affected with dermatomyositis were reviewed. The most common histologic features were follicular atrophy and perifollicular inflammation in 30 dogs, which correlated with alopecia clinically. Other less common findings included formation of colloid bodies, basal cell vacuolation, and subepidermal vesiculation. Results indicated that the most diagnostic cutaneous histologic features of dermatomyositis may be follicular atrophy and inflammation.

Animals↗

Juvenile dermatomyositis induced by toxoplasmosis.

A 10-year-old girl from southern Alberta, Canada, who had close contact with cats, developed typical features of dermatomyositis. The diagnosis was confirmed by muscle biopsy. A toxoplasmosis titer was 1:16,384 by indirect fluorescent antibody technique, and the IgM response to toxoplasma was positive. Only minimal improvement followed prednisone and azathioprine administration, but she rapidly improved after 4 weeks of treatment for toxoplasmosis with pyrimethamine and sulfadiazine. A year after the onset of dermatomyositis, she showed no weakness or cutaneous lesions, and a repeat muscle biopsy no longer showed inflammation, perifascicular atrophy, or regeneration of myofibers. She remains asymptomatic more than 2 years after discontinuation of all medications. Investigation for immune deficiency disease 1 year after therapy revealed that lymphocytic response to T-cell and B-cell mitogens was normal, as were immunoglobulin and complement levels. She had mild impairment of natural killer cell activity and a positive antinuclear factor. Her rapid improvement on specific therapy and lack of significant long-term immune deficiency is consistent with acute toxoplasmosis infection in an immunologically competent child.

Antibodies↗

Cardiac and central nervous system vasculitis in a child with dermatomyositis.

Cerebral vasculitis and clinically important myocardial inflammation are rare in juvenile dermatomyositis. We report a previously healthy 6-year-old girl with dermatomyositis who died after a fulminating clinical deterioration. Postmortem examination of the heart revealed characteristic endothelial tubuloreticular aggregates and evidence of capillary necrosis and secondary thrombosis, associated with extensive hemorrhagic myocardial necrosis. Endothelial necrosis was also evident in the cerebrocortical capillaries.

Autopsy↗

Interstitial lung diseases associated with amyopathic dermatomyositis.

The aim of the present study was to clarify the clinical characteristics and prognosis of patients with interstitial lung disease (ILD) associated with amyopathic dermatomyositis (ILD-ADM). The study consisted of 14 consecutive patients with ILD-ADM. Patients were classified into two categories, acute/subacute and chronic forms, according to the clinical presentation of ILD. The clinical features, responsiveness to therapy, and prognosis between the two forms were compared. Nine ILD-ADM patients were categorised as the acute/subacute form, and five as the chronic form. Arterial oxygen tension was significantly lower in the acute/subacute ILD than chronic ILD patients. On high-resolution computed tomography, ground-glass opacities were frequently found in the two forms, but consolidation was more common in acute/subacute ILD than chronic ILD. Bronchoalveolar lavage analysis showed higher numbers of total cells and lymphocytes in acute/subacute ILD than chronic ILD. Histologically, the most common finding was nonspecific interstitial pneumonia in the two forms, while diffuse alveolar damage was only found in acute/subacute ILD. Acute/subacute ILD was generally resistant to therapy, while chronic ILD responded well. Notably, the mortality of acute/subacute ILD was much higher than that of chronic ILD (67 versus 0%, respectively). In conclusion, interstitial lung disease associated with amyopathic dermatomyositis includes two different forms, the acute/subacute and chronic forms, with distinct prognoses.

Acute Disease↗

Immunosuppressive agents in corticosteroid-refractory childhood dermatomyositis.

Five patients with childhood dermatomyositis, followed for 18 to 96 months, improved when treated with a combination of corticosteroids and methotrexate (one patient) or corticosteroids and cyclophosphamide (four patients) after having become refractory to corticosteroid therapy alone. Complications of vascular involvement in childhood dermatomyositis or from immunosuppressive therapy were observed in most of these patients. Disease components involving skin, muscle, or systemic vessels sometimes varied independently in regard to disease activity or therapeutic responsiveness. In two patients with stable or improving muscle function, cerebrovascular complications occurred. Monitoring of disease activity was best accomplished by clinical evaluation; serum muscle enzymes usually failed to rise prior to or during periods of clinical worsening.

Child↗

Childhood dermatomyositis in monozygotic twins.

Identical twins who developed childhood dermatomyositis 2 weeks apart following upper respiratory infections are reported. Observations suggest that genetic influence on host susceptibility may play a role in childhood dermatomyositis and that a combination of host and environmental factors is most likely necessary to produce the disease.

Autoimmune Diseases↗

Dermatomyositis-polymyositis and malignancy. Is there a direct relation?

An association between dermatomyositis-polymyositis and malignancy has been reported. However, controversy exists regarding a direct causal relationship between the two diseases. We describe a patient who had the concurrent onset and parallel clinical course of dermatomyositis and malignancy, and discuss the possible mechanisms for the association of the two disorders.

Adenocarcinoma↗

Treatment approaches to juvenile dermatomyositis.

Juvenile dermatomyositis (JDM) is a rare disease of childhood with significant morbidity. Although the mortality and morbidity has improved over recent decades, it is still a chronic disease for a significant proportion of children with JDM. There is a paucity of controlled trials in both adult and juvenile dermatomyositis. In this article, the different therapeutic agents used in clinical practice are outlined. Furthermore, our approach to the treatment of JDM with methotrexate and aggressively tapered steroids in an algorithmic fashion, is discussed.

Consensus↗

Muscle pathology in juvenile dermatomyositis.

OBJECTIVE: To study muscle biopsies, using histochemistry, on ten children with infantile dermatomyositis. DESIGN: Series of ten patients (of whom eight patients had received treatment and two had not) were submitted to muscle biopsy in order to diagnose possible inflammatory myopathy or to detect recurrences. PLACE OF DEVELOPMENT OF THE STUDY: Public Health Service of São Paulo State. PARTICIPANTS: Children with clinical features of inflammatory myopathy. INTERVENTION: Biopsies were performed on the vastus lateralis using local anesthetic. Histochemistry was performed according to standardized methods. RESULTS: Architectural changes of the muscle fibers, necrosis of variable intensity and accentuated evidence of regeneration were observed in patients who had not received treatment (2 cases) and in one case where muscular weakness persisted in spite of corticosteroid therapy. Necrosis and regeneration were minimal or absent in cases treated for one year or more (4 cases). In 3 cases with clinical and laboratorial recurrences, muscle necrosis and architectural changes were detected. CONCLUSIONS: It was concluded that muscle biopsy could aid in diagnosing infantile dermatomyositis as well as in detecting recurrences even in cases without clinical activity of the disease.

Adolescent↗

Chronic lymphocytic leukemia associated with nephrotic syndrome and dermatomyositis.

Several types of autoimmune complications of chronic lymphocytic leukemia (CLL) have been previously reported. However, the tendency to develop autoantibodies is usually restricted to the hematopoietic system. We report a 68-year-old man who had developed dermatomyositis after ten years of chemotherapy for CLL. He also had secondary nephrotic syndrome at the onset of CLL. Subsequently, the patient died of perforation of the small intestine. The association of both nephrotic syndrome and dermatomyositis with CLL is very rare. We discuss the possibility of a casual relation.

Aged↗

High-dose immunoglobulin therapy for a patient with dermatomyositis.

A 46-year-old woman was admitted to our department complaining of dermal eruption and weakness of muscles. She was diagnosed as having dermatomyositis and was initially treated with prednisolone. Since her condition rapidly deteriorated, high-dose intravenous immunoglobulin (i.v.IG) therapy (0.4 g/kg/day i.v. for 5 days) was administered. Marked improvement in muscle strength was observed the following day after the first administration. She unfortunately died of pneumocystis carinii pneumonitis 2 weeks after the i.v.IG therapy. Autopsy revealed no inflammatory cells in the muscles, suggesting that i.v.IG therapy has an important clinical application for refractory dermatomyositis.

Creatine Kinase↗

Epstein-Barr virus-associated gastric cancer in a patient with dermatomyositis.

A 59-year-old man was admitted presenting systemic rash and muscle weakness. He was diagnosed to have dermatomyositis and a check was made for internal malignancy. Gastrointestinal endoscopy revealed a Borrmann type II tumor on the middle body of the stomach. Biopsy specimens showed a well differentiated adenocarcinoma, and total gastrectomy was performed. The final diagnosis was moderately differentiated adenocarcinoma invading into the proper muscular layer, with metastases to regional lymph nodes. Most of the neoplastic cells were shown to be positive for Epstein-Barr virus by means of EBV-encoded RNA in situ hybridization. The symptoms of dermatomyositis disappeared completely after surgery.

Adenocarcinoma↗

Anti-Ku antibody-positive scleroderma-dermatomyositis overlap syndrome developing Graves' disease and immune thrombocytopenic purpura.

Graves' disease (GD) has been reported to be frequently complicated with other autoimmune diseases. However, it is rarely complicated with scleroderma-polymyositis overlap syndrome. Recently, we encountered a 35-year-old woman who developed GD and immune thrombocytopenic purpura during follow-up observation of scleroderma-dermatomyositis overlap syndrome. Platelet counts recovered after high-dose gamma-globulin therapy and bolus methylprednisolone therapy. The present case is the first report of a combination of scleroderma, dermatomyositis, GD, and immune thrombocytopenic purpura. The patient was anti-Ku antibody-positive and had relatively low natural killer T cell counts, both of which might contribute to the complication of multiple autoimmune diseases.

Adult↗

Nonspecific interstitial pneumonia with poor prognosis associated with amyopathic dermatomyositis.

Amyopathic dermatomyositis (ADM) is a clinical subtype of dermatomyositis, characterized by the lack of motor weakness and the presence of normal muscle enzyme levels. ADM is sometimes accompanied by interstitial pneumonia that shows a rapid progressive course associated with a poor prognosis. We report a 49-year-old patient who presented with nonspecific interstitial pneumonia (NSIP) associated with ADM. The patient failed to respond to prednisolone and immunosuppressive therapy and died. Although idiopathic NSIP is known to have a better prognosis, NSIP in ADM could be a fatal disease. Therefore, we should appropriately treat interstitial pneumonia in ADM even if it is NSIP.

Dermatomyositis↗

[An autopsy case of dermatomyositis with rapidly progressive interstitial pneumonia].

An autopsy case of dermatomyositis with rapidly progressive interstitial pneumonia is reported. a 48-year-old woman was admitted because of facial edematous erythema and muscular weakness. A diagnosis of dermatomyositis was made because of typical erythema and myogenic pattern of EMG, although serum creatine kinase was within normal range. The chest X-ray film showed that interstitial pneumonia was complicated. She was treated with prednisolone of 60 mg/day. Although erythema and althralgia was improved remarkably, interstitial pneumonia was progressed unfavorably. Therefore pulse therapy with methylprednisolone was administered twice, but failed to respond. After that interstitial pneumonia was progressed rapidly, she was died of respiratory failure. The autopsy findings detected that a degeneration and necrosis of striated muscular fibers in whole body, that a mixed findings of usual interstitial pneumonia and diffuse alveolar damage in the lung. Vasculitis and fiblinous pleuritis was also found in the lung. Malignancy was not detected.

Dermatomyositis↗