Lecture 14: DILD Flashcards
1
Q
Pulmonary function tests: DILD
A
- symmetric reduction in static lung volumes
- decrease in diffusion capacity (DLCO)
- normal FEV1/FVC
Known Etiologies
- Inorganic – pneumoconioses, asbestosis
- Organic - hypersensitivity pneumonitis: bird, farmer’s, humidifier
- drugs: nitrofurantoin, chemo, amiodarone
Unknown Etiologies
- Idiopathic Interstitial Pneumonia (IIP): most common form of IIP is IDIOPATHIC PULMONARY FIBROSIS (IPF)
- IIP with Collagen Vascular Disorders: dermatomyositis, MCTD, scleroderma, SLE, etc.
- Sarcoidosis
- Miscellaneous: eosinophilic granuloma, alveolar proteinosis, etc.
2
Q
IPF Clinical Presentation
A
History
- 65 year old male presents with gradually progressive dyspnea and dry cough
- No travel history or relevant exposures
Physical
- Clubbing
- bibasilar inspiratory crackles; sounds like “velcro”
- by definition, other etiologies of DILD have been ruled out!
Prognosis: average survival 4-5 years but quite variable
- Complications
- Respiratory failure
- Pulmonary hypertension
- Cor pulmonale
Treatment
- corticosteroids of no benefit in classic IPF/UIP
- Pirfenidone
- Transplantation
3
Q
Sarcoidosis: Clinical
A
Presentation
- Constitutional complaints very common
- Thorax involved in 90+% of cases but chest exam often normal !
- Possible associated splenomegaly & peripheral lymphadenopathy
- Can present to any sub-specialist !
- Patho description: non-caseating granulomatous disease
Pulmonary
- Lofgren’s Syndrome: erythema nodosum with bilateral hilar lymphadenopathy (BHL)
- progressive dyspnea - BHL + parenchymal infiltrates
- chronic - upper lobe pulmonary fibrosis
- Extrapulmonary
Extrapulmonary
- Skin - erythema nodosum, lupus pernio • Eye - uveitis
- Hypercalcemia
- Liver - granulomatous hepatitis
- Joints – peripheral pauci-articular arthritis
- Cardiac - esp. arrhythmias, heart block
- CNS - cranial n., brainstem infiltrates
Labs
- Common: elevation of liver enzymes, hypergammaglobulinemia, elevated serum angiotensin-converting enzyme (ACE)
- Uncommon: hypercalciuria, hypercalcemia
