pulmonary fibrosis
Jump to navigation
Jump to search
Etiology
- idiopathic (most common)
- pharmacologic agents
- connective tissue disease
- sarcoidosis
- histiocytosis X
- occupational exposure
- inherited diseases
- post-infectious
- lymphangitis carcinomatosis
- radiation
Hamman-Rich syndrome: rapidly progressive pulmonary fibrosis
Clinical manifestations
- dyspnea
- dry cough
- clubbing (most common cause of clubbing)
- absence of orthopnea
- inspiratory rales (Velcro-like sound)
- tachypnea
- augmented P2 heart sound
Laboratory
- complete blood count: normocytic anemia
- erythrocyte sedimentation rate
- antinuclear antibody
- rheumatoid factor
- antibodies to:
- serum Ca+2 (sarcoidosis)
- angiotensin-converting enzyme (ACE) may be elevated in sarcoidosis
- urinalysis (sediment) Goodpasture's syndrome
Diagnostic procedures
- pulmonary function testing
- restrictive lung disease
- restriction combined with obstruction
- decreased diffusion capacity of CO
- bronchoscopy & bronchoalveolar lavage
- Pneumocystis
- fungus or tuberculosis
- alveolar proteinosis
- lymphangitis carcinomatosis
- histiocytosis X
- pulmonary hemorrhagic syndrome (Goodpasture's)
- exclude cancer
- open lung or thorascopic biopsy
- ambulatory pulse-oximetry exercise-induced hypoxemia
Radiology
- may be normal
- reticulonodular pattern (most common)
- upper lobe
- sarcoidosis
- histiocytosis X
- silicoisis
- lower lobe
- pleural effusion
- computed tomography (high resolution) of thorax
- obtain prior to biopsy to delineate distribution of disease & direct selection of site for biopsy
Complications
- respiratory failure (infectious) 39%
- heart failure 14%
- exposure to particulate matter <= 2.5 um in diameter is associated with worse baseline severity, disease progression, & mortality[3]
Differential diagnosis
- congestive heart failure (CHF) may masquarade as pulmonary fibrosis
- Goodpasteur's syndrome
- pneumocystis pneumonia
Management
- pirfenidone for idiopathic pulmonary fibrosis
- steroids: unclear if steroids alter course of disease
- prophylaxis against respiratory infection
- calcium channel blockers may be of benefit
- nitrates may be of benefit
- colchicine 0.5 mg PO BID may be of benefit
More general terms
More specific terms
- acute interstitial pneumonia; Hamman-Rich syndrome; idiopathic alveolar fibrosis; rapidly progressive pulmonary fibrosis (IAF)
- chronic pulmonary fibrosis
- idiopathic pulmonary fibrosis (IPF); usual interstitial pneumonitis (UIP)
- progressive massive fibrosis
Additional terms
References
- ↑ Saunders Manual of Medical Practice, Rakel (ed), WB Saunders, Philadelphia, 1996, pg 790
- ↑ Medical Knowledge Self Assessment Program (MKSAP) 11, 19 American College of Physicians, Philadelphia 1998, 2022.
- ↑ 3.0 3.1 Goobie GC, Carlsten C. Johannson KA et al Association of Particulate Matter Exposure With Lung Function and Mortality Among Patients With Fibrotic Interstitial Lung Disease. JAMA Intern Med. 2022;182(12):1248-1259. PMID: https://www.ncbi.nlm.nih.gov/pubmed/36251286 PMCID: PMC9577882 Free PMC article https://jamanetwork.com/journals/jamainternalmedicine/fullarticle/2797557
- ↑ National Heart, Lung, and Blood Institute (NHLBI) Idiopathic Pulmonary Fibrosis https://www.nhlbi.nih.gov/health-topics/idiopathic-pulmonary-fibrosis