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PubMed2005

[Two children with skin rash and muscle weakness: juvenile dermatomyositis].

Two girls aged 1.5 and 3 years, presented with a skin rash, loss of proximal muscle power and malaise. The younger girl recovered quickly after a short course of corticosteroids but the elder girl proved more difficult to treat effectively with corticosteroids, methotrexate, ciclosporin, intravenous immunoglobulins and hydroxychloroquine. This combination of symptoms should make one consider the diagnosis of juvenile dermatomyositis (JDM). To make the diagnosis of JDM, a characteristic skin rash, proximal muscle weakness, elevated muscle enzymes and, possibly, an abnormal EMG or muscle biopsy should be present. Treatment consists, of steroids and, if necessary, immunosuppressive agents or intravenous immunoglobulins. If cutaneous lesions are serious or persistent, hydroxychloroquine may also be prescribed.

Anti-Inflammatory Agents↗
PubMedDate not supplied

Dermatomyositis associated with impairment in both muscle aerobic and anaerobic function.

We report a 50-year-old Japanese woman with dermatomyositis in whom an anti-mitochondrial antibody was detected. Muscle biopsy demonstrated periodic acid Schiff- (PAS) positive vacuoles in addition to infiltrates of mononuclear cells. Histochemical analysis showed reduced phosphorylase activity. An aerobic exercise test demonstrated that the concentrations of serum lactate and pyruvate were elevated before corticosteroid therapy but decreased after the therapy. On the other hand, in forearm ischemic exercise tests, the responses of serum lactate and pyruvate were attenuated before corticosteroid therapy but recovered after the therapy. These findings indicate that an inflammatory mechanism interfered with myophosphorylase activity and muscle aerobic function.

Adrenal Cortex Hormones↗
PubMed2004

Dermatomyositis.

A 51-year-old woman presented with weakness in her arms and legs and an eruption on the dorsal aspects of the hands, upper back, and face. Histopathologic features showed vacuolar alteration of the basal layer, a thick basement membrane, and deposits of connective-tissue mucin in the papillary dermis and the upper reticular dermis. Dermatomyositis is an idiopathic disease that is characterized by specific cutaneous manifestations and myopathy, which may be associated with occult malignancy. Treatment options include corticosteroids and other immunosuppressive agents such as methotrexate, cyclosporine, mycophenolate mofetil, and azathioprine.

Dermatomyositis↗
PubMedDate not supplied

Disease course, frequency of relapses and survival of 73 patients with juvenile or adult dermatomyositis.

OBJECTIVE: Our aim is to present the disease course, frequency of relapses and survival of juvenile and adult dermatomyositis (JDM/DM) patients. METHODS: Analysis was performed using data on 73 patients. The median follow-up for 38 JDM patients was 32 months and 78 months for 35 adult DM patients. RESULTS: 23/38 JDM patients (60%) had monophasic, 12/38 (31.6%) had polycyclic and 3/38 (7.9%) had chronic disease. Among children treated only with glucocorticoids, 12/20 (60%) had monophasic and 8/20 (40%) had polycyclic disease. 10/17 (58.8%) children, who required second-line immunosuppressive agents, had monophasic and 4/17 (23.5%) had polycyclic disease. 18/35 DM (51.4%) patients had monophasic, 13/35 (37.1%) had polycyclic, 1/35 (2.9%) had chronic disease and 3/35 (8.6%) had fulminant myositis. Among DM patients requiring only glucocorticoids, 12/20 (60%) were monophasic and 8/20 (40%) were polycyclic. In patients requiring second-line immunosuppressive agents, 6/15 patients (40%) had monophasic and 5/15 (33.3%) had polycyclic disease. Among patients with polycyclic disease, the risk of relapse was higher during first year than later in the disease course. None of the JDM patients have died, while 4 disease-specific deaths occurred in adult patients. There was no significant difference between the survival of JDM and DM patients. DISCUSSION: There was no correlation between relapse-free survival and the initial therapeutic regimen. Many of our patients had polycyclic or chronic disease. As relapses can occur after a prolonged disease-free interval, patients should be followed for at least 2 years. Although we found a favourable survival rate, further investigations are needed to assess functional outcome.

Adolescent↗
PubMed2005

Bone mineral density in children and adolescents with systemic lupus erythematosus, juvenile dermatomyositis, and systemic vasculitis: relationship to disease duration, cumulative corticosteroid dose, calcium intake, and exercise.

OBJECTIVE: To describe the frequency of abnormal bone mineralization in a population of children with juvenile systemic lupus erythematosus (JSLE), juvenile dermatomyositis (JDM), and systemic vasculitis; and to investigate the relationship of bone mineral density (BMD) to cumulative corticosteroid dose, disease duration, Tanner stage, calcium intake, and exercise in these patients. METHODS: A retrospective chart review of children attending the pediatric rheumatology clinic at British Columbia's Children's Hospital was conducted to obtain demographic data (sex, ethnicity, disease duration, cumulative corticosteroid dose, and mean daily corticosteroid dose). All patients had at least one BMD measurement by dual energy x-ray absorptiometry (DEXA) at lumbar spine, hip, and total body. BMD was expressed as g/cm2 and Z scores; an abnormal Z score was defined as </= -1.5. Daily calcium intake was calculated. Physical activity was scored using a validated 7 day physical activities questionnaire. RESULTS: A total of 36 patients were studied. Twenty-five patients had SLE, 7 had JDM, and 4 had systemic vasculitis. Fourteen subjects were Caucasian, 13 Asian, 6 East Indian, and 3 Canadian First Nations. An abnormal Z score at one or more sites was found in 10/25 (40%) with JSLE, and in 3/11 (27%) JDM/vasculitis patients. The mean Z scores (+/- SD) for lumbar spine were -1.02 (+/- 1.2), for hip -0.88 (+/- 1.3), and for total body -0.77 (+/- 1.5). Compared to children with normal bone densities, those with lower bone density tended to be younger (13.5 +/- 2.2 vs 15.5 +/- 1.7 yrs), received higher corticosteroid dosages at the time of the study (0.78 +/- 0.6 vs 0.35 +/- 0.2 mg/kg), and were more often prepubertal (OR for total body scores 5, 95% CI 0.7-46). CONCLUSION: Decreased bone density is common in children with SLE and other systemic rheumatic diseases. Age, corticosteroid dose, and pubertal stage all appear to have some influence on bone mass in these children.

Adolescent↗
PubMed2005

The reliability of an aerobic and an anaerobic exercise tolerance test in patients with juvenile onset dermatomyositis.

OBJECTIVE: To investigate the reliability of an aerobic and an anaerobic exercise test in patients with juvenile dermatomyositis (JDM). METHODS: Sixteen patients with JDM (mean age 13.85 +/- 6.4 yrs, range 6.7-27.2) participated. Anaerobic exercise capacity was measured using the Wingate Anaerobic Exercise Test (WAnT). Aerobic exercise test was performed using a graded exercise test to volitional exhaustion on an electronically braked cycle ergometer. Patients were tested and retested within 12.8 +/- 5.7 days. RESULTS: Correlation coefficients and other reliability statistics indicated that peak power and mean power on the WAnT and peak oxygen uptake (VO2peak) and maximal workload (Wmax) on the aerobic exercise test could be reliably assessed in patients with JDM. Pearson (R) and intraclass correlation coefficients (ICC) were > 0.85, and typical error was < 20% for the peak power and mean power on the WAnT. On the aerobic exercise test the R and ICC were > 0.95, and typical error was < 6% for the VO2peak and Wmax. CONCLUSION: We found acceptable reliability for the WAnT and very good reliability for the aerobic exercise test. This indicated that these exercise tests could be performed reliably in patients with JDM.

Adolescent↗
PubMed2005

A child with dermatomyositis and a suspicious lymphadenopathy.

Dermatomyositis (DM) in adults is frequently associated with cancer. In contrast, juvenile DM (JDM) is predominantly idiopathic and rarely reported with occult neoplasm. We describe a patient who presented with DM that was found to be a paraneoplastic manifestation of nasopharyngeal carcinoma. Although rare, paraneoplastic JDM must be suspected in the presence of unusual features such as elevated inflammatory markers or lymphadenopathy. Accurate clinical assessment including appropriate biopsies is needed before starting glucocorticoid therapy.

Adolescent↗
PubMed2005

Carcinoma of the gall bladder presenting as dermatomyositis.

Dermatomyositis (DM), manifested as paraneoplastic syndrome, is not a very common clinical entity but its association with various internal malignancies is well-documented in literature. We present such a case of DM associated with characteristic skin lesions and subacute onset of proximal muscle weakness, acquired from a very rare malignancy like adenocarcinoma of gall bladder.

Adenocarcinoma↗
PubMed1992

[A case of dermatomyositis associated with thymic abnormalities].

A case of dermatomyositis (DM) associated with thymic cyst with lymphoid follicle formations has been reported. A 57-year-old man developed polyarthralgia, muscle weakness, heliotrope rash and Gottron's sign. Laboratory findings showed elevated values of creatine-phosphokinase, aldolase and transaminase. He was diagnosed as DM and was treated with prednisolone 50 mg/day. Muscle weakness was improved immediately, although rash persisted unchangeably. He was examined for coexistence of malignant tumor which had been reported frequently as associated with DM. Enlargement of thymus was found by computer tomography and then thymectomy was performed. In his thymus, lymphoid follicle formations, which are as often encountered in other autoimmune diseases, were found. So far, the beneficial effect of thymectomy on the improvement of immunological abnormalities not only in patients with myasthenia gravis but also in patients with other autoimmune diseases has been recognized. In this case, intractable rash in DM improved after thymectomy. Further study needs to clarify the relationships between DM and thymic abnormalities.

Dermatomyositis↗
PubMed2005

Eye findings in patients with juvenile dermatomyositis.

OBJECTIVE: Reports of eye involvement in juvenile dermatomyositis (JDM), including significant retinopathy with visual loss, have led some to recommend routine formal ophthalmologic assessments for all patients with JDM at diagnosis. Our objective was to document the frequency and spectrum of eye involvement in patients followed in a single clinic caring for children with JDM. METHODS: A chart review was conducted of formal ophthalmologic consultation notes for patients with JDM followed at the Hospital for Sick Children between 1981 and 2002. RESULTS: Ophthalmologic assessments were found for 82 of 108 patients with JDM. The mean age at diagnosis of JDM was 7.0 years and 68.3% were female. Forty-five patients (55.6%) had abnormal eye examinations. Lid manifestations, found in 37 patients (45.7%), were the most common abnormality. Fourteen patients (17.1%) had corticosteroid-induced cataracts. Two patients had retinal abnormalities; one had a small retinal hemorrhage, the other an incidental chorioretinal scar. Neither had impairment of vision. No patient had uveitis. CONCLUSION: Eyelid and lens abnormalities are common in patients with JDM, while retinopathy is rare. As lid lesions and cataracts are easily detected by non-ophthalmologists, and retinal lesions are rare, we feel that JDM patients without visual symptoms do not require routine formal ophthalmologic assessment for disease manifestations.

Adolescent↗
PubMed2005

[Juvenile dermatomyositis in 12 years old girl].

We would like to present a selected case of 12-aged girl, with recognition of dermatomyositis (DM). At the age of 11 in the normally developing child, erythematous-oedematous changes have appeared on the face, particularly intensified in the vicinity of the orbital cavities (so called: pseudoglasses), as well as erythema and teleangiectasis on the dorsum of hands and small-sized diarthroidal joints (the Gottron's symptom). Subsequently, lower physical efficiency and distinctly weakness in the child's extremities occurred. In EMG (quadriceps muscle of the thigh) myogenous traits have been proven. Neurological examination revealed as follows: muscular weakness (adynamia), mainly lower limbs (grade 3 in the Lovett's scale, along with decreased loss of muscles tone), lack of the periosteal reflex near lower limbs, positive Gower's symptom and increased anterior spinal curvature. In the biochemical examinations accelerated erythrocyte sedimentation rate (ESR), and a rise of activity in muscles enzymes were stated. In the child's blood serum, we disclosed antinuclear antibodies ANA (type of granular luminescence, titre 160), to be rather evident to presence of autoimmunological process. During examinations of the musculocutaneus specimen, DM-markers have been detected. Capillaroscopy proved specific presence of numerous vessels, multiple capillary tubes, individual gemmated vessels and completely invisible dermatomyositous border. Patient was treated with per os sterid--Encorton at the initial dose of 2 mg/day, every other day during the lapse of 6 weeks to reach the normal CPK-activity, and consequently clinical picture under "on-line" surveillance, gradually reducing a specific medicine up to maintenance dose through 18 months. At present, the patient is subjected to check-up and monitoring by Neurological Outpatient and Rehabilitain Clinic for Children.

Antibodies, Antinuclear↗
PubMed2006

Rituximab as therapy for refractory polymyositis and dermatomyositis.

We describe response to rituximab treatment of refractory inflammatory myopathy. Three patients with long-standing polymyositis (PM) or dermatomyositis (DM) poorly responsive to prednisone combined with several immunosuppressants were given intravenous rituximab 1,000 mg on Days 0 and 14. Prior to rituximab, each had significant proximal weakness with creatine phosphokinase (CPK) elevation to>3 times the normal upper limit (range 789-3,123 U/l). Patients were receiving prednisone plus methotrexate (MTX) or azathioprine. CPK decrease was observed 1 month post-infusion, with normalization of levels averaging 4.6 months (range 2.6-7.7 mo). Muscle strength improved in all, with strength returning to normal in 2. Average daily prednisone dose decreased from 16.7 mg (range 10-20 mg) to 4 mg (range 0-7 mg) after infusion. MTX dose was tapered by 50% in 2 patients. The third patient eventually discontinued all additional therapies. Percentage of CD19+ cells in each were suppressed at 0-1% 5 to 6 months after infusion (normal 5-21%). Elevated CPK with return of clinical symptoms occurred in 2 patients 6 and 10 months post-infusion, requiring rituximab retreatment. CD19+ cells remained suppressed at 1% in one patient, but were almost normal at 4% in the other. The third patient remains disease-free 12 months after initial treatment, even though her CD19+ cells are now normal at 8%. Thus, short-term beneficial effects with rituximab were observed in patients with DM and PM. However, the need for retreatment did not correlate with levels of CD19+ cells.

Antibodies, Monoclonal↗
PubMedDate not supplied

Ulcerative paraneoplastic dermatomyositis secondary to metastatic breast cancer.

A 40-year-old Chinese-American woman with breast carcinoma metastatic to her lungs presented with a 3-month history of erosions on her inner thighs (Figure 1) and medial left shoulder. Faint livedo reticularis was evident on her legs as well. She had difficulty in walking and raising her shoulders. Her cutaneous examination was also notable for cuticular erythema (Figure 2) and mild periorbital erythema and edema. She had no systemic or rheumatologic complaints other than some difficulty in swallowing. Her blood chemistry values were notable for a creatinine kinase of 564 IU/L (5-200 IU/L), alanine aminotransferase 161 U/L (0-40 U/L) and aspartate aminotransferase 93 U/L (0-40 U/L), and an antinuclear antibody titer of 1:2560. Other blood chemistries and antibody serologies (anti-Jo-1, anti-Mi-2 and other anti-tRNA synthetase, anti-Ro/SSA, anti-U1RNP, anti-PM/Scl, and anti-Ku) were within normal limits. A biopsy specimen was obtained from an area of intact skin close to a right thigh ulceration that showed subtle vacuolar alteration at the dermo-epidermal junction with occasional necrotic keratinocyte (Figure 3). Melanophages and telangiectases were present. Within the subcutis there was fibrin deposition and neutrophils. A diagnosis of dermatomyositis was made. The patient received oral prednisone 20 mg three times a day, and her ulcerations resolved. Her creatinine kinase, alanine aminotransferase, and aspartate aminotransferase values returned to normal over the course of 3 weeks, but her antinuclear antibody was unchanged. Radiographic studies concurrently noted that her breast cancer had recurred in her lungs; plans were made to treat her with chemotherapy. The patient was lost to close follow-up, but it was learned that her erosions had reoccurred while her prednisone was tapered and resolved when her dosage of prednisone was increased.

Adult↗
PubMed2006

[A case of dermatomyositis associated with chronic idiopathic myelofibrosis].

A 74-year old woman had been suffering from chronic idiopathic myelofibrosis (CIMF) for three years before noticing skin rash and subsequent muscle weakness. On admission, purplish and erythematous skin rash was seen spreading over large parts of her body, including the face, chest, back and extremities. She could not stand up without assistance due to weakness. On laboratory examination, her hemoglobin was found to be 11.8g/dl, platelet 17,000/microl, WBC 22,500/microl (with blast cells), and CK 1,757 IU/I. Auto-antibodies including antinuclear antibody, Jo-I antibody and rheumatoid factors were negative. Abdominal CT revealed giant splenomegaly. She was diagnosed as having dermatomyositis (DM) associated with CIMF. Although administration of prednisolone followed by methyl-prednisolone pulse therapy ameliorated the weakness and skin rash, WBC increased to 35,000/ microl. In case of worsening of CIMF, azathioprine (AZP) administered. This decreased the WBC count to 13,700/microl and the CK to 49 IU/I 40 days after the administration. Patients with CIMF have an increased incidence of complications of other autoimmune diseases, indicating that the immunological mechanism plays some roles in the progression of the disease. There has been only one other reported case of DM complication. This case suggests usefulness of AZP treatment combined with prednisolone in these patients.

Aged↗
PubMed2006

Role of endothelial damage in the pathogenesis of interstitial pneumonitis in patients with polymyositis and dermatomyositis.

OBJECTIVE: Polymyositis and dermatomyositis (PM/DM) are often complicated by interstitial pneumonitis (IP), which is an important cause of death. It has been reported that blood concentration of transforming growth factor-beta (TGF-beta), which is produced by a wide range of cells including endothelial cells and enhances the fibrotic changes in various tissues, is increased in PM/DM with IP. Endothelial damage is likely to exist in PM/DM. We studied the relationship between endothelial damage and IP in PM/DM. METHODS: Blood levels of sialylated carbohydrate antigen KL-6, TGF-beta, endothelin-1 (ET-1), thrombomodulin (TM), and plasminogen activator inhibitor-1 (PAI-1) were determined in 43 patients with PM or DM with or without IP, and the relationship between these measures was analyzed. RESULTS: Blood levels of KL-6 and TGF-beta were higher in the patients with IP than those without, and these measures were well correlated with each other. Levels of ET-1, TM, and PAI-1, all known to reflect the extent of endothelial damage, were also increased in patients with IP, and these measures correlated well with TGF-beta. CONCLUSION: Our data suggest that endothelial damage might play an important role through the production of fibrosis-enhancing factors such as TGF-beta or ET-1 in PM/DM.

Adult↗