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At least 1,009 records · Page 56Linked to original sources
PubMed2003

Case report: severe central nervous system involvement in juvenile dermatomyositis.

We present 3 patients with juvenile dermatomyositis (JDM) and severe central nervous system (CNS) complications. All patients had at least 4 positive criteria of Bohan and Peter, which confirmed a definite diagnosis of JDM. They were all male, and had a relatively high creatinine kinase value at admission (1532-4260 U/l). Besides, progressive proximal muscle weakness and rash, one patient presented with rapid irreversible decline of vision. Ophthalmologic examination showed active vasculitis of the retina. After 2 weeks of treatment with immunosuppressive drugs and being in improved, relatively stable clinical condition, all 3 patients developed generalized tonic-clonic convulsions. Other causes of the neurological symptoms could be excluded. In all 3 patients, the course of JDM was fatal. The clinical symptoms and further investigations in our patients show CNS involvement in JDM. Although rarely reported, CNS vasculopathy can be a serious and life-threatening complication of JDM.

Angiokeratoma↗
PubMed1992

[An autopsy case of dermatomyositis associated with interstitial pneumonia probably due to cytomegalovirus infection].

An autopsy case of dermatomyositis (DM) associated with interstitial pneumonia probably due to cytomegalovirus infection is reported. After corticosteroid therapy for 1 month, a 79-year-old man with DM developed acute respiratory failure due to interstitial pneumonia and died in spite of intensive respiratory care. By polymerase chain reaction method (PCR), DNA of cytomegalovirus (CMV) was detected in the bronchoalveolar lavage fluid (BALF). CMV was also detected by the method of conventional virus culture from BALF. These findings suggested that initial infection or reactivation of CMV had occurred in the lungs. The autopsy specimen revealed the findings of interstitial pneumonia compatible with CMV pneumonitis, but without the presence of intranuclear inclusion bodies. Although the present case of interstitial pneumonia should not strictly be diagnosed as definite CMV pneumonitis without the presence of intranuclear inclusion bodies in the lung tissue, initial infection or reactivation of CMV in the lungs may have contributed to the pathogenesis of interstitial pneumonia. In other cases of collagen vascular disease associated with interstitial pneumonia, CMV or other viruses may contribute to the pathogenesis of interstitial pneumonia.

Aged↗
PubMed1992

[Ovarian cancer and dermatomyositis. A clinical case].

Dermatomyositis (DM) is associated to malignant neoplasia in up to one third of the cases. Not considering breast neoplasia, ovarian cancer is the malignancy most frequently associated with DM in women. This study shows the evolution and outcome of a case of this association managed in our unit. The principal features of similar cases reported in the literature are summarized.

Cystadenocarcinoma, Papillary↗
PubMedDate not supplied

Molecular detection of persistent Borrelia burgdorferi in a man with dermatomyositis.

A 40-year-old white man with a several year history of various immunologic disorders, including anti-Jo-1 autoantibody positive dermatomyositis, developed clinical Lyme disease after being biten by a tick. The patient was treated with oral tetracycline and his initial symptoms resolved; however, he suffered an exacerbation of his muscle disease which was difficult to control despite cytotoxic therapy. Antibiotic therapy was reinstituted after Borrelia burgdorferi was detected in the patient's peripheral blood leukocytes by the polymerase chain reaction (PCR). All serologic, T-cell stimulation, and western blot analyses, however, were negative. The patient's disease responded to oral ampicillin, probenecid therapy and concurrent cytotoxic therapy. Subsequent leukocyte PCR testing has been negative for the causative agent of Lyme disease. This case may provide an example of the in vivo immuno-modulatory effects of spirochetes in human autoimmune disease. In addition, this case emphasizes the potential clinical utility of PCR technology in evaluating the persistent sero-negative Lyme disease which may occur in immunocompromised individuals.

Administration, Oral↗
PubMed1992

Streptococcus pyomyositis occurring in a patient with dermatomyositis in a country with temperate climate.

We describe a man in whom pyomyositis developed in a temperate climate. Three facts make this case unique. First the pyomyositis developed in someone with underlying dermatomyositis, this being the second reported case to our knowledge. Second, the organism involved was a Streptococcus and not a Staphylococcus as in most cases described, and the course of the disease was acute and not subacute as is usually reported. Finally, contrary to most described cases, surgical drainage was not necessary, probably because of the early diagnosis. Pyomyositis should be considered a possible cause of localized pain in patients with underlying inflammatory muscle disease.

Adult↗
PubMed2003

[Polymyositis, dermatomyositis and inclusion body myositis, nosological aspects].

THREE GROUPS OF PRIMARY INFLAMMATORY MUSCLE DISEASES: The primary inflammatory muscle diseases comprise three main subsets: polymyositis (PM), dermatomyositis (DM) and inclusion body myositis (IBM). PM and DM are characterized by a proximal weakness that develops along weeks to months and by elevated creatine phosphokinase levels. Cutaneous involvement including both erythema and edema and infantile or adult onset are DM specific. PM and IBM only concern adults. Several PM/DM manifestations must be searched for because of their severity: swallowing disorders, various mechanisms of respiratory dysfunction (swallowing pneumopathies, interstitial lung disease, respiratory muscle deficiency) and cardiac involvement. DIAGNOSTIC ELEMENTS FOR PM AND DM: Two investigations, beside biopsy, are particularly useful: muscle MRI imaging showing inflammatory pattern and specific detection of antisynthetase autoantibodies (PM/DM with interstitial lung disease) and anti-Mi-1 and 2 in DM. PHYSIOPATHOLOGICAL DATA: PM and DM differ in their histological and physiopathological characteristics: perivascular B and CD4 lymphocyte infiltrates and complement deposits at the origin of humoral induced vascular disease in DM and perimysial CD8 lymphocytes inducing a cellular mediated cytotoxic injury in PM. Class I HLA antigen expression on the muscle fibers and production of cytokines play a crucial role in the pathogenesis of these two diseases. PM and DM may be associated with cancers, connective-tissue disease (overlap syndrome). Some PM are secondary to HIV, HTLV1 virus and toxoplasmosis infection. CHARACTERISTICS OF INCLUSION BODY MYOSITIS: IBM, the most frequent acquired myopathy after 50 years of age, is characterized by particular features: not only clinical (late onset, selective weakness, early distal involvement, slow course, unresponsiveness to corticosteroid and immunosuppressant agents); but also histological (rimmed vacuoles, filamentous inclusions) and pathogenic (cytotoxic and degenerative inflammatory process, similar to Alzheimer's disease, with beta-amyloid protein accumulation).

Adult↗
PubMed2003

Urticarial vasculitis and dermatomyositis in a patient with nasopharyngeal carcinoma.

Urticarial vasculitis has rarely been described in association with polymyositis. We report the case of a 37-year-old man with dermatomyositis and nasopharyngeal carcinoma who presented initially with urticarial vasculitis. The lesions of urticarial vasculitis were initially photodistributed, indicating photosensitivity. The patient was treated with systemic steroids, chemotherapy (cisplatin and fluorouracil), and radiation therapy. The tumor and urticarial vasculitis completely resolved, and the myositis improved.

Adult↗
PubMed1992

Juvenile dermatomyositis and pregnancy: report and literature review.

Reports of dermatomyositis (DM) and polymyositis complicating pregnancy are rare. Only nineteen cases have been published. We describe the first case in which juvenile DM appeared during pregnancy. An emergency cesarean section had to be performed due to fetal distress at 37 weeks of gestation. Patient and infant are doing well after 8 months of followup.

Adolescent↗
PubMed2004

Immunohistochemical analysis of adhesion molecule expression on muscle biopsy specimens from patients with juvenile dermatomyositis.

OBJECTIVE: To assess expression of intercellular adhesion molecule-1 (ICAM-1) and vascular cell adhesion molecule-1 (VCAM-1) on muscle biopsy specimens from patients with untreated juvenile dermatomyositis (JDM). METHODS: Histochemical and immunohistochemical tests for ICAM-1 and VCAM-1 were performed on serial frozen sections from 27 JDM muscle biopsy specimens. ICAM-1 and VCAM-1 expression was analyzed on capillaries, perimysial and endomysial large vessels, and muscle fibers. Expression was assessed and graded semiquantitatively. RESULTS: Increased ICAM-1 expression was observed on capillaries and perimysial large vessels on semiquantitative analysis, and was statistically more evident than on endomysial large vessels. In all cases, only a few muscle vessels showed expression of VCAM-1. Expression of ICAM-1 and VCAM-1 was observed on few muscle fibers. CONCLUSION: The observation of ICAM-1 expression on muscle vessels, mainly on capillaries of patients with untreated JDM compared to controls, and VCAM-1 expression to a lesser extent, mostly on muscle vessels surrounded by inflammatory infiltrate, supports the participation of these adhesion molecules in the pathologic mechanism of vascular injury in JDM.

Adolescent↗
PubMed2004

[A case of amyopathic dermatomyositis with interstitial pneumonia: successful treatment with a combination of prednisolone and cyclosporin A].

A 53-year-old woman visited our hospital in July 2002 with complaints of skin eruptions on the face and extremities, dry cough and dyspnea. A diagnosis of amyopathic dermatomyositis (ADM) was made on the basis of characteristic skin lesions and skin biopsy findings with slight muscle symptoms and mild elevation of muscle enzymes. Interstitial shadows were observed in both lower lung fields on chest radiographs, and thoracoscopic lung biopsy specimens revealed a diffuse alveolar damage (DAD) pattern. After induction of chemotherapy with prednisolone (50 mg/day) and cyclosporin A (150 mg/day), the respiratory symptoms and interstitial shadows were gradually reduced. Although it is known that interstitial pneumonia has an acute course and poor prognosis in ADM, the present case responded well to the chemotherapy with prednisolone and cyclosporin A.

Anti-Inflammatory Agents↗
PubMed2004

Skin involvement in juvenile dermatomyositis is associated with loss of end row nailfold capillary loops.

OBJECTIVE: To determine associations of dermatological findings in children with juvenile dermatomyositis (JDM) with specific nailfold capillary (NFC) structural abnormalities. METHODS: Sixty newly diagnosed, previously untreated children who met the Bohan-Peter criteria for definite JDM were seen between 1993 and 2002. They were classified by duration of untreated disease and by a disease activity score (DAS) composed of separate subscores for dermatological (DAS skin) and musculoskeletal (DAS muscle) findings. Routine NFC measurements yielded the number of end row loops, arboreal (bushy), and dilated capillary loops. Laboratory testing included muscle enzymes, von Willebrand Factor Antigen, and neopterin. RESULTS: DAS skin, but not DAS muscle, was associated with NFC end row capillary loss (rs = -0.394, p = 0.008). End row capillary loss (reflecting avascularity), arboreal (bushy), and dilated capillary loops (reflecting change in vascular morphology) were each associated with longer untreated symptom duration (rs = -0.401, rs = 0.534, rs = 0.371). CONCLUSION: End row capillary loss measured by NFC was associated with the dermatological, but not musculoskeletal manifestations of JDM, suggesting that damage to skin and muscle may each have distinct disease pathophysiology. In JDM, skin involvement indicates a vasculopathy that progresses with increasing duration of untreated disease and is not revealed by standard serological laboratory tests. We propose that the cutaneous manifestations of JDM are associated with vascular disease and warrant aggressive therapy.

Adolescent↗
PubMedDate not supplied

Dermatomyositis associated with Sjögren's syndrome: VEGF involvement in vasculitis.

Two patients with dermatomyositis complicated with Sjögren's syndrome (SjS), are reported. Both patients exhibited sensory-dominant polyneuropathy, compatible with neurologic involvement in SjS. Vascular endothelial growth factor (VEGF) levels were increased in their plasma. Histological examination demonstrated vasculitic changes in biopsied specimens of muscle and salivary glands from the patients, and VEGF was overexpressed in the vasculitic lesions. These findings suggest that VEGF overexpression was associated with the development of vasculopathy in skeletal muscle and salivary glands and possibly in the peripheral nervous system.

Dermatomyositis↗
PubMed2004

HLA-DRB1*15021 is the predominant allele in Japanese patients with juvenile dermatomyositis.

OBJECTIVE: To investigate HLA molecules and genes in Japanese patients with juvenile dermatomyositis (JDM). METHODS: Twelve patients (8 girls and 4 boys) with ages of onset between 3 and 15 years were included. HLA class I antigen phenotypes were serologically typed by the Terasaki-NIH standard method. DNA was extracted from peripheral blood leukocytes using the phenol-chloroform extraction procedure, and stored at -70 degrees C until use. Genomic DNA for HLA-DRB1, HLA-DQA1, and HLA-DQB1 alleles in JDM patients and controls was determined by the direct sequence method. RESULTS: HLA-A24 and B52 were each detected in 7 cases (OR = 0.86, 5.02, p = 0.930, 0.006, respectively). HLA-DRB1*15021 was observed in 7 patients. This was significantly more frequent than occurred in the controls (OR = 5.72, p = 0.002). Seven patients out of 12 (58%) had the combination HLA-B52, DRB1*15021, DQA1*0103, and DQB1*0601. CONCLUSION: Our results suggest that the susceptibility gene for JDM either is HLA-DRB1*15021 or is present near the HLA-DRB1 locus. This differs from previous reports that describe the association with HLA-DQA1*0501 in Caucasian patients with JDM. The combination HLA-B52, DRB1*15021, DQA1*0103, and DQB1*0601 may contribute to the pathogenesis of JDM in Japanese patients.

Adolescent↗
PubMed1992

Juvenile dermatomyositis.

Juvenile dermatomyositis is a relatively rare, multisystem disease characterized by a nonsuppurative myositis which causes symmetrical weakness, rash and vasculitis; this last can affect the gastrointestinal tract and the myocardium. Late development of calcinosis is seen in approximately two thirds of patients. Its etiology is unknown, although there are clues that it may be an unusual response to a viral infection. Some 50% of children will have a very acute, rapidly progressive disease, while the remainder may present subacutely with rash and a gradually progressive weakness of muscles, joint contractures and very occasionally calcinosis. When there is acute muscle damage, the creatine phosphokinase will be raised, but it is not uncommon to have a normal erythrocyte sedimentation rate, and antinuclear antibodies are usually present. Early in acute cases immune complexes will often be detected. In the presence of vasculitis, monitoring the disease by levels of von Willebrand's factor 8 antigen may be helpful. Although the prognosis for survival has steadily improved, it remains a serious illness and death can occur in the acute phase due to myocarditis, progressive unresponsive myositis, perforation of the bowel as a sequel to vasculitis ulceration or occasionally lung involvement. Intercurrent infections during the course of the disease also give rise to problems. In its management, there is still a question as to whether intravenous pulses of methylprednisone might be more valuable than oral corticosteroids; in either case it must be given in adequate amounts early in the course of the disease to control muscle inflammation. Once this is controlled rehabilitation commences.(ABSTRACT TRUNCATED AT 250 WORDS)

Adrenal Cortex Hormones↗
PubMed1992

Juvenile dermatomyositis.

Juvenile dermatomyositis is a chronic inflammatory disease of skeletal muscle accompanied by skin involvement. It is a presumed autoimmune disease. Other organs may be involved. Corticosteroids are standard therapy and are associated with decreased mortality. Significant morbidity may occur.

Child↗
PubMed2005

Differences in clinical features and prognosis of interstitial lung diseases between polymyositis and dermatomyositis.

OBJECTIVE: To assess the difference in clinical features and prognosis of patients with interstitial lung disease (ILD) comparing polymyositis (PM) and dermatomyositis (DM). METHODS: Medical records of 28 ILD patients with PM/DM (16 PM-ILD, 12 DM-ILD) were reviewed retrospectively. RESULTS: Serum CPK concentrations were significantly higher in PM-ILD than in DM-ILD. Bronchoalveolar lavage analysis showed that the percentages of lymphocytes and eosinophils were significantly higher in DM-ILD than in PM-ILD. Ten patients (5 PM-ILD, 5 DM-ILD) underwent surgical lung biopsy, and 3 (3 DM-ILD) had an autopsy. Nonspecific interstitial pneumonia (NSIP) was found in 7 (4 PM-ILD, 3 DM-ILD) and usual interstitial pneumonia (UIP) in 3 (1 PM-ILD, 2 DM-ILD). Interestingly, diffuse alveolar damage (DAD) was found in 3 patients with DM-ILD, who all died of deterioration of ILD; but no one with PM-ILD had DAD. Corticosteroid treatment alone achieved a favorable response in 6 patients (37.5%) with PM-ILD, but in only one (8.3%) with DM-ILD. Administration of cyclosporine in the early phase of onset benefited 4 corticosteroid-resistant patients with DM-ILD. Conclusively, survival in DM-ILD was significantly worse than that in PM-ILD. CONCLUSION: DM-ILD is more refractory to corticosteroid therapy, resulting in poorer prognosis compared with PM-ILD. These data indicate that intensive therapy, including cyclosporine, should be considered for DM-ILD.

Bronchoalveolar Lavage Fluid↗
PubMed2004

Dermatomyositis-like syndrome following acute toxoplasmosis.

The case of a patient who developed an acute dermatomyositis-like syndrome upon infection by Toxoplasma gondii is reported. Emphasis is given on the chronology of the anti-Toxoplasma IgM and IgG antibody rise, the muscle enzyme movements, the antinuclear antibody development in comparison to the clinical symptoms and their follow-up after Toxoplasma treatment. The close monitoring of the sequential clinical and biological pattern allows insights into the infectious and auto-immune diseases presented by this patient. Taking into account other reported cases and the clinical presentation of this patient some conclusions and recommendations are inferred.

Animals↗
PubMed2005

Bilateral ocular myositis as a late complication of dermatomyositis.

The association of bilateral ocular myositis with primary inflammatory muscle disease is rare. We describe a 30-year-old man with a 2-month history of eyelid edema, erythema, and orbital pain, occurring in the course of previously undiagnosed and untreated dermatomyositis (DM). Although ocular myositis is a very rare manifestation of DM, it might be overlooked by clinicians who are not aware of this complication.

Adult↗