Genetically transmitted respiratory disease
characterized by rapidly progressive dyspnea and cyanosis, digital clubbing,
polycythemia, pulmonary hypertension, and diffuse pulmonary fibrosis (fatal
within months of onset).
Corrigan Cirrhosis; Cryptogenetic Fibrosing Alveolitis;
Familial Interstitial Lung Disease; Hamman Rich Disease; Idiopathic
Pulmonary Fibrosis; Liebow Pneumonia; Usual Interstitial Pneumonia of
First described by Sir Dominic John Corrigan in 1838.
Respiratory disorder characterized by chronic nonproductive cough and
dyspnea with changes in the diffusion capacity of the lung. The disease
evolves toward respiratory failure and death.
Prevalence: 1.34:1,000,000 population in the United
Kingdom. Occurs more frequently in males than in females.
Could result from an inherited anomaly in the
immune system, but it is yet to be confirmed.
Based on clinical, radiologic, and respiratory function
tests and lung biopsy if necessary. Radiologic changes show a nodular
ground-glass appearance, predominantly in the bases. Respiratory function
tests indicate a diffusion defect that is associated with a decrease in
arterial oxygen saturation made worse by exercise. Usually, there is no
restrictive pulmonary disorder until very late in the disease. Biopsy
demonstrates the presence of interstitial fibrosis and cysts.
Affected patients present with a nonproductive
cough that is associated with dyspnea, chest pain, and cyanosis. The disease
evolves over many years toward hypoxemia, respiratory failure, and right
heart failure. It can be exacerbated by spontaneous pneumothorax and
recurrent infections. Clubbing and pulmonary osteoarthropathy are usually
present. Patients become very disabled in the third or fourth decade, and
death usually occurs in their 50s or earlier if they are victims of
Before going to the operating room,
the patient should be evaluated by a pneumologist for respiratory function
testing, arterial blood gas, recurrent infections, and exacerbation of
symptoms. Also obtain a chest radiograph and an ECG. If right heart failure
is suspected, an echocardiogram is recommended. An anesthesiology
consultation is highly recommended for elective surgery.
Because patients are “respiratory
cripples,” tracheal intubation should be avoided as much as possible to
prevent serious postoperative complications, including prolonged mechanical
ventilation. Regional anesthesia may be preferable.
Be careful when using opioids alone
or in combination with benzodiazepines because patients may be sensitive to the respiratory depressant effect of the medication.
Green FH: Overview of pulmonary fibrosis. Chest 122(6 suppl):334S, 2002.
Verleden GM, du Bois RM, Bouros D, et al: Genetic predisposition and
pathogenetic mechanisms of interstitial lung diseases of unknown origin.
Eur Respir J Suppl 32:17s, 2001.
Whitsett JA: Genetic basis of familial interstitial lung disease: Misfolding
or function of surfactant protein C? Am J Respir Crit Care Med