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Sarcoidosis is a multisystem granulomatous disease with nonspecific clinical manifestations that commonly affects the pulmonary system and other organs including the eyes, skin, liver, spleen, and lymph nodes
- Systemic disease of unknown etiology.
- Adults from 20-40 years of age.
- The disease is characterised by the presence of noncaseating epithelioid cell granulomas (‘sarcoid granuloma’) in the affected tissues and organs, notably lymph nodes and lungs.
- Other sites are the skin, spleen, uvea of the eyes, salivary glands, liver and bones of hands and feet.
- The histologic diagnosis is generally made by exclusion
ETIOLOGY AND PATHOGENESIS
- Infectious agents include Propionibacter acnes, atypical mycobacteria and mycobacterial protein of M. Tuberculosis.
- The disease is characterised by granulomatous tissue reaction, possibility of cell-mediated immune mechanisms has been suggested.
- The following observations point towards a possible immune origin of sarcoidosis:
MICROSCOPICALLY FEATURES
- The diagnostic feature in sarcoidosis of any organ or tissue is the noncaseating sarcoid granuloma, composed of epithelioid cells, Langhans’ and foreign body giant cells and surrounded peripherally by fibroblasts.
- Typically, granulomas of sarcoidosis are ‘naked’ i.e. either devoid of peripheral rim of lymphocytes or there is paucity of lymphocytes.
- In late stage, the granuloma is either enclosed by hyalinised fibrous tissue or is replaced by hyalinised fibrous mass.
- The giant cells in sarcoid granulomas contain certain cytoplasmic inclusions
as follows:
- Asteroid bodies which are eosinophilic and stellateshaped structures.
- Schaumann’s bodies or conchoid (conch like) bodies which are concentric laminations of calcium and of iron salts, complexed with proteins.
- Birefringent cytoplasmic crystals which are colourless. Similar types of inclusions are also observed in chronic berylliosis
KVIEM’S TEST. It is a useful intradermal diagnostic test. The antigen prepared from involved lymph node or spleen is injected intradermally. In a positive test, nodular lesion appears in 3-6 weeks at the inoculation site which on microscopic examination shows presence of non-caseating granulomas
CLINICAL FEATURES
Between 30% and 60% of cases are asymptomatic, detected incidentally by an abnormal chest radiograph.
Constitutional symptoms may occur and consist of :
- Fever,
- Fatigue,
- Anorexia,
- Cough,
- Dyspnea, and
- Vague retrosternal discomfort.
Syndromes such as erythema nodosum, anterior uveitis, arthritis, parotid enlargement, and facial nerve palsy occur in acute sarcoidosis.
Diagnosis requires relevant clinical features and a tissue biopsy showing characteristic noncaseating granulomas.
Death from sarcoidosis usually results from progressive pulmonary, cardiac, or neurologic involvement.
TREATMENT
- Many people with sarcoidosis do not need treatment as the condition often goes away on its own, usually within a few months or years.
- In this case, simple lifestyle changes and over-the-counter painkillers are often all that's needed to control the pain of any flare-ups.
- Doctors will monitor your condition to check if it's getting any better or worse without treatment. This can be done with regular X-rays, breathing tests and blood tests.
