Pulmonary Disease

Vasculitides (Small and medium vessel: ANCA associated)

Differential diagnosis of Pulmonary-Renal Syndrome
  • ANCA-associated vasculitides (AAV)
    • Granulomatosis with polyangiitis (GPA) - pulmonary nodules and infiltrates
    • Eosinophilic granulomatosis with polyangiitis (EGPA) - less often renal involvement; pulmonary: asthma or eosinophilic pneumonia
    • Microscopic polyangiitis (MPA) - capillaritis or hemorrhage
  • Polyarteritis nodosa (PAN)
    • 25% have skin manifestation: livedo, retiform purpura, ulcers, subcutaneous nodules, digital infracts
    • Strictly medium vessle vasculitis
    • Renal vascular hypertension
  • Goodpasteur syndrome
  • Systemic lupus erythematosus (SLE)
    • dsDNA, antiSmith
  • Rheumatoid vasculitis
    • Rheumatoid factor
 

Granulomatosis with Polyangiitis (GPA)

  • Granulomatous inflammation of the respiratory tract (nasal sinus, tracheal and ear involvement)
    • Recurrent epistaxis, mucosal ulcerations, nasal septal perforations, saddle nose deformity
    • Dyspnea, couph, hemotyses, pleuritis
    • Chest X ray - irregular infiltrates or nodules
  • Systemic necrotizing small and medium-sized vessel vasculitis
  • Pauci-immune glomerulonephritis → neutrophils directly mediate vessel damage without complement
  • cANCA/PR3 (+) 80-90%
  • Treat with steroids and cyclophosphamide

Eosinophilic Granulomatosis with Polyangiitis (EGPA)

  • Necrotizing granulomatous vasculitis
  • Cutaneous lesions
    • Palpable purpura
    • Subcutaneous nodules (scalp or extremities)
    • Urticaria
    • Livedo reticularis
    • Retiform purpura
    • Papulonecrotic lesions
  • Renal involvement less common than GPA
  • Associated with asthma and eosinophilia
  • pANCA/MPO in 40%
  • Most common cause of death is cardiac (not pulmonary)

Systemic sclerosis

  • Pulmonary disease is the most common cause of death
  • Pulmonary findings
    • Bibasilar pulmonary fibrosis
    • Pulmonary arterial hypertension (more common in limited systemic sclerosis or CREST)
  • Cutaneous findings
    • Salt and pepper leukoderma
    • Pitted scars on digital pulps

Dermatomyositis

  • Pulmonary disease present in 15-30%
  • Diffuse interstitial fibrosis
  • Two types with lung involvement: anti-synthetase and MDA5
  • Anti-synthetase: Jo-1, PL-7, PL-12 → mechanic’s hands
  • Clinically amyopathic: MDA5 → tender palmar papules, ulcers on elbows/knees, vasculopathic lesions, diffuse alopecia
  • Lung disease and malignancy tend to be mutually exclusive

Sarcoidosis

Hands and the lungs

  • Clubbing of the digits
    • Yellow nail syndrome (usually also have peripheral edema)
      • Chronic bronchitis
      • Bronchiectasis
      • Sinusitis
      • Pleural effusions