Sarcoidosis def., cause, manifestations, diagnosis, prognosis and treatment

This article is to discuss Sarcoidosis regarding its definition, pathology, clinical picture, diagnosis, investigations , indications of treatment and method of management .

What is meant by Sarcoidosis ?

 It is a systemic granulomatous disease of unknown etiology characterized by T.cell abnormality with lymphopenia and infiltration of tissues with T cells ===> Noncaseating epithelioid granuloma in various organs with derangement of normal tissue architecture.
There is giant cells within the granuloma (Ianghans) with inclusions e.g schaumann and asteroid bodies.

Immunologic defects:

• There is impaired cellular immunity characterized by a complete skin anergy to tuberculin and other common skin antigens .
• Humoral immunity is normal and susceptibility to infections is not increased .

What are the manifestations of Sarcoidosis ?

Clinical picture
1- Lung : manifestations of interstitial pulmonary disease e.g exertional dyspnea, cough with fine crepitations.
2- Extrapulmonary manifestations:

* Sacrocidosis may be presented with acute onset (giving rise to two syndromes):

Erythema nodosum, acute arthritis and hilar adenopathy (Lofgren's syndrome), uveitis, parotid enlargement and facial plasy (Heerfordt waldenstrom syndrome).
* Insidious onset sarcoid presented mainly by respiratory manifestations with less frequent constitutional or extra thoracic manifestations.
* Pleurisy is uncommon in sarcoidosis.

How to diagnose Sarcoidosis ?

1) X-ray stages0- Normal
1- Bilateral hilar L.N. enlargement
2- Interstitial pulmonary infiltrate + hilar L.N.
3- Interstitial pulmonary infiltrate only.
4- Fibrosis and honeycombing
2)High S.Ca and high ESR
3) Hypoxia (by blood gases) due to diffusion defect
4) Bronchoalveolar lavage (SAL) showing increase of lymphocytes (indicator of disease activity.
5) Transbronchial biopsy from lung showing non caseating granuloma
6) Gallium lung scan showing diffuse uptake.
7) High serum level of angiotensin converting enzyme (indicator of disease activity).
8) Tuberculin is -ve in 80% of cases (anergy).
9) Kveim test by intradermal injection of sarcoid extract leading to sarcoid like lesions after 4-6 wks.
10)Pulmonary function tests showing diffusion defect with evidence of restrictive hypoventilation.

Treatment of Sarcoidosis

 Many cases remit spontaneously, corticosteroid administration is the principal treatment.

The indications of treatment of Sarcoidosis:

* Symptomatic lung disease
* C.N.S. involvement.
* Eye lesions
* Hypercalcemia
* Cardiac involvement
• The usual therapy is prednisone 1 mg/kg/d for 4-6 weeks then slow tapering over 2-3 months, this regimen is repeated if the disease again becomes active. Cyclosporine may be useful in extrathoracic sarcoid not responding to steroids. Angiotensin converting enzyme and BAL (indicators of disease activity) are used for follow up.

Prognosis and outcome of sarcoidosis:

• Most patients with acute disease are left with no significant residual effects.
• 50% of patients have some permanent organ dysfunction.
• 15-20% of patients remain with active or recurrent disease.
• Death occur directly due to disease in 10% of cases.
• The mortality and morbidity are mainly related to the respiratory tract abnormality.

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