A case of dermatomyositis presented as bronchiolitis obliterans organizing pneumonia has been rarely reported. We describe a 46-year-old female patient with dermatomyositis without elevation of creatine kinase presented as bronchiolitis obliterans organizing pneumonia. She was treated with prednisolone and azathioprine. Over a 2-year follow-up she has had no elevation of creatine kinase. The patient remains asymptomatic and has no medication for dermatomyositis and bronchiolitis obliterans organizing pneumonia two years after initial treatment. It has been suggested that the prognosis of dermatomyositis without creatine kinase elevation may be poor. Because the prognosis of bronchiolitis obliterans organizing pneumonia is generally believed to be good, we tentatively suggest that the normal value of creatine kinase in dermatomyositis does not always seem to herald a poor prognosis, an associated malignancy or severe interstitial lung disease.
Azathioprine/administration +ACY- dosage ; Biopsy, Needle ; Bronchiolitis Obliterans Organizing Pneumonia/pathology ; Bronchiolitis Obliterans Organizing Pneumonia/diagnosis+ACo- ; Case Report ; Creatine Kinase/blood+ACo- ; Dermatomyositis/pathology ; Dermatomyositis/enzymology ; Dermatomyositis/drug therapy ; Dermatomyositis/diagnosis+ACo- ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Human ; Middle Age ; Prednisone/administration +ACY- dosage ; Tomography, X-Ray Computed

We report a 46-year-old woman with primary biliary cirrhosis (PBC) presenting with Sjogren's syndrome and systemic mononuclear inflammatory vasculopathy. Biopsy specimens of sural nerve showed findings consistent with vasculitic neuropathy. Perivascular inflammatory mononuclear cell infiltration was observed on muscle biopsy specimen. The findings of abdominal computed tomography and brain magnetic resonance imaging were suggestive of vasculitis. Clinical manifestations and radiologic findings were improved after high dose prednisolone therapy.
Biopsy, Needle ; Case Report ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Human ; Liver Cirrhosis, Biliary/diagnosis+ACo- ; Liver Cirrhosis, Biliary/complications+ACo- ; Middle Age ; Prednisone/administration +ACY- dosage ; Sjogren's Syndrome/pathology ; Sjogren's Syndrome/diagnosis+ACo- ; Sjogren's Syndrome/complications ; Sural Nerve/pathology ; Treatment Outcome ; Vasculitis/pathology ; Vasculitis/drug therapy ; Vasculitis/diagnosis+ACo- ; Vasculitis/complications