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We report a case presenting with 'flagellate' erythema which is a characteristic skin lesion of dermatomyositis seen in Japan. A possible pathological mechanism is proposed in comparison with the 'flagellate' erythema caused by bleomycin.
A dermatomyositis-like syndrome developed in a patient treated with a nonsteroidal anti-inflammatory agent (NSAI), niflumic acid, and regressed after the cessation of treatment. Previously an eruption had occurred under treatment with another NSAI, diclofenac. Our report shows that NSAI can induce not only lupus-like syndromes but also other connective tissue disorders.
A case of dermatomyositis is described with the simultaneous appearance of clinical and laboratory features of myositis, skin involvement and idiopathic thrombocytopenia with associated anti-platelet antibodies.
A 10-year-old boy with systemic lupus erythematosus exhibiting prominent features of dermatomyositis has been treated with 600 mg prednisolone intravenously per day on 3 consecutive days. Clinical and serologic improvement started after the first course of pulse therapy, and was remarkable during a second course. Basic treatment using oral prednisolone is recommended.
A high dose of methylprednisolone (pulse therapy) was given to 3 patients with uncontrolled dermatomyositis (DM) accompanied by severe muscle weakness. The effects of pulse therapy were compared with findings in 5 patients with severe DM who were treated with the usual dose of prednisolone (controls). The pulse therapy led to an improvement in the clinical symptoms in the 3 patients with uncontrolled DM, who were in danger of aspiration pneumonia. A rapid reduction in muscle enzyme levels in this group was also evident.
There are few reports in the literature of pregnancy in dermatomyositis (DM), in contrast with those of other connective tissue diseases. We describe 2 patients who had an established diagnosis of DM and then became pregnant. In these cases, both the fetal and maternal outcome were good, and differed from those in previously reported cases.
A nearly symmetrical ureteral necrosis was observed in a child with dermatomyositis. The ureters were treated conventionally by resection, anastomosis and repeated catheterization with good results.
We report findings in 70 patients with both diffuse interstitial lung disease and either polymyositis (PM) or dermatomyositis (DM). Initial presentations were most commonly either musculoskeletal (arthralgias, myalgias, and weakness) or pulmonary (cough, dyspnea, and fever) symptoms alone; in only 15 patients (21.4%) did both occur simultaneously. Pulmonary disease usually took the form of acute to subacute antibiotic-resistant community-acquired pneumonia. Chest radiographs and computed tomography most commonly demonstrated bilateral irregular linear opacities involving the lung bases; occasionally consolidation was present. Jo-1 antibody was present in 19 (38%) of 50 patients tested. Synchronous associated malignancy was present in 4 of 70 patients (5.7%). Surgical lung biopsies disclosed nonspecific interstitial pneumonia (NSIP) in 18 of 22 patients (81.8%), organizing diffuse alveolar damage (DAD) in 2, bronchiolitis obliterans organizing pneumonia (BOOP) in 1, and usual interstitial pneumonia (UIP) in 1. Treatment usually included prednisone in 40-60 mg/d dosages for initial control, followed by lower dose prednisone plus an immunosuppressive agent such as azathioprine or methotrexate for disease suppression. Survival was significantly better than that observed for historical control subjects with idiopathic UIP, and was more consistent with survival previously reported in idiopathic NSIP. There was no difference in survival between Jo-1 positive and Jo-1 negative groups.
Open lung biopsies from 14 patients and autopsy tissue from one patient with polymyositis/dermatomyositis were reviewed in an attempt to correlate histologic features with clinical, radiographic, and prognostic variables. Three major groups based on histologic patterns were identified: bronchiolitis obliterans organizing pneumonia (BOOP), usual interstitial pneumonia (UIP), and diffuse alveolar damage (DAD). Patients with BOOP had a more favorable prognosis than did patients with UIP. Patients with DAD had a uniformly poor prognosis. One patient had a cellular interstitial pneumonia and did well. Histologic subclassification of the interstitial lung disease proved to be a better predictor of survival than did the radiographic appearance or the clinical presentation. These findings suggest that there is a broader range of histologic findings in polymyositis-dermatomyositis than is suggested in the literature and subclassification may be useful for prognosis.
In patients with dermatomyositis (DM) the earliest lesion is microvasculopathy mediated by deposition of C5b-C9 membranolytic attack complex (MAC) on intramuscular capillaries. This leads sequentially to muscle ischemia, necrosis of muscle fibers, and muscle weakness. High-dose intravenous immunoglobulin (IVIG), which can modulate complement-dependent tissue damage in animal models, has been shown to be effective in the treatment of patients with DM. We used an in vitro C3 uptake assay to examine 55 coded sera from 13 patients with DM and 5 patients with other non-complement-mediated neuromuscular diseases, before and after treatment with IVIG or placebo. Patients with active DM had a significantly higher baseline C3 uptake compared with the others (geometric mean 12,190 vs 3,090 cpm). Post-IVIG but not post-placebo sera inhibited the C3 uptake, without depleting the complement components, by 70.6-93.4%. The maximum inhibition of C3 uptake occurred within hours after IVIG infusion, started to rebound 2 d later, and reached pretreatment levels after 30 d. The serum levels of SC5b-9 complex production were high at baseline but normalized after IVIG therapy. Repeat biopsies from muscles of improved patients showed disappearance of C3b NEO and MAC deposits from the endomysial capillaries and restoration of the capillary network. We conclude that IVIG exerts its beneficial clinical effect by intercepting the assembly and deposition of MAC on the endomysial capillaries through the formation of complexes between the infused immunoglobulins and C3b, thereby preventing the incorporation of activated C3 molecules into C5 convertase. These findings provide the first serological and in situ evidence that IVIG modulates complement attack in a human disease.
Polymyositis (PM) and dermatomyositis (DM) are the prototypical inflammatory diseases of skeletal muscle. In PM, CD8+ T cells invade and destroy muscle fibers, whereas humoral effector mechanisms prevail in DM. We studied the expression of the cytotoxic mediator perforin in inflammatory cells in PM and DM muscle by semiquantitative PCR, immunohistochemistry and confocal laser microscopy. Similar levels of perforin mRNA were expressed in PM and DM, and abundant perforin-expressing CD3+CD8+ and CD3+ CD4+ T cells were observed in both diseases. However, there was a striking difference in the intracellular localization of perforin. In DM, perforin was distributed randomly in the cytoplasm of the inflammatory T cells. In contrast, 43% of the CD8+ T cells that contacted a muscle fiber in PM showed perforin located vectorially towards the target muscle fiber. The results suggest (a) that the random distribution of perforin in the cytoplasm of muscle-infiltrating T cells observed in DM reflects nonspecific activation, and (b) that the vectorial orientation observed only in PM reflects the specific recognition via the T cell receptor of an antigen on the muscle fiber surface, pointing to a perforin- and secretion-dependent mechanism of muscle fiber injury.
A forty-five-year-old woman with a history of dermatomyositis for the past fifteen years developed sick sinus syndrome during an exacerbation of her illness. This association has not been previously described in the literature, as far as the author can determine.
A sixty-two-year-old woman with chronic dermatomyositis (DM) receiving steroid monotherapy developed coronary artery stenosis. She had lung fibrosis and complained of dyspnea, but no ischemia was suggested by electrocardiogram. Ateriographic findings and clinical symptoms of coronary artery disease in DM have not been previously reported.
The authors present a forty-year-old man, with a history of dermatomyositis for the past twelve years, managed intermittently with prednisone. During an exacerbation of his illness he developed an acute anterior wall myocardial infarction with normal coronary arteries. A literature review indicates this represents a rare association.
The simultaneous occurrence of juvenile dermatomyositis (DMS) and diabetes mellitus is described in 2 pediatric patients. Both these patients presented with significant weight loss, polyuria, and polydypsia within a short time of being diagnosed with JDMS, while these patients were taking oral prednisone (40-60 mg/day in divided doses). Laboratory evaluation detected ketonuria, significant hyperglycemia (696 and 913 mg/dL) and low serum levels of insulin and C-peptide. Both these patients were treated with high doses of insulin. Islet cell and GAD65 antibodies were found to be positive in 1 of the patients, pointing toward a diagnosis of insulin-dependent diabetes mellitus. The other patient tested negative for these antibodies and required insulin therapy for approximately 6 months. Steroid-induced diabetes mellitus seemed highly likely in this case. We hypothesize that a common environmental trigger possibly a viral infection might have been responsible in causing 2 different autoimmune pathologies in these genetically predisposed individuals.
The presentation, clinical course, and long-term follow-up (3-22 years) of 39 children with dermatomyositis followed from 1962-1982 is presented. The medical course of these patients was complicated by respiratory diseases (20%), gastrointestinal diseases (24%), and calcinosis (30%). Patients presenting prior to 1972 received a wide variety of treatments. Since 1972, treatment has consisted of long-term prednisone, supplemented in some patients with azathioprine. Ten patients died (eight of whom were first seen before 1972) of complications after intestinal perforation (5) or aspiration pneumonia (5). Of 29 survivors, three have persistent calcinosis and/or contractures. Improved outcome since 1972 probably relates to better clinical assessment, management of complications, and regulation of drug therapy.
HLA class I gene products are immunocytochemically detectable in the skeletal muscle of many patients with inflammatory conditions. We report three cases of juvenile dermatomyositis with minimal histologic abnormalities and no inflammation in the muscle biopsies. All three showed strong expression of HLA class I antigens on muscle fibers. Analysis of HLA class I expression is thus a useful marker for the diagnosis of inflammatory muscle disease, even in the absence of histologic changes.
A series of 33 patients with juvenile dermatomyositis was reviewed in terms of their prognosis in relation to their drug therapy. This retrospective study was intended to help clarify the use of various therapies in this rare, heterogeneous disease from our hospital's experience in the last 24 years. The results confirmed that oral corticosteroids should remain the undisputed first line of treatment. For more refractory, chronic patients, the results suggest that azathioprine should be the favored drug of first choice (in addition to corticosteroids). There may be a role for cyclosporine as a "rescue" treatment, but this needs to be further defined.