Sarcoidosis is a systemic disease of unknown origin. It causes widespread non-caseating granulomas that may affect not only the lung but also the myocardium, eyes, skin, liver, spleen, lymph nodes, bones, joints, nervous system, endocrine system and digestive tract. The peak incidence is in patients between the ages of 20-40 years and up to 50% of patients are asymptomatic. The overall mortality rate varies between 5 and 10%.
The symptoms usually consist of cough that may be paroxysmal in the acute phase and may cause incapacitation. The non-respiratory symptoms may consist of anorexia, malaise, lassitude, joint pains (arthralgia) and the almost pathognomonic occurrence erythema nodosum.
The presence of Lofgrens Syndrome with erythema nodosum (nodular skin lesions), bilateral hilar lymphadenopathy, and a low-grade fever, imply a favourable prognosis.
Lung function is often normal despite some chest X-ray changes. Of those presenting with interstitial pulmonary disease alone, only one quarter show complete resolution. The remainder may progress to pulmonary fibrosis with impaired lung function, and then, cor pulmonale.
Acute sarcoidosis normally resolves within 2 months to 2 years. There are risks of sudden disabling symptoms in acute sarcoid.
The risk of chronicity is reduced if the acute illness is of short duration (less than a year), the onset occurs at a young age, there is presence of erythema nodosum, (a good prognostic factor), there is minimum lung involvement i.e. any interstitial shadowing should be mild and short lived, there is no cardiac involvement and no steroids are required.
Hyperalcaemia or hypercalciuria may occur as non caseating granulomas secrete 1,25 vitamin D. This occurs in about 10-13% of patients. The presence of elevated vitamin D levels are associated with protracted treatment in sarcoidosis. The Angiotensin Converting Enzyme (ACE) may be elevated.
One of the main concerns with sarcoidosis is the possibility of cardiac sarcoidosis. Thallium myocardial scans suggest granulomatous involvement of the heart in 30% of cases of sarcoid.
There is clinical evidence that tachy-arrhythmias, heart blocks, cardiomyopathy, congestive cardiac failure and sudden death may occur. The instances of sudden death in patients known clinically to have myocardial involvement are almost 50%, with 65% of these being due to arrhythmia. Unfortunately the risk of sudden death and cardiac dysfunction persists for up to 15 years, perhaps more, after the onset of symptoms, even once the sarcoid symptoms have resolved.
Chronic disease causes a wider and more severe complex of symptoms than the acute disease, and tends to occur in patients in middle age or older. The condition may evolve to progressive pulmonary fibrosis. Advanced disease will cause breathlessness, cough, reduced lung function and exercise intolerance. Any suggestion that the disease is becoming chronic and progressive usually implies that the condition is incompatible with flying status.
A Class 1 applicant who first present with a history of Sarcoidosis should be considered as having a condition that is of aeromedical significance.
A Class 2 or 3 applicant who first present with a history of Sarcoidosis should be considered as having a condition that is of aeromedical significance unless:
