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. Author manuscript; available in PMC: 2018 Jul 1.
Published in final edited form as: Arch Pathol Lab Med. 2017 May 3;141(7):960–969. doi: 10.5858/arpa.2016-0427-OA

Table 1.

Criteria for Interstitial Pneumonia With Autoimmune Features (IPAF)a,b

A. Clinical Domain B. Serologic Domain C. Morphologic Domain

  • Raynaud phenomenon

  • Palmar telangiectasia

  • Distal digital fissuring (ie, “mechanics hands”)

  • Distal digital tip ulceration

  • Inflammatory arthritis or polyarticular morning joint stiffness >60 min

  • Unexplained digital edema

  • Unexplained fixed rash on the digital extensor surfaces (Gottron sign)

  • ANA titer >1:320, diffuse, speckled, homogeneous patterns

    or

    ANA nucleolar pattern (any titer)

    or

    ANA centromere pattern (any titer)

  • RF >2 × 3 ULN

  • Anti-CCP

  • Anti-dsDNA

  • Anti-Ro antibodies (SS-A)

  • Anti-La antibodies (SS-B)

  • Antiribonucleoprotein

  • Anti-Smith antigen

  • Antitopoisomerase (Scl-70)

  • Anti-tRNA synthetase (eg, Jo-1, PL-7, PL-12 [others are EJ, OJ, KS, Zo, tRS])

  • Anti-PM-Scl

  • Anti-CADM (MDA-5)

 Radiology features

  • Suggested NSIP pattern

  • Suggested OP pattern

  • Suggested mixed NSIP/OP pattern

  • Suggested LIP pattern
Histopathology features (SLB)

  • NSIP

  • OP

  • NSIP with OP overlap

  • LIP

  • Interstitial lymphoid aggregates with GCs

  • Diffuse lymphoplasmacytic infiltration (±lymphoid follicles)

Multicompartment involvement

  • Unexplained pleural effusion or thickening

  • Unexplained pericardial effusion or thickening

  • Unexplained intrinsic airways disease (by PFT, HRCT, or pathology)

  • Unexplained pulmonary vasculopathy

Abbreviations: ANA, antinuclear antibody; CADM, clinically amyopathic dermatomyositis; CCP, cyclic citrullinated peptide; CTD, connective tissue disease; dsDNA, double stranded deoxyribonucleic acid; GC, germinal center; HRCT, high-resolution computed tomography; LIP, lymphoid interstitial pneumonia; MDA, melanoma differentiation-associated; NSIP, nonspecific interstitial pneumonia; OP, organizing pneumonia; PFT, pulmonary function tests; PM-Scl, polymyositis/systemic scleroderma; RF, rheumatoid factor; SLB, surgical lung biopsy; SS-A, Sjögren syndrome–related antigen A; SS-B, Sjogren syndrome–related antigen B; tRNA, transfer RNA; ULN, upper limit of normal.

a

The criteria for IPAF are (1) the presence of an interstitial pneumonia (by HRCT or surgical lung biopsy) and (2) the exclusion of alternative etiologies and (3) incomplete features of a defined CTD and (4) at least one feature from at least 2 domains (columns A, B, or C).

b

This material has not been reviewed by European Respiratory Society before release; therefore, the European Respiratory Society is not responsible for any errors, omissions, or inaccuracies, or for any consequences arising therefrom, in the content. Reproduced with permission of the European Respiratory Society: ©European Respiratory Journal. 2015;46(4):976–987.