2.3.16 – VA Ambulatory Report Pearls – ILD + Connective Tissue Disorders

Distinguishing Clinical Features of Idiopathic Pulmonary Fibrosis/Usual Interstitial Pneumonia (IPF/UIP) versus Non-Specific Interstital Pneumonia (NSIP)

Adapted from Richard du Bois and Talmadge E King, Jr’s classic paper in Thorax in 2007: Challenges in pulmonary fibrosis: The NSIP/UIP debate

Duration of illness Chronic (> 12 months) Subacute to chronic (months to years)
Chest radiograph Bilateral reticular opacities in lower zones; volume loss Bilateral hazy and reticular opacity
HRCT Peripheral, basal predominance, with extension into the subpleural space Peripheral, basal predominance, symmetrical, and usually spares the subpleural space
Honeycombing No honeycombing
Typically reticular opacities throughout with focal ground-glass opacities Ground-glass opacities
Treatment Poor response to any treatment Corticosteroid responsiveness
Prognosis 50-70% mortality in 5 years Unclear; <15% mortality in 5 years

ILD and Rheumatologic Disorders

We briefly discussed some of the rheumatologic disorders that can have lung and peripheral joint involvement. ILD can complicate the course of most of the connective tissue diseases. Here are the five that we discussed and some associated pulmonary findings. Estimates of frequency of pulmonary involvement in the associated diseases appear in the parentheses.

  • Rheumatoid arthritis (20-30%)
    • Can present with UIP or NSIP
  • Systemic lupus erythematosus (2-8%)
    • Pleural thickening
    • Pleural/pericardial effusions
  • Systemic sclerosis/scleroderma (45%)
    • In ILD, typically associated with NSIP > UIP
    • May see esophageal dilatation on CT
    • Usually associated with pulmonary HTN
  • Sjogren’s syndrome (up to 25%)
    • In ILD, typically associated with NSIP >> UIP
    • Lower-lobe predominant GGOs, which can be nonspecific
    • Thin walled cysts may also be present which would be concerning for lymphocytic interstitial pneumonia
  • Polymyositis/dermatomyositis (20-50%)
    • GGOs are predominant finding
    • Can be associated with cryptogenic organizing pneumonia and diffuse alveolar damage
    • Honeycombing is rare to see

A Word on Anti-Synthetase Syndrome

  • Rare, chronic autoimmune disease
  • Epidemiology: adults (40-50s); men = women
  • Subgroup of the idiopathic inflammatory muscle diseases – the most common of these include polymyositis and dermatomysitis and anti-synthetase syndrome is a separate entity within this group
  • The hallmark is the presence of serum autoantibodies (e.g., Anti-Jo-1)
  • Clinical features: myositis (>90%), ILD (60%), arthropathy (50%), and Raynaud’s phenomenon (40%)
  • Treatment: prednisone + steroid-sparing agents such as azathioprine and methotrexate

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