Sarcoidosis: Treatment, symptoms, advice & help
Sarcoidosis is a multi-system disorder of unknown cause that is characterized by the presence of non-caseating granulomas.
Sarcoidosis: Incidence, age and sex
The condition is more frequently described in colder parts of northern Europe. It also appears to be more common and more severe in those from a West Indian or Asian backgrounds; Inuit (Eskimos), Arabs and Chinese are rarely affected.
Signs & symptoms of sarcoidosis: Diagnosis
Over 90% of cases affect the lungs, but the condition can affect almost any organ. Pulmonary disease presents insidiously with cough, exceptional breathlessness and radiographic infiltrates; chest auscultation is often unremarkable.
Erythemanodosum and arthralgia (20 – 30%), eye symptoms (5-10%), skin sarcoid (including lupus pernio) (5%), superficial lymphadenopathy (5%), hypercalcaemia, diabetes insipidus, cranial nerve palsies, cardiac arrhythmias and nephrocalcinosis may be seen.
Hypercalcaemia may be present (reflecting increased formation of calcitriol(1,25 dihydroxyvitamin D3) – by alveolar macrophages), particularly if the patient has been exposed to strong sunlight. Hypercalciuria may also be seen and may lead to nephrocalcinosis. Serum angiotensin – converting enzyme (ACE) is a non-specific marker of disease activity and can assist in monitoring the clinical course. Chest radiography has been used to stage sarcoid.
Characteristic HRCT appearances include reticulonodular opacities that follow a perilymphatic distribution centred on bronchovascular bundles and the subpleural areas. The occurrence of erythemanodosum with HBL on chest x-ray is often sufficient for a confirmed diagnosis.
Causes and prevention of sarcoidosis:
The tendency for sarcoid to present in the spring and summer has led to speculation that infective agents, including mycobacteria, propionibacteria and viruses may be involved, but the cause remains elusive. Genetic susceptibility is supported by familial clustering.
Complications such as bronchiectasis, aspergiloma, pneumothorax, pulmonary hypertension and corpulmonale have been reported but are rare. The overall mortality of low (1- 5 %) and usually reflects cardiac involvement or pulmonary fibrosis.
Patients who present with acute illness and erythemanodosum should receive NSAIDs and, if symptoms are severe, a short course of corticosteroids. Topical steroids may be useful in cases of mild uveitis, and inhaled corticosteroids have been used to shorten the duration of systemic corticosteroid use in asymptomatic parenchymal sarcoid. Patients should be warned that strong sunlight may precipitate bypercalcaemia and endanger renal function. In patients with severe disease methotrexate (10 – 20 mg / week), azathioprine (50-150mg / day) and specific TNF-a inhibitors (p.1080) have been effective. Chloroquine, hydroxychloroquine and low-dose thalidomide may be useful in cutaneous sarcoid with limited pulmonary involvement selected patients may be referred for consideration of single lung transplantation.