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Significance of connective tissue diseases features in pulmonary fibrosis

Vincent Cottin
European Respiratory Review 2013 22: 273-280; DOI: 10.1183/09059180.00003013
Vincent Cottin
1Hospices Civils de Lyon, Hôpital Louis Pradel, Service de pneumologie – Centre de référence national des maladies pulmonaires rares et Centre de compétences de l'hypertension artérielle pulmonaire, Lyon, and 2Université de Lyon, Université Claude Bernard Lyon 1, INRA, UMR754 INRA-Vetagrosup EPHE IFR 128, Lyon, France.
1Hospices Civils de Lyon, Hôpital Louis Pradel, Service de pneumologie – Centre de référence national des maladies pulmonaires rares et Centre de compétences de l'hypertension artérielle pulmonaire, Lyon, and 2Université de Lyon, Université Claude Bernard Lyon 1, INRA, UMR754 INRA-Vetagrosup EPHE IFR 128, Lyon, France.
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  • For correspondence: vincent.cottin@chu-lyon.fr
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    Figure 1.

    High-resolution computed tomography scan of a 59-year-old female patient with undifferentiated connective tissue disease, showing patterns of nonspecific interstitial pneumonia (with ground-glass opacification and traction bronchiectasis) and oesophageal dilation. Features of systemic sclerosis were present (Raynaud's phenomenon, megacapillaries at nailfold capillaroscopy, oesophageal hypomotility, anti-topoisomerase-1 antibodies), but no sclerodactily or telangiectasia was visible at 5 years follow-up.

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  • Table 1. Key symptoms to detect underlying connective tissue disease
    OrganMain manifestations to be evaluated#
    Peripheral circulationRaynaud’s phenomenon
    SkinSclerodactily
    Digital ulcerations or scars
    Telangiectasia
    Violaceous erythematous rash over the interphalangeal joints, knuckles, elbows and knees (Gottron's sign)
    Lilaceous rash of the eyelids
    Rash of the neck and upper chest and shoulders (heliotrope rash, e.g. photosensitivity)
    Mechanics’ hand
    JointsJoint pain or swelling (arthritis, arthralgia)
    Morning stiffness
    MuscleMuscle pain and muscle weakness
    Mouth and eyesDry mouth and dry eyes (sicca syndrome)
    • #: this list is not exhaustive.

  • Table 2. Indicative criteria for which patients may be referred for rheumatologic evaluation
    Features suggesting CTD
    DemographicsFemale sex
    Age <50 years
    Clinical manifestationsAny patient with extra-thoracic manifestations highly suggestive of CTD:
        Raynaud’s phenomenon, oesophageal hypomotility, inflammatory arthritis of the metacarpophalangeal joints or wrists, digital oedema, symptomatic keratoconjonctivitis sicca
    Lung histopathologyAll cases of NSIP, LIP or ILD patterns with histopathology features that might suggest CTD:
        Extensive pleuritis, dense perivascular collagen, lymphoid aggregates with germinal centre formation, prominent plasmacytic infiltration
    BiologyPatients with positive high-titre antinuclear antibodies (>1:320) or rheumatoid factor (>60 IU·mL−1), nucleolar staining antinuclear antibodies or specific antibodies (anti-cyclic citrullinated peptide, anti-Scl70, anti-Ro, anti-La, anti-dsDNA, anti-Smith, anti-RNP, anti-tRNA synthetase)
    • CTD: connective tissue disease; NSIP: nonspecific interstitial pneumonia; LIP: lymphoid interstitial pneumonia; ILD: interstitial lung disease; RNP: ribonucleoprotein. Adapted from [37].

  • Table 3. Diagnostic criteria for undifferentiated connective tissue disease (UCTD)
    UCTD diagnostic criteria#Broader definition of UCTD#,¶
    A. Symptoms associated with CTDAt least one of: 1) Raynaud’s phenomenon; 2) arthralgias/multiple joint swelling; 3) morning stiffness; 4) dry mouth or dry eyes (Sicca features); 5) proximal muscle weaknessAt least one of: 1) Raynaud’s phenomenon; 2) arthralgias/multiple joint swelling; 3) photosensitivity; 4) unintentional weight loss; 5) morning stiffness; 6) dry mouth or dry eyes (Sicca features); 7) dysphagia; 8) recurrent unexplained fever; 9) gastro-oesophageal reflux; 10) skin changes (rash); 11) oral ulceration; 12) nonandrogenic alopecia; 13) proximal muscle weakness
    B. Positive autoimmune serologyPositive finding of at least one of: 1) ANA (high titre); 2) RF (high titre); 3) positive ENA; 4) anti-Scl70 antibody; 5) anti-RNP antibody; 6) anticentromere antibody; 7) SS-A or SS-B; 8) Jo-1 antibodyPositive finding of at least one of: 1) ANA; 2) RF; 3) anti-Scl70 antibody; 4) SS-A or SS-B; 5) Jo-1 antibody; 6) ESR (>2 times normal), CRP
    • CTD: connective tissue disease; ANA: antinuclear antibody; RF: rheumatoid factor; ENA: extractable nuclear antigen; RNP: ribonucleoprotein; SS-A: anti-Ro; SS-B: anti-La; ESR: erythrocyte sedimentation rate; CRP: C-reactive protein. #: a diagnosis of UCTD requires having one or more of the symptoms outlined in A, and one or more of the laboratory findings listed in B; ¶: criteria proposed by Kinder et al. [5]. Reproduced from [6] with permission.

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Significance of connective tissue diseases features in pulmonary fibrosis
Vincent Cottin
European Respiratory Review Sep 2013, 22 (129) 273-280; DOI: 10.1183/09059180.00003013

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Significance of connective tissue diseases features in pulmonary fibrosis
Vincent Cottin
European Respiratory Review Sep 2013, 22 (129) 273-280; DOI: 10.1183/09059180.00003013
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  • Article
    • Abstract
    • Introduction
    • When should CTD be suspected?
    • Does the diagnosis of established CTD affect prognosis or management?
    • CTD features may be missed in the diagnostic approach of ILD
    • Are auto-antibody and/or limited extra-thoracic manifestations significant?
    • Conclusions
    • Footnotes
    • References
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  • Interstitial and orphan lung disease
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