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PubMed1994

Monitoring disease activity in juvenile dermatomyositis: the role of von Willebrand factor and muscle enzymes.

OBJECTIVE: To assess the usefulness of serial measurement of plasma von Willebrand factor (vWF) and serum levels of muscle enzymes in the detection and prediction of flares of disease activity in children with juvenile dermatomyositis (JDM). METHODS: Retrospective study of serial measurements of vWF and muscle enzymes in 16 patients with JDM followed for 540 patient-months. Charts were reviewed by an investigator blinded to laboratory results, and disease flare was defined as 2 of worsening function, increasing weakness, increasing muscle enzymes, increasing medication requirements. RESULTS: vWF was increased on at least one occasion in all but 2 patients with levels up to 4.9 IU/ml (normal 0.5 to 1.5). Laboratory evaluations were available in 23/29 disease flares. The sensitivity (0.85) and specificity (0.45) of vWF to detect disease flare were not better than that of LDH or AST even when muscle enzyme levels were excluded from flare definition. CK and ALT were not significantly associated with disease flare. The risk of flare increased 3-fold after a > 20% increase in AST levels, but vWF did not reliably predict disease flare. CONCLUSION: Although vWF is associated with disease flares, it does not seem to offer more information than enzyme measurements. LDH and AST were most useful in the detection of a flare, while AST was the only test capable of predicting it.

Aspartate Aminotransferases↗
PubMed1994

Juvenile dermatomyositis: a Roy nursing perspective.

With current medical treatment, juvenile dermatomyositis (JDMS) is rarely fatal. However, for most families, it is a chronic illness that interrupts life for both the affected child and the rest of the family. This article provides an overview of the disease, presents a case history of a 9-year-old affected girl, and discusses nursing care using the Roy Adaptation Model and nursing diagnoses.

Adaptation, Psychological↗
PubMed1993

[Immunoglobulins or plasma exchange? New treatment methods in polymyositis and dermatomyositis: plasma exchange and intravenous immunoglobulins].

Plasma exchange (PE) and intravenous polyvalent immunoglobulins (IVIG) offer a new therapeutic approach to polymyositis (PM) and dermatomyositis (DM). Plasma exchange. The largest open study of PE was reported by Herson et al., in 57 patients with inflammatory myopathies (33 DM, 24 PM) who were resistant to classical treatments. The patients were described as having acute (< 6 mois, n = 38) or subacute or chronic (n = 19) disease. There were 41 females and 16 males with a mean age of 40.4 +/- 20.5 years. The mean number of PE was 14.8 +/- 9.2. The score of muscle function improved in 54% of the patients. A significant improvement in the muscle test was only seen in the acute forms, particularly in severe cases with impaired swallowing. The difference was not significant in the subacute and chronic forms. Intravenous immunoglobulins. Several recent publications have emphasized the importance of polyvalent IVIG in the treatment of inflammatory myopathies. In our experience, 30 patients (21 females, 9 males, mean age 44.5 +/- 18) with PM (n = 22) or DM (n = 8) were given IVIG after unsuccessful classical therapies including corticosteroids (n = 29), immunosuppressors (n = 28), PE (n = 8), total body irradiation (n = 1), and lymphopheresis (n = 1). Three to 6 monthly cures of 2 g/kg/cure polyvalent IVIG were given. Clinical improvement was significant as evaluated by muscle tests in 18 of the 30 patients. Global scores for the 30 patients rose from 44.2 +/- 12.7 to 65.3 +/- 17.9 points (for a theoretical maximum of 88 points) after the third cure (p < 0.01).(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1994

Dermatomyositis occurring during psoralen A (PUVA) therapy.

Dermatomyositis (DM) developed in a 35-year-old woman after 2 months of psoralen ultraviolet-A (PUVA) therapy for presumed psoriasis. Her disease was characterized by symmetrical proximal muscle weakness and cutaneous lesions compatible with DM. In addition, laboratory abnormalities included a positive antinuclear antibody, and elevated levels of creatine kinase and aldolase. This is the first report of DM developing during PUVA therapy.

Adult↗
PubMed1993

[Juvenile dermatomyositis: clinical, immunologic, and therapeutic characteristics].

Although dermatomyositis is a rare disease in infancy, it involves several systems and tissues throughout the body causing death or serious disabilities at the musculoesquelethal level. The increased incidence of vasculitis and other characteristics in children make their clinical presentation different from the adult, which could modify the diagnostic criteria, course disease and treatment of the child. We analyze the clinical experience at Hospital Infantil de Mexico Federico Gomez with 21 children studied during a period of ten years. All patients fulfilled the Bohan and Peters diagnostic criteria and 91% of them responded to treatment. At the present time 52% are in remission without treatment, 37.7% improved with treatment, 4.5% with little response to treatment and 4.7% have died because of infections.

Child↗
PubMed1993

[Two cases of acute progressive interstitial pneumonia associated with dermatomyositis--clinical features and immunological disorders].

Some patients of dermatomyositis (DM) with interstitial pneumonia (IP) have common clinical features. Clinical features of these patients are acute onset, very poor prognosis and that patients have fever, arthritis, typical skin rash, mild myositis and show low ratio of CPK/LDH, low incidence of antinuclear antibody (ANA) appearance, low inflammatory signs. We experienced two cases of this category of DM with IP and examined immunological aspects. Case 1. A 52-year-old woman was admitted in June 1, 1990 with a one-month history of arthralgia and a ten-days history of fever, skin rash, myalgia and dyspnea on exertion. On examination she had Gottron's papules on her fingers, erythema on back, bilateral elbows and legs, proximal muscle weakness and arthritis. Fine crackles were audible in the lower lung fields. Laboratory data included CPK 200 IU/l, ALD 3.2 IU/l, LDH 805 IU/l. Analysis of bronchoalveolar lavage fluid (BALF) revealed increased cellularity with lymphocytosis. She was treated with oral corticosteroid (CS), pulse CS, cyclosporin A. Inspite of these therapies, she died of progressive respiratory insufficiency in July 10, 1990. Case 2. A 23-year-old woman was admitted in April 1, 1991, with a two-month history of arthralgia and a one-month history of fever, skin rash, stomatitis, alopecia. On examination she had Gottron's papules on her fingers, erythema on malar, bilateral elbows and legs, arthritis and stomatitis. Laboratory data included CPK 97 IU/l, ALD 8.5 IU/l, LDH 779 IU/l. She began experiencing dry cough and dyspnea on exertion in May 1991. Analysis of BALF revealed increased cellularity with lymphocytosis. She was treated with oral corticosteroid(CS), pulse CS, pulse cyclophosphamide.(ABSTRACT TRUNCATED AT 250 WORDS)

Acute Disease↗
PubMed1993

Reinduction of remission of chronic myeloid leukemia by donor leukocyte transfusion following relapse after bone marrow transplantation: recovery complicated by initial pancytopenia and late dermatomyositis.

A significant proportion of patients relapse after allogeneic BMT for CML. These relapses have been treated by induction of a graft-versus-leukemia effect by transfusing donor leukocytes. We have treated a 27-year-old woman with interferon and donor leukocyte transfusion and a complete haematological and cytogenetic remission was obtained coincident with the onset of GVHD. Her course was complicated by prolonged and profound pancytopenia which was fully reversed by the administration of rGM-CSF. She remains in CR with mild dermatomyositis due to chronic GVHD 17 months after the procedure.

Adult↗
PubMed1993

Juvenile and adult dermatomyositis among the Chinese: a comparative study.

Between 1983 and 1992, 11 children and 38 adults were seen at Taichung Veterans General Hospital with a definite diagnosis of dermatomyositis. Their clinical pictures, laboratory findings, courses and outcomes were compared. The mean age at diagnosis was 12 years and 50 years, respectively. Children had a higher female-to-male ratio (2.7:1 vs. 1.2:1) and a more acute onset, while adults had a higher incidence of malignancy and other connective tissue disease associations. Clinically, shawl sign was more common in the adults, whereas myalgia was more frequently seen in the children (0.05 < p < 0.1). Hemograms, serological parameters and immunological investigations showed no significant differences between the two groups. Elevated erythrocyte sedimentation rate and circulating immune complexes were found in over half of both groups of patients; however, their values bore no relationship to either disease activity or future outcome. Although no significant differences were demonstrated between the two groups of patients with regard to either clinical manifestations or laboratory findings, the disease entity seemed not to be the same because evidence of vasculopathy in muscle pathology was more prominent in the children, with endothelial swelling and necrosis the most common findings. Steroids had been used as the first choice of therapy in both groups of patients, followed by cytotoxic drugs and immunosuppressants. Children had a more favorable outcome compared to a 24% mortality rate in the adult group. Experience here recommends a complete cancer work-up in adults, especially for nasopharyngeal carcinoma among the Chinese. Various autoantibodies screenings may also be helpful for early detection of other associated connective tissue diseases.

Adolescent↗
PubMed1993

Dermatomyositis and ovarian cancer: a report of 7 cases and literature review.

The relationship between malignancy and inflammatory myopathies is now statistically well established. However, the link between polymyositis (PM) or dermatomyositis (DM) and ovarian cancer (OC) has never been clearly emphasized. OC is the 6th most frequent malignancy, occurring in about 1% of the general female population. We describe 7 cases of DM associated with OC (proven in 6 patients, and very probable in a 7th), in a series of 140 adult inflammatory myopathies followed in our institution [84 PM and 56 DM (including 45 female)]. Twenty-four patients had associated malignancy (12 PM and 12 DM). Without considering the probable case, OC occurred in 13.3% (6/45) of the total female DM population, a much higher rate than the 1% observed in the general female population; and in 21.4% (6/28) of females aged over 40 with DM. In women over 40 with DM, ovarian cancer has a major position among associated internal malignancies, and has to be adequately looked for by physicians.

Adult↗
PubMed1993

Solid phase radionuclide esophageal motility in polymyositis and dermatomyositis.

Polymyositis (PM) and dermatomyositis (DM) may be associated with motor dysfunction of the entire gastrointestinal tract. Abnormal esophageal motor function is a well-recognized complication of these diseases. In this study, we used a solid phase esophageal study to evaluate the motor function in patients with PM or DM. Twenty-three patients and 36 age-matched normal volunteers were studied. Each subject was placed in a supine position above a gamma camera linked to a computer and was given a 4-ml bolus of solid gelatin containing 1 mCi of Tc-99m phytate. Data were acquired in the list mode. A computer method modified from Kelim and Wald and Russell et al. was used to calculate the following: A) total mean transit time (MTT); B) residual fraction after the first swallow (RF); and C) retrograde index (RI). All values are presented as mean +/- standard deviation (SD). The Student's t-test was used to test statistical significance. Our preliminary results suggest: 1) delayed esophageal emptying is common (17/23) in PM/DM, indicating frequent malfunction of the smooth muscle of the upper gastrointestinal tract in PM/DM and 2) measurement of esophageal motility may monitor disease activity in PM/DM.

Adolescent↗
PubMed1977

Small blood vessel involvement in childhood dermatomyositis. An ultrastructural study of a case.

The electron microscopic changes in a muscle biopsy from a child with the clinical picture of acute dermatomyositis are described. Skin and muscle tissue examined by light microscopy did not revealed vascular alterations. Degeneration of isolated muscle fibers was detected with complete loss of myofilaments and disintegration of the Z-band material. Muscle arteries and veins were free of thrombi, but striking ultrastructural changes were seen in the smallest blood vessels in the muscle. These consisted mainly of vascular occlusion due to necrotic cells mixed with degenerated platelets. Dissoluted endothelial cells and denuded basement membrane were usually seen in the wall of the occluded capillaries. Cytoplasmic tubular particles were present in otherwise unaltered endothelial cells. The possibility that the capillary lesions are related to an acute immunological injury is mentioned.

Capillaries↗
PubMed1993

First case of pneumatosis cystoides intestinalis in adult dermatomyositis.

We describe the first case of pneumatosis cystoides intestinalis in dermatomyositis (DM) of an adult. Our patient had been in remission of her DM for years when pneumatosis cystoides intestinalis occurred. Discovery of it was concomitant with that of interstitial lung disease. The overall course of pneumatosis cystoides intestinalis was benign. We discuss the mechanism of bowel and lung involvement and hypothesize that vasculitis could be the underlying process.

Aged↗
PubMed1993

HLA class II alleles in juvenile dermatomyositis.

HLA class II alleles were investigated in 27 Czech patients (11 females and 16 males) with juvenile dermatomyositis. The immunogenetic investigation comprised determination of DRB1, DRB3, DRB5, DQA1, DQB1, and DPB1 alleles. Their prevalence was compared with that found in healthy Czech controls. No allele was found significantly more frequently in patients than in controls.

Adolescent↗
PubMedDate not supplied

Cyclosporin A in the treatment of juvenile chronic arthritis and childhood polymyositis-dermatomyositis. Results of a preliminary study.

In this study we have investigated the efficacy and safety of cyclosporin A (CyA) in a group of pediatric patients with juvenile chronic arthritis (JCA, 9 cases) and polymyositis-dermatomyositis (PM-DM, 3 cases). Of the 9 JCA patients, 7 had the systemic and 2 the polyarticular form of the disease. All of the patients received CyA after the failure of corticosteroids and/or cytotoxic drugs. CyA was administered for 9 to 48 months at a mean dose of 5 mg/kg/day. Ten patients received corticosteroids with CyA. The results of CyA treatment were satisfactory overall, both in the PM-DM patients and in the JCA patients, as assessed by clinical and laboratory evaluation. CyA-related side effects included alopecia, hypertension, hypertrichosis, tremors, and hirsutism. One JCA patient developed polyserositis with hypoproteinemia of unknown origin while receiving CyA. CyA blood levels did not correlate with clinical efficacy and/or side effects. This study suggests that CyA represents a promising agent for the treatment of JCA and childhood PM-DM.

Adolescent↗
PubMed1995

[Use of intravenous megadoses of methylprednisolone for treatment of dermatomyositis in children].

Intravenous treatment with megadoses of glycocorticoids was used in 8 children with juvenile dermatomyositis (JDM). Methylprednisolone (Mp-Solu-Medrol) was administered in a dose of 20 mg/kg, not exceeding 1 g, in the form of drop infusion over a period of 1.5-2.5 hours. An initial 3-6 consecutive day course of intravenous pulse was followed by further treatment twice a week for 2 to 5 weeks. A good response this therapy was observed in all of the children. However the best results were obtained in those patients who had not been treated previously and in when MP pulses were given soon from the onset of the disease (group I). MP pulse therapy administered in patients nonresponsive to conventional treatment (group II) did not prevent further relapses. When the treatment was introduced at the later, exacerbated stage of the disease, none of the patients entered into remission (group III). No side effects were observed during the pulse therapy. This study confirms the efficacy of MP pulse therapy in JDM, specially in the early severe stage of the disease.

Adolescent↗
PubMed1996

Scrotal pyoderma gangrenosum associated with dermatomyositis.

Pyoderma gangrenosum (PG) of the scrotum is extremely rare. We report a case of scrotal PG associated with dermatomyositis. Initial healing was slow despite the use of oral prednisolone and azathioprine. Healing was accelerated by the local use of a potent topical corticosteroid. PG should be considered in the differential diagnosis of ulcerating lesions of the genitalia.

Administration, Topical↗