Diffuse Parenchymal Lunz Disease - DPLD (ILD)

Interstitial lung diseases (ILD) are uncommon, inflammatory and fibrotic processes. Affect interstitium but often also alveoli and airways. Goal is to make early dx and identify those d/o which are treatable.

Etiology

  • DPLD can be broken into the following categories (% of all DPLD)
  • Idiopathic Pulmonary Fibrosis (20%)
  • Chronic hypersensitivity pneumonitis - mold, bird exposure, mycobacteria, other (20%)
  • Connective tissue disease related - rheumatoid arthritis, systemic sclerosis (scleroderma), Sjogrens, polymyositis/dermatomyositis (20%)
  • Sacroidosis (20%)
  • Pneumoconioses - asbestosis, silicosis, berylliosis (10%)
  • Other interstitial lung diseases - meds, NSIP, acute or chronic eosinophilic pneumonias (CEP), cryptic organizing pneumonia (COP), acute interstitial pneumonia (AIP), XRT, other idiopathic (10%)

Risk Factors

  • Exposures (meds, environmental, radiation) or underlying diseases (e.g. sarcoid, CTD, other) as above
  • For some connective tissue disorders (CTDs), lung findings and symptoms of DPLD will pre-date the diagnosis of CTD

Symptoms

  • Typically sub-acute, progressive SOB, DOE, chronic cough; can take months (or longer) prior to establishing dx
  • AIP, COP, AEP tend to present acutely
  • For some connective tissue disorders (CTDs), lung findings and symptoms will pre-date the diagnosis of CTD
  • Symptoms suggestive CTD: Reynauds, muscle weakness, joint pain, dry eyes, dry mouth, GERD

Physical Exam Findings

  • Raynauds
  • May have hypoxemia
  • Assess for clubbing, cyanosis
  • Lung exam w/dry sounding crackles
  • Joint abnormalities or inflammation
  • Skin findings: e nodosum w/sarcoid, heliotropic rash w/dermatomyositis, scaly papules on knickers (Grotton's papule); sub-cu calcifications (dermatomyositis), thickened skin (sclerodactyly associated w/scleroderma)
  • Elevated JVP, prominent P2, peripheral edema w/pulmonary hypertension
  • Lymphadenopathy, hepatomegaly (sarcoid)

Sub-Diseases

Tests

Links