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• W2000448559 abstract "Dear Editor, Juvenile dermatomyositis (JDMS) is a multisystem disease which is characterized by inflammation of the striated muscles and skin. As it is a systemic vasculitis, various other organs like joints, heart, gastrointestinal tract and lungs may also be involved. Spontaneous pneumomediastinum has been reported in several connective tissue disorders, like rheumatoid arthritis, systemic lupus erythematosus and systemic sclerosis.1 However, it is extremely uncommon in children with juvenile dermatomyositis. We report this complication in a young boy. To the best of our knowledge this is the youngest child to be reported as having developed this manifestation of juvenile dermatomyositis. A 9-year-old boy presented to us in October 2009 with chief complaints of prolonged pyrexia of 6 months duration and rash over the face. He had also noticed progressively increasing weakness of both upper and lower limbs and at presentation, was unable to squat or climb stairs. He measured 128 cm (50th centile) and weighed 16.5 kg (< 3rd centile). He had a respiratory rate of 20/min, heart rate 96/min and blood pressure 110/70 mmHg. A heliotrope rash was noted over the eyelids. He had typical Gottron’s papules and ulcers suggestive of vasculitic rash over the elbows (Fig. 1), fingertips and pinna. Respiratory examination and cardiovascular systems were unremarkable. Neurological examination revealed proximal muscle weakness (grade 3/5) in both upper and lower limbs and weakness of neck flexor muscles. Gag reflex was also weak. Elbow showing vasculitic ulcer. Investigations revealed hemoglobin of 106 gm/L, total leucocyte count 11 × 109/L, platelet count of 620 × 109/L, erythrocyte sedimentation rate of 34 mm/h (Westergreen). Alanine aminotransferase was 116 U/L (5–45) and aspartate aminotransferase was 65 U/L (5–45). Creatinine phosphokinase (CPK) was within normal limits (96 IU/L; 26–308) and lactate dehydrogenase was elevated (877 IU/L; 240–280). Magnetic resonance imaging (MRI) of thighs revealed bilateral inflammatory myositis. Antinuclear antibodies (ANA) and antiphospholipid antibodies (APLA) were negative. In view of muscle weakness, Gottron’s papules and classic heliotrope rash along with investigations showing elevated muscle enzymes and myositis on MRI, a diagnosis of juvenile dermatomyositis was made. The patient received five daily pulses of intravenous methylprednisolone (30 mg/kg) and was subsequently started on oral prednisolone (2 mg/kg per day) and subcutaneous methotrexate weekly (15 mg/m2). Muscle power improved to normal over the next 4 months. At this time, he presented to the follow-up clinic with the complaint of painless progressive swelling over neck and anterior chest wall. He did not have any respiratory complaints. Examination revealed subcutaneous emphysema. Chest and neck X-ray showed pneumomediastinum. On strong suspicion of underlying interstitial lung disease Computed tomography (CT) chest scan was performed which showed pneumomediastinum with interstitial lung disease (Fig. 2). This complication was managed conservatively and subcutaneous emphysema resolved completely in 2 weeks. The child remained asymptomatic for the next 2 months; however, he again developed subcutaneous emphysema. Again he did not have any respiratory symptoms. This was managed conservatively and it resolved in a week. At a follow-up after 4 months, the child is completely asymptomatic and has been continued on tapering doses of steroids and weekly subcutaneous methotrexate. CT scan showing interstitial lung disease and pneumomediastinum. JDMS is a systemic autoimmune vasculopathy, characterized by cutaneous rash and proximal myopathy. Pulmonary complications associated with this disease include interstitial pneumonitis, diffuse alveolar damage and bronchiolitis obliterans with organizing pneumonia. Although the exact incidence of interstitial lung disease in polymyositis (PM)/dermatomyositis (DM) is not known, it has been reported that 10–40% of these patients have this complication.1, 2 Spontaneous pneumomediastinum has been reported as a rare complication in DM. Most of these patients are adults (age range 10–65 years)3, 4 and our patient is perhaps the youngest ever reported to have developed this complication. Risk factors for developing pneumomediastinum in a patient with DM include interstial lung disease, presence of cutaneous vasculopathy, mild or absence of CPK elevation and steroid use.4-6 The prevalence of vasculopathy is significantly higher in patients with pneumomediastinum than in those without it.5 This suggests that it may be an important factor leading to disruption of the bronchial mucosal barrier and causing development of pneumomediastinum in DM. Our patient also had cutaneous ulceration suggesting vasculitis so it can be assumed that vasculitis played a major role in the development of pneumomediastinum. He also had other reported risk factors like normal CPK levels, steroid use and underlying interstitial lung disease. Kono et al.5 suggested that it is not necessary for all patients with pneumomediastinum to have underlying interstitial lung disease (ILD). Even in our case, the clinical assessment at the time of first admission did not suggest the presence of an ILD. In view of this a CT scan was not considered necessary at that time. However, on follow-up while his muscle power improved, he developed this subcutaneous emphysema which prompted us to do a detailed evaluation for ILD. It has been suggested that onset of spontaneous pneumothorax in dermatomyositis must alert the clinician to the possibility of vasculitic activity and that immunosuppressive therapy may need to be hiked.7 Addition of cyclopsporin or cyclophosphamide7, 8 has been suggested in such cases but there is no consensus on any particular regimen. In our case, the child has remained stable on prednisolone and methotrexate and we plan to continue this regimen. Pneumomediastinum is an uncommon complication of juvenile dermatomyositis, which may be secondary to an underlying interstitial lung disease. Most patients respond well to continued immunosupression." @default.
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• W2000448559 date "2011-06-29" @default.
• W2000448559 modified "2023-09-26" @default.
• W2000448559 title "Spontaneous pneumomediastinum: a rare complication of juvenile dermatomyositis" @default.
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