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At least 703 records · Page 39Linked to original sources
PubMed2003

Inflammatory myopathy in a patient with cutaneous findings of pityriasis rubra pilaris: a case of Wong's dermatomyositis.

We report the case of a 46-year-old woman presenting cutaneous lesions similar to those of pityriasis rubra pilaris, characterized by diffuse scaling erythroderma and palmoplantar keratoderma; skin biopsy showed follicular hyperkeratosis with a perivascular lymphocytic infiltrate. One year later she developed an inflammatory myopathy; on the basis of clinical features, a diagnosis of dermatomyositis was made. Treatment with prednisone and hydroxychloroquine led to complete control of the cutaneous and muscular involvement, which was maintained during prednisone tapering.

Anti-Inflammatory Agents↗
PubMed2004

Electric shock wave lithotripsy (ESWL) as a pain control measure in dermatomyositis with calcinosis cutis-old method, new discovery.

A 23-year-old Chinese man with dermatomyositis associated calcinosis cutis received electric shock wave lithotripsy (ESWL) as an alternate to a conventional pharmacological regimen to reduce pain associated with the complications of subcutaneous calcinosis nodules. He became symptom and opioid free after two courses of ESWL. No significant adverse effect had been noted. ESWL may serve as a means of pain killing in patients suffering from debilitating pain caused by complicated calcinosis cutis.

Adult↗
PubMed2004

Clinical significance of von Willebrand factor in patients with adult dermatomyositis.

Because the clinical significance of von Willebrand factor (vWF), a marker of endothelial injury, has not been well studied in adult patients with dermatomyositis (DM), we evaluated whether plasma vWF levels are useful as an index of disease activity in these patients. We measured plasma vWF antigen levels in 11 patients with active adult DM, 13 patients with inactive DM, and 18 healthy subjects using an enzyme-linked immunosorbent assay. The association of vWF level with clinical condition and muscle-derived enzyme leakage among DM patients was examined using analysis of covariance and logistic regression analysis. Furthermore, we studied the effects of treatment on the vWF antigen level. The mean vWF antigen level was significantly higher in active DM patients than in inactive DM patients and healthy subjects. Higher vWF levels were associated with clinical symptoms, such as general fatigue, fever, and muscle weakness. They were also associated with the levels of aspartate aminotransferase, alanine aminotransferase, and aldolase, but not with those of lactate dehydrogenase and creatine kinase (CK). vWF antigen was correlated with muscle enzymes except for CK. The plasma vWF levels in six patients with active DM significantly decreased after successful corticosteroid treatment. Plasma vWF level may be considered a useful marker of disease activity in adult DM patients.

Adolescent↗
PubMed2004

Recurrent extensor tendon rupture in adult-onset dermatomyositis: a case report.

We report on a case in which recurrent extensor tendon rupture was caused by tenosynovitis without skin ulcer and calcinosis in adult-onset dermatomyositis involving the hand. Proliferative tenosynovitis of the extensor tendons led to recurrent elongation or rupture of the extensor tendons, and tendon graft produced the good postoperative result in this case.

Adult↗
PubMed2005

Cytomegalovirus-induced interstitial pneumonitis in a patient with dermatomyositis.

Dermatomyositis (DM) patients might present with pulmonary involvement as the first manifestation or during the follow-up period. It is sometimes difficult to determine whether the clinical manifestations related with pulmonary involvement are due to DM or to an infectious process. We report a case of DM patient who developed interstitial pneumonitis induced by cytomegalovirus (CMV) while receiving immunosuppressive treatment. To the best of our knowledge, this is the second report in the literature of interstitial pneumonitis secondary to CMV infection in a patient with DM.

Adult↗
PubMed2006

Spontaneous pneumomediastinum and dermatomyositis may be a not so rare association: report of a case and review of the literature.

One patient with dermatomyositis (DM) who developed spontaneous pneumomediastinum (SPnM) is described. A review of 15 previously reported cases shows that this is a complication of DM but not of polymyositis. It occurs in young patients with recent onset of disease characterized by cutaneous vasculopathy, and patients treated with immunosuppressive drugs have, in general, better outcomes. We suggest that the onset of SPnM in DM patients must alert to possible vasculitic activity and that immunosuppressive agents with progressive tapering of corticosteroids are part of the most reasonable approach in these cases.

Adrenal Cortex Hormones↗
PubMed2005

Early assessment of rapidly progressive interstitial pneumonia associated with amyopathic dermatomyositis.

Amyopathic dermatomyositis (ADM) is occasionally complicated by rapidly progressive interstitial pneumonia (RPIP), and in such cases, diffuse alveolar damage (DAD) is usually diagnosed at autopsy. Here, we present three patients with RPIP accompanied by ADM in whom lung disease was assessed at an early stage. High-resolution computed tomography (HRCT) carried out before the onset of dyspnoea revealed uniformly subpleural reticular opacity with faint ground-glass attenuation. At that stage, surgical lung biopsies from two patients showed histological patterns typical of cellular nonspecific interstitial pneumonia (NSIP). Despite pulse methylprednisolone and subsequent high-dose oral administration of prednisolone, lung disease progressed in all patients, with extensive areas of ground-glass opacity and consolidation observed in HRCT scans. DAD was confirmed histologically in one case. Additional administration of cyclosporine, pulse cyclophosphamide or high-dose intravenous administration of immunoglobulin rescued all patients. Our data suggest that ADM-associated interstitial pneumonia takes an aggressive course even when the radiological and histological features are consistent with NSIP. Aggressive combination therapy with high-dose steroids and immunosuppressive agents is required as early as possible for patients with this life-threatening disorder.

Adult↗
PubMed2006

Dermatomyositis complicated with Kaposi sarcoma: a case report.

We describe the case of a 75-year-old Italian woman affected by dermatomyositis (DM) treated with steroid, high-dose intravenous immunoglobulins (IVIgs) and cyclophosphamide (CPX), taken orally. After a few months, the patient presented multiple red vascular skin lesions diagnosed as Kaposi sarcoma (KS). Steroid was furtherly reduced, and CPX was stopped. We put the patient on chemotherapy with intravenous infusion of vinblastine and vincristine on alternate weeks obtaining the remission of KS. DM is well controlled by a low-dosage steroid and high-dose IVIgs.

Aged↗
PubMed2005

Successful treatment of rapidly progressive interstitial pneumonia with autologous peripheral blood stem cell transplantation in a patient with dermatomyositis.

Aggressive autoimmune diseases are often treated by intensive immunosuppressive treatment such as high-dose methylprednisolone and intravenous cyclophosphamide. Autologous hematopoietic stem cell transplantation can facilitate high-dose immunosuppressive therapy (HDIT), which is myeloablative. We describe a 54-year-old female patient with rapidly progressive and refractory interstitial pneumonia due to dermatomyositis, which was successfully treated with high-dose cyclophosphamide and autologous blood stem cell transplantation. Following transplantation, dyspnea disappeared, and arterial blood gas analysis and respiratory function test showed marked improvement. This improvement was confirmed by diminished interstitial shadows on chest X-ray and computed tomography scans. Eighteen months after transplantation, the patient is doing well without symptoms and signs of interstitial pneumonia.

Combined Modality Therapy↗
PubMed2006

Prognosis and mortality of polymyositis and dermatomyositis patients.

The objective of this study was to assess the long-term outcome of polymyositis (PM) and dermatomyositis (DM) and the factors predictive of this outcome in a nationwide series in Finland. One hundred and seventy-six patients with PM and 72 patients with DM diagnosed in Finland in 1969-1985 were selected from the national hospital discharge register according to the diagnostic criteria of Bohan and Peter and followed up until death or till the end of August 1995. Gender, age, delay of therapy, serum creatine kinase, erythrocyte sedimentation rate, initial dose of corticosteroids and duration of cytostatic therapy were assessed as factors prognostic of death. The 5-year survival rate for PM was 75% [95% confidence interval (CI): 68-81%] and that for DM 63% (50-73%), and the respective 10-year survival rates were 55% (47-62%) and 53% (41-64%). The median survival for polymyositis was 11.0 years (95% CI: 9.5-13.3) and that for DM 12.3 years (5.5-20.7). The standardized mortality ratio for the combined group of PM and DM showed approximately threefold mortality compared to the general population. Compared to PM patients, the subjects with DM had a 1.47-fold (95% CI: 0.99-2.12) age- and sex-adjusted risk of mortality. Except for age in both groups and the delay in diagnosis in the PM group, no other individual factor reached significance as a predictor of death. However, cancer had a hazard ratio (HR) of 2.16 for death (95% CI: 0.95-4.50) in the DM group and 1.99 (95% CI: 1.01-3.94) in the PM group. A comparison of the causes of death in the PM and DM groups showed that the patients with DM had a greater risk of dying from cancer (age-adjusted HR 5.11, 95% CI: 2.31-11.3). According to this nationwide analysis of survival and its prognostic factors in patients with PM and DM, the latter group had an increased age-adjusted risk for mortality compared to the former. The difference seems to be mostly explained by the fact that the patients with DM had a higher risk of dying from cancer.

Adrenal Cortex Hormones↗
PubMed2006

Treatment of early and refractory dermatomyositis with infliximab: a report of two cases.

The idiopathic inflammatory myopathies embody the largest group of acquired and potentially treatable causes of skeletal muscle weakness. The three major groups of this disorder are polymyositis (PM), dermatomyositis (DM), and inclusion body myositis. Corticosteroids continue to be the mainstay of initial treatment in the majority of cases of PM/DM. The treatment of refractory disease can be challenging despite the utilization of the medications currently available. We report two patients with refractory DM who were treated with infliximab. We describe their presentation, clinical course, treatment, and outcomes.

Adult↗
PubMed2000

Efficacy of early treatment of severe juvenile dermatomyositis with intravenous methylprednisolone and methotrexate.

A pilot study was conducted to assess the efficacy of early treatment of severe juvenile dermatomyositis (JDMS) patients with intravenous methylprednisolone (IVMP) and methotrexate (MTX). Twelve children diagnosed with severe JDMS were treated with IVMP and MTX. Six patients were treated early (within 6 weeks of the diagnosis) while in the other six patients, MTX was started 5-72 months after the diagnosis was made. The clinical responses of the patients to treatment, including alterations in muscle strength, muscle enzyme levels and corticosteroid dosage as well as the development of side-effects, were recorded. The indications for starting the treatment were defined and documented. The primary measures of response were resolution of the clinical indications for treatment, decreased activity of the disease manifestations and tapering of the corticosteroids to the minimal dose or discontinuation without clinical or biochemical flare. The main indications for starting IVMP and MTX were dysphagia and severe cutaneous vasculitis. All the patients received MTX orally for at least 8 months, as well as IVMP (30 mg/kg/dose), but none of the patients was on additional second-line treatments. The six patients who were treated early with MTX showed a significant clinical improvement. In five out of the six, the corticosteroid dosage was eventually reduced to <5 mg/day. None of them developed calcinosis. In contrast, two of the six patients who were treated late with MTX developed calcinosis. This study suggests that MTX and IVMP are a useful combination in the early treatment of severe JDMS. Given the fact that our sample was small, further studies in a controlled trial are necessary to confirm these findings.

Administration, Oral↗
PubMed1999

Clinico-laboratory characteristics of patients with dermatomyositis accompanied by rapidly progressive interstitial lung disease.

The clinico-laboratory features of 16 patients with dermatomyositis (DM) were compared between patients with accompanying rapidly progressive interstitial lung disease (RP-ILD, n = 7) and those with chronic interstitial lung disease (C-ILD, n = 9), and also between deceased (seven RP-ILD and three C-ILD) and living patients (six C-ILD). The extent of muscle weakness of the extremities and frequency of autoantibody positivity were significantly lower in DM patients with RP-ILD than in DM patients with C-ILD. Furthermore, significantly lower serum creatine kinase/lactate dehydrogenase levels (0.26+/-0.27) were found in the 10 patients who died than in the six living patients (1.21+/-1.09). A higher CD4+/CD8+ T-lymphocyte ratio in the peripheral blood (3.51+/-2.65) was detected in the four DM patients with RP-ILD who died than in the six living DM patients with C-ILD (1.22+/-0.49).

Adult↗
PubMed2000

Elevation of serum soluble tumour necrosis factor receptors in patients with polymyositis and dermatomyositis.

The aim of the study was, to examine the relationship between serum levels of soluble tumour necrosis factor receptors (sTNF-R) and the gene expression of two types of receptor for TNF (TNF-R), a 55 a receptor (TNF-R1) and a 75 kDa receptor (TNF-R2), bloodin peripheral mononuclear cells (PBMC) from patients with polymyositis and dermatomyositis (PM/DM). Soluble tumour necrosis factor receptor 1 (sTNF-R1) and soluble tumour necrosis factor receptor 2 (sTNF-R2) levels in sera from patients were measured by enzyme-linked immunosorbent assay. Expression of TNF-R1 and TNF-R2 mRNAs in PBMC was analysed by Northern blotting. Serum sTNF-R1 and sTNF-R2 levels were elevated significantly in 25 patients with active-stage PM/DM, compared to those in 18 patients with inactive-stage PM/DM and 32 normal controls. Serum concentrations of sTNF-R1 and sTNF-R2 were correlated with PM/DM disease activity. TNF-R1 gene expression was enhanced in freshly isolated PBMC from patients with active-stage PM/DM. In contrast, TNF-R2 mRNA was expressed constitutively in patients with active-stage PM/DM and in normal controls. The expression of TNF-R1 and TNF-R2 mRNAs in PBMC and elevation of their soluble forms in the sera of patients with active-stage PM/DM suggest increased proteolytic cleavage of cell surface TNF-R from PBMC in patients with active-stage PM/DM, and that sTNF-R may regulate TNF-alpha-mediated muscle fibre damage in PM/DM.

Adolescent↗
PubMed2001

Amyopathic dermatomyositis associated with cervical cancer.

We here report a 44-year-old female patient who is the first documented case of cervical carcinoma with amyopathic dermatomysitis (ADM). The association of ADM with malignancy is clearly stated in the literature. This patient first presented with classic cutaneous findings of dermatomyositis with no signs and symptoms of muscle involvement for the first 7 months following the diagnosis. When this case has been worked up for malignancy by invasive procedures such as cervical cone biopsy, an increase in muscle enzymes and exacerbation of skin findings were detected.

Adult↗
PubMed2000

Immunogenetic associations in childhood dermatomyositis.

Childhood dermatomyositis is a multisystem autoimmune disease characterized by acute and chronic inflammatory infiltrate and vasculitis seen primarily in skin and muscle. The disease occurs in genetically susceptible individuals with a yet undescribed environmental trigger. Genetic associations in this disease demonstrate disease susceptibility as well as chronicity and severity and likely play a role in disease pathogenesis. The majority of the associations to date have been seen with the HLA alleles, with additional immune responsive and influential genes being increasingly investigated.

Child↗
PubMed2005

Long-term outcomes in juvenile dermatomyositis: how did we get here and where are we going?

Juvenile dermatomyositis (JDM) affects two to four children per million. Prior to treatment with corticosteroids, JDM had a high mortality rate (> 30%) and left 50% of those who survived with serious permanent impairments. After the introduction of corticosteroids, mortality rapidly dropped to less than 10%, and is currently reported to be less than 2% to 3%. Because most children now survive this illness, there is greater interest in long term outcomes. However, review of the literature shows while much is known about mortality, relatively little is known about long term outcomes such as physical function, quality of life, pain, educational and vocational achievement, patient satisfaction, and ongoing disease activity. Furthermore, the literature that has been published has not typically used the same outcomes making comparisons across studies difficult. Current efforts to identify key outcomes and validate measures for those outcomes will allow researchers in the future to provide this much needed information.

Adolescent↗
PubMed2005

Interstitial lung disease in polymyositis and dermatomyositis.

Interstitial lung disease occurs in approximately one-third of patients with polymyositis and dermatomyositis (PM/DM) and has an adverse effect on survival. It is commonly a component of early PM/DM and can precede the onset of muscle or skin disease. Its most common histopathology is nonspecific interstitial pneumonia. This is a more benign pattern, with respect to response to immunosuppression and also long-term survival, than the pattern of usual interstitial pneumonia seen in idiopathic pulmonary fibrosis. The clinical course of PM/DM lung disease is heterogeneous. Progressive and nonprogressive disease needs to be distinguished by clinical and physiologic monitoring to avoid over-treatment. Patients with ongoing functional deterioration mostly benefit from immunosuppression. The experience with corticosteroid monotherapy is discouraging but cyclophosphamide, given as daily oral or intravenous pulse therapy together with corticosteroids, was found to be beneficial in many patients. Other immunosuppressants may be of benefit as well, but the weight of the current evidence supports the use of cyclophosphamide first.

Dermatomyositis↗