- Presence of an interstitial pneumonia (by HRCT or surgical lung biopsy) and,
- Exclusion of alternative etiologies and,
- Does not meet criteria of a defined connective tissue disease and,
- At least one feature from at least two of these domains:
- Clinical domain
- Serologic domain
- Morphologic domain
|
| A. Clinical domain |
- Distal digital fissuring (ie, "mechanic hands")
- Distal digital tip ulceration
- Inflammatory arthritis or polyarticular morning joint stiffness ≥60 minutes
- Palmar telangiectasia
- Raynaud phenomenon
- Unexplained digital edema
- Unexplained fixed rash on the digital extensor surfaces (Gottron's sign)
|
| B. Serologic domain |
- ANA ≥1:320 titer, diffuse, speckled, homogeneous patterns or
- ANA nucleolar pattern (any titer) or
- ANA centromere pattern (any titer)
- 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 (eg, Jo-1, PL-7, PL-12; others are: EJ, OJ, KS, Zo, tRS)
- Anti-PM-Scl
- Anti-MDA-5
|
| C. Morphologic domain |
- Suggestive radiology patterns by HRCT (please refer to UpToDate topic text for additional details):
- NSIP
- OP
- NSIP with OP overlap
- LIP
- Histopathology patterns or features by surgical lung biopsy:
- NSIP
- OP
- NSIP with OP overlap
- LIP
- Interstitial lymphoid aggregates with germinal centers
- Diffuse lymphoplasmacytic infiltration (with or without lymphoid follicles)
- Multi-compartment involvement (in addition to interstitial pneumonia):
- Unexplained pleural effusion or thickening
- Unexplained pericardial effusion or thickening
- Unexplained intrinsic airways disease* (by PFT, imaging or pathology)
- Unexplained pulmonary vasculopathy
|
