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PubMed2006

Enzyme elevation in patients with juvenile dermatomyositis and steroid myopathy.

OBJECTIVE: Steroid myopathy can occur in patients with juvenile dermatomyositis (JDM) receiving chronic steroid therapy. We report an elevation of serum muscle enzymes, normal strength by manual muscle testing (MMT), and electromyographic (EMG) findings of steroid myopathy in children with JDM. METHODS: We prospectively studied children with JDM with a history of chronic steroid use (> 3 mo) and ongoing inflammatory myositis who were referred to our institution. RESULTS: We identified 5/9 children with JDM receiving longterm high dose steroids who had muscle enzyme elevation with no definable weakness and EMG findings consistent with steroid myopathy. All subjects improved after withdrawal of their steroid therapy. CONCLUSION: Longterm high dose steroids may lead to steroid myopathy with muscle enzyme elevation, previously reported only with acute steroid myopathy. We recommend that muscle derived enzyme levels should not be used to differentiate steroid myopathy from inflammatory myopathies.

Adolescent↗
PubMedDate not supplied

Dermatomyositis associated with breast cancer.

An increased risk of underlying malignancy has been found in patients with dermatomyositis (DM). The risk is increased even in patients with DM aged 45 or younger and remains high for many years after diagnosis. Breast carcinoma does not represent the most common solid tumor associated with this autoimmune disorder. In the present report, two new cases of DM associated with breast cancer are described, together with an extensive literature review.

Adult↗
PubMed2006

Efficacy of probenecid for a patient with juvenile dermatomyositis complicated with calcinosis.

Calcinosis of juvenile dermatomyositis (JDM) is a crucial problem because it is refractory to various therapies. An 11-year-old boy who had been treated for JDM with interstitial pneumonia developed calcinosis of both legs despite treatment with corticosteroid and cyclosporin A. Images of his knees showed massive calcinosis with restricted range of motion. Probenecid was used to reduce calcinosis, resulting in remarkable improvement of calcinosis accompanied by normalization of serum phosphorus level and disability after 17 months of administration. We suggest that probenecid is useful for the treatment of calcinosis of JDM.

Anti-Inflammatory Agents↗
PubMed2006

Use of etanercept in the treatment of dermatomyositis: a case series.

OBJECTIVE: To evaluate the efficacy of etanercept, a recombinant human soluble fusion protein of tumor necrosis factor-alpha (TNF-alpha) type II receptor and IgG1, in patients with adult dermatomyositis (DM). METHODS: Five patients with active DM were studied. All patients reported muscle weakness and had elevated muscle enzymes creatine kinase and lactate dehydrogenase. Because of lack of response to steroid and cytotoxic therapy, etanercept was given at a dose of 25 mg subcutaneously twice a week for at least 3 months. RESULTS: All patients experienced an exacerbation of disease, with increase of muscle weakness, elevation of muscle enzyme levels, and unchanged rash. Treatment with etanercept was stopped. After receiving a combination of methotrexate (MTX) and azathioprine, disease manifestations improved in all patients. CONCLUSIONS: In our case series, TNF-alpha inhibition by etanercept was not effective, suggesting that a broad immunosuppressive therapy is needed to treat DM.

Adult↗
PubMed2006

[Validity of intraoperative administration of dexmedetomidine in a patient with dermatomyositis-derived respiratory failure].

A 66-year-old female with dermatomyositis and severe respiratory failure from collagen lungs, dependent on domiciliary oxygen therapy, was scheduled for a mastectomy. Anesthesia was induced with dexmedetomidine (DEX) 6 microg x kg(-1) x h(-1) for 10 minutes and maintained at 0.7 microg x kg(-1) x h(-1), along with a target controlled infusion of propofol combined with epidural anesthesia. No narcotic or muscle relaxant was used. The airway was secured using a laryngeal mask airway and spontaneous breathing was preserved. The perioperative course was uneventful without any pulmonary complications. DEX has a certain analgesic property and a mild respiratory depressant effect. Therefore, it is considered useful as a concomitant anesthetic agent for perioperative management of patients with respiratory failure.

Aged↗
PubMed1991

[Effectiveness of intravenous immunoglobulins in polymyositis and dermatomyositis. An open trial in 15 patients].

Polymyositis (PM) and dermatomyositis (DM) are dysimmune diseases usually treated with corticosteroids and immunosuppressants. Human polyvalent immunoglobulins administered intravenously (IgIV) are known to be effective in some dysimmune diseases. Between August 1987 and September 1989 we conducted an open trial of IgIV in 15 patients (mean age 44 +/- 14 years) with either PM (12 cases) or DM (3 cases) associated with a collagen disease in 2 patients. In 14 of these 15 patients the conventional treatments (corticosteroids, immunosuppressants, plasmapheresis, total body irradiation, lymphopheresis) had failed. One patient was seropositive for picornavirus and received IgIV as initial treatment. IgIV infusions were given 4 +/- 3.9 years on average after the onset of PM or DM. Twelve of the 15 patients received another treatment, starting at least 6 weeks before IgIV and pursued without dosage increase, which consisted of corticosteroids (11 cases), methotrexate (5 cases) or plasmapheresis (1 case). Human polyvalent immunoglobulins for intravenous use were prescribed in doses of 2 g/kg/monthly course. All but two patients (1 course) received 3 to 6 courses on average. The IgIV infusions were well tolerated in 12 patients; 3 patients showed allergic manifestations which regressed. Therapeutic effectiveness was evaluated by muscle testing and by repeated assays of creatine phosphokinase (CPK). Clinical improvement, usually perceptible after the first course, was observed in 13/15 patients; it was associated with a more than 30 percent decrease of the initial CPK level in 13 patients and with a reduction of associated therapies in 9 patients. In the entire patient population a statistically significant lowering of mean CPK value was observed as early as in the first course (P less than 0.001). In view of their effectiveness, rapid action and safety, intravenous Ig infusions may be regarded as an interesting treatment in PM or DM patients.

Adrenal Cortex Hormones↗
PubMed1991

[Infantile dermatomyositis. Clinical aspects and prospective treatments].

Juvenile dermatomyositis (DMI) is a rare illness of multisystem inflammatory disease of unknown etiology. It is usually considered as a part of the spectrum of polymyositis/dermatomyositis (PM/DM), and is distinguished from the adult form in having a number of different clinical features as well as distinctive pathologic findings. Although the clinical findings and course of the disease, laboratory evaluation and therapy have been reviewed, many of these studies are old and may not accurately reflect the current status of the disease as it is now defined and treated. Generally it is first diagnosed by the appearance of typical signs in both the skin and muscles. The clinical picture shows early signs of heterogeneity and is confirmed by these latter developments. Its classification falls into three groups: acute single cycle, chronic polycycle and continued chronic; this sickness invariably evolves into one or another of these three. Our current work involves 15 cases of DMI; 8 have acute monocycle, 4 chronic polycycle and 3 continued chronic. This illustrates the clinical and biological heterogenousness of this illness which in itself presents extreme difficulties when considering possible treatment. From our experience, high dose of corticosteroida intraveins have had the best result. Where cases have been acute at the beginning of the sickness and where there has been complications, particularly those of a bronco-pulmonary nature (we treated 3 such cases) it has proven to be beneficial to use corticosteroid together with the immunosuppresant: ciclofosfamide. The use of ciclosporina in one case who had resisted other forms of therapy has appeared to be effective, though this has yet to be verified.

Acute Disease↗
PubMed1991

Juvenile dermatomyositis.

Juvenile dermatomyositis is a multisystem disease characterized by muscular inflammation affecting primarily the skin and muscle; it presents as a nonsuppurative myositis causing symmetrical weakness and typical skin rashes that affect the face and hands in particular, but can occur at any site. In the last few years, a number of reviews have been published, this article only highlights some points of interest.

Adolescent↗
PubMedDate not supplied

[Juvenile dermatomyositis: clinical manifestations and laboratory tests].

Juvenile dermatomyositis (JDM) is a chronic, inflammatory degenerative entity with multisystemic involvement, particularly of skin and striated muscle. This is a description of the clinical features, laboratory findings and treatment results in 10 children with JDM which were under control along a 14 years period (1974-1988) in a general hospital at metropolitan Santiago, Chile. Onset was insidious in 8 cases and acute in the remaining patients. All children had muscle and skin involvement, 90% of them showed heliotrops erythema and 6 cases (among the total 10) had one or more additional skin signs. Ninety percent of patients had risen serum activity of muscle associated enzymes, lactic dehydrogenase (LDH) being the most constantly elevated: 7/7 studied cases. All patients showed abnormal electromyographic and histologic findings in muscle biopsy. Treatment was based upon rehabilitation, prednisone (average daily dose: 1.5 mg.kg of body weight) associated or not with cytotoxic drugs, accordingly to each individual case. Clinical course was considered to be favourable in 9 of the 10 cases because all of them showed no evidence of disease activity on last follow up control 26 to 93 months after initial consultation, six had only slight restriction to wider range movements of affected joints and three showed no sequelae. Only one death occurred in this series, to a patient who rejected treatment.

Child↗
PubMed1976

Aberrant serum enzyme patterns in dermatomyositis associated with hepatoma.

A Caucasian male developed florid dermatomyositis documented by serum enzyme elevation, electromyography, and histology of skin and muscle. Serum enzymes, including creatine phosphokinase (CPK), aldolase, glutamic oxaloacetic transaminase (SGOT), and lactic dehydrogenase (LDH), decreased initially during high dose systemic corticosteroid therapy, although profound muscle weakness persisted. Subsequent elevation of serum LDH and SGOT levels during treatment provided a clue to underlying neoplasia. Primary hepatoma with widespread metastases was found at necropsy.

Adult↗
PubMed1991

[Dermatomyositis--pathogenesis, diagnosis, therapy].

Dermatomyositis is a rare systemic autoimmune disease that principally involves the skeletal muscles. Currently, the etiopathology is suspected to involve a viral infection with underlying genetic susceptibility, resulting in an abnormal immune reaction. Autoantibodies, in particular against enzymes involved in protein synthesis--especially aminoacyl-tRNA synthetases--appear to be found for the most part when the lungs are involved. Differential diagnosis is facilitated by the detection of various auto-antibodies. Treatment is unsatisfactory, and controlled studies, in particular when resistance to prednisone presents, are lacking. The prognosis depends, among other things, on the internal organs involved (heart, lungs, gastrointestinal tract, endocrine system). The search for viruses (e.g. hepatitis B) should be intensified using the latest, more sensitive, methods.

Autoantibodies↗
PubMed1991

[Interstitial pulmonary involvement of polymyositis and dermatomyositis. Apropos of 3 cases. Review of the literature].

The authors report 3 new cases of myositis associated with pulmonary lesions that preceded or succeeded the muscular disorder. In one of these cases, which was particularly difficult to diagnose, the patient's serum was positive for the anti-Jo1 antibody. These 3 cases have encouraged the authors to review the literature with particular attention to the diagnostic approach, the latest physiopathological data and the therapeutic basis of the "specific" pulmonary lesions associated with polymyositis and dermatomyositis.

Adult↗
PubMed1991

[Adult dermatomyositis with angiopathy and cecum perforation].

This previously healthy 43-year-old man was admitted to our hospital with a history of rash, dysphagia and severe myalgia for two months. Physical examination showed prominent edema and erythema over the face and the chest, scattered ulcerations on the trunk, and muscle atrophy most prominent proximally. Serum levels of muscle enzymes were remarkably increased. Two weeks of oral prednisolone therapy (40 mg/day) was not effective, and betamethasone intravenous pulse therapy (3 x 1000 mg) was followed by slight clinical improvement. However, 12 days after pulse therapy, he complained abdominal pain on the right lower quadrant. The surgical findings included peritonitis due to single perforation of the cecum. After operation, cyclosporine therapy was added and over the next 14 month a considerable clinical improvement was noted. Prednisolone was reduced from 80 mg to 10 mg daily. Biopsy specimens from ulcerated+ skin and perforated cecum showed prominent vascular abnormalities: arterial and venous intimal hyperplasia, occlusion of vessels by fibrin thrombi, and lymphocytic infiltration which affected veins of all sizes. The evidence strongly suggests that both skin ulcers and cecum perforation were caused by vasculitis and occlusion of vessels, which often seen in childhood dermatomyositis.

Cecal Diseases↗
PubMed1991

Disseminated Nocardia brasiliensis infection: an unusual complication of immunosuppressive treatment for childhood dermatomyositis.

The use of steroids combined with cytotoxic drugs has increased in the last decade. The concomitant increase of opportunistic infections has contributed significantly to morbidity and mortality of patients treated with immunosuppressive agents. We describe a child with dermatomyositis who developed disseminated Nocardia brasiliensis infection while receiving steroids and methotrexate. Infectious etiology was established by gram stain. The patient was treated successfully. Disseminated Nocardia brasiliensis infection is rare with a high reported mortality. Diagnosis may be delayed secondary to insidious onset, similarity of clinical manifestations to other pathogens and slow growth in routine culture media. Nocardia should be considered early in the evaluation of infection in patients treated with immunosuppressive agents.

Adolescent↗
PubMed1991

[Juvenile dermatomyositis, systemic lupus erythematosus and mixed connective tissue disease].

This paper covers the current insights into the classification, etiology, management and prognosis of juvenile dermatomyositis (JDMS) systemic lupus erythematosus (SLE) and mixed connective tissue disease (MCTD). Since specific diagnostic tests for these autoimmune diseases are not available, diagnosis depends on the presence of defined criteria. Drug therapy in JDMS consists of prednisone at a low dosage (1 mg/kg/day). In SLE different therapeutic approaches are known depending on the occurrence of life threatening symptoms. The prognosis of MCTD is good, it is of prime importance to avoid overdosage of corticosteroids, since drug treatment is often not needed.

Adrenal Cortex Hormones↗
PubMedDate not supplied

[Juvenile dermatomyositis].

Juvenile dermatomyositis (JDM) is a chronic multisystem inflammatory disease that primarily involves skin and muscles. The clinical picture is mainly characterized by the typical cutaneous rash and by skeletal muscle weakness. Nevertheless the vasculitic process may extend in some cases to other organs such as lung, joints or gastrointestinal system. Calcinosis represents a relatively frequent complication of JDM and occurs in most cases during the late stage of the disease. Usually serum levels of muscle enzymes are elevated and electromyography shows signs of myositis. Muscle biopsy is generally performed only in doubtful cases. JDM should be differentiated from the muscle involvement of other diffuse inflammatory connective tissue diseases and from non inflammatory myopathies. The treatment is mainly based on steroids, whose dosage and regimen depend on disease severity. Steroid therapy has greatly improved the prognosis of JDM.

Adolescent↗