Interstitial lung disease as first clinical manifestation within the antisynthetase syndrome--dermatomyositis.
Diffuse interstitial pneumopathies within the diseases of the connective tissue are often a diagnosis challenge, sometimes being the initial or dominant manifestation of the underlying autoimmune disease. The case of a young man shall be presented with relatively quick dyspnoea which has appeared under the conditions of an efficient physical status and without any notable pathological history. The cause of the dyspnoea is found to be a interstitial lung disease with radiological and histopathological pattern of non-specific interstitial pneumonia. The disease screening system discovers Ac anti Ro-52 which commits research in the direction of an autoimmune disease, confirmed by highlighting Ac anti PL-7 as being an antisynthetase syndrome. Subgroup of idiopathic inflammatory myopathies, the antisynthetase syndrome is a rare chronic autoimmune disease, which is characterised by the presence of antibodies directed against aminoacyl-t-RNA-synthetases (family of intracytoplasmic enzymes with a vital role in the protein synthesis). Shortly after specifying the relevant immunological status, the consistent clinical expression with dermatomyositis is also configured. The treatment is commenced with systemic corticoid and azathioprine; despite the treatment we are witnessing a slowly progressive clinical and functional deterioration.
Daniel
D
Trăilă
Ovidiu Fira
OF
Mlădinescu
Cristian
C
Oancea
Voicu
V
Tudorache
D000328:Adult; D000974:Antibodies, Antinuclear / Q000097:blood; D001323:Autoantibodies / Q000097:blood*; D001379:Azathioprine / Q000627:therapeutic use; D015415:Biomarkers / Q000097:blood; D003882:Dermatomyositis / Q000097:blood / Q000150:complications*; D018450:Disease Progression; D004359:Drug Therapy, Combination; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007155:Immunologic Factors / Q000097:blood*; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D017563:Lung Diseases, Interstitial / Q000097:blood / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology / Q000276:immunology*; D008297:Male; D009220:Myositis / Q000097:blood / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology / Q000276:immunology*; D014797:Vital Capacity
D000974:Antibodies, Antinuclear; D001323:Autoantibodies; D015415:Biomarkers; D005938:Glucocorticoids; D007155:Immunologic Factors; D007166:Immunosuppressive Agents; D001379:Azathioprine