Presence of an interstitial pneumonia (by HRCT or surgical lung biopsy) and, Exclusion of alternative aetiologies and, Does not meet criteria of a defined connective tissue disease and, At least one feature from at least two of these domains: A. Clinical domain B. Serologic domain C. Morphologic domain
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A. Clinical domain Distal digital fissuring (i.e. “mechanic hands”) Distal digital tip ulceration Inflammatory arthritis or polyarticular morning joint stiffness ≥60 min Palmar telangiectasia Raynaud's phenomenon Unexplained digital oedema Unexplained fixed rash on the digital extensor surfaces (Gottron's sign)
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B. Serologic domain ANA ≥1:320 titre, diffuse, speckled, homogeneous patterns or Rheumatoid factor ≥2× upper limit of normal Anti-CCP Anti-dsDNA Anti-Ro (SS-A) Anti-La (SS-B) Anti-ribonucleoprotein Anti-Smith Anti-topoisomerase (Scl-70) Anti-tRNA synthetase (e.g. Jo-1, PL-7, PL-12; others are: EJ, OJ, KS, Zo, tRS) Anti-PM-Scl Anti-MDA-5
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C. Morphologic domain Suggestive radiology patterns by HRCT (see text for descriptions): a. NSIP b. OP c. NSIP with OP overlap d. LIP
Histopathology patterns or features by surgical lung biopsy: Multi-compartment involvement (in addition to interstitial pneumonia): a. Unexplained pleural effusion or thickening b. Unexplained pericardial effusion or thickening c. Unexplained intrinsic airways disease# (by PFT, imaging or pathology) d. Unexplained pulmonary vasculopathy
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