methyl-prednisolonate and Lung-Diseases--Interstitial

A 36-year-old man was admitted to a hospital with complaints of fever, polyarthralgia and dyspnea. Erythema was observed on his face, extensor surface of the fingers and extremities, and a chest X-ray revealed massive bilateral pleural effusion. He had no sign of myopathy at this point. Pleural fluid was proved to be exudative and contained extremely high levels of hyaluronic acid. He was also complicated with interstitial pneumonitis and was given a pulse therapy with methyl prednisolone followed by daily administration of 55 mg prednisolone (PSL). Twenty days after the commencement of the therapy, pleural effusion decreased but muscle weakness gradually appeared, accompanied by elevation of myogenic enzymes. Myogenic changes on electromyogram, and irregularity of the muscle fibers with slight inflammatory cell infiltrates in a biopsy specimen were demonstrated. He was transferred to our hospital, and a diagnosis of dermatomyositis was made. Later, pleural effusion waxed and waned depending on the dosage of PSL, but no other causative disorder was demonstrated by extensive examinations. This case indicates that the pleuritis could be one of the vasculitic manifestations of dermatomyositis.

Topics: Adult; Biomarkers; Dermatomyositis; Diagnosis, Differential; Drug Therapy, Combination; Humans; Hyaluronic Acid; Lung Diseases, Interstitial; Male; Muscular Diseases; Pleural Effusion; Prednisolone; Pulse Therapy, Drug

Antisynthetase syndrome is a relatively uncommon entity, and can be easily missed if not specifically looked for in adults whose initial presentation is with interstitial lung disease. Its presentation with interstitial lung disease alters its prognosis.. This case report describes a 27-year-old Pakistani, Asian man, a medical student, with no previous comorbidities or significant family history who presented with a 3 months' history of low grade fever and lower respiratory tract infections, associated with exertional dyspnea, arthralgias, and gradual weight loss. During these 3 months, he had received multiple orally administered antibiotics for suspected community-acquired pneumonia. When he presented to us, he was pale and febrile. A chest examination was significant for bi-basal end-inspiratory crackles. Preliminary investigations revealed raised erythrocyte sedimentation rate. High resolution computed tomography of his chest showed fine ground-glass attenuation in posterior basal segments of both lower lobes suggestive of interstitial lung disease. He was started on dexamethasone, to which he responded and showed improvement. However, during the course of events, he developed progressive proximal muscle weakness. Further investigations revealed raised creatinine phosphokinase and lactate dehydrogenase. A thorough autoimmune profile was carried out which showed positive anti-Jo-1 antibodies in high titers. A muscle biopsy was consistent with inflammatory myopathy. Clinical, radiological, serological, and histopathological markers aided in making the definitive diagnosis of antisynthetase syndrome. Antisynthetase syndrome is a variant of polymyositis but with visceral involvement, that is, interstitial lung disease and positive anti-Jo-1 antibodies. Our patient responded very well to glucocorticoids and azathioprine.. Antisynthetase syndrome is a rare clinical entity which apart from clinical presentation requires specific serological investigations for diagnosis. Concomitant association of interstitial lung disease gives it a guarded prognosis.

Topics: Adult; Antibodies, Antinuclear; Autoantibodies; Azathioprine; Creatine Kinase; Diagnosis, Differential; Dyspnea; Humans; Lung Diseases, Interstitial; Male; Muscle Weakness; Myositis; Prednisolone