Sarcoidosis

Sarcoidosis

This systemic disease of unknown aetiology with widespread granulomatous

lesions shows a uniform histological picture of epitheloid cell non-caseating

follicles which undergo resolution by hyalinisation followed by fibrosis.

Sarcoidosis is believed to be a malformation of T-lymphocytes and patients

with sarcoidosis have long been known to give a negative Mantoux reaction.

It is more common in females over the age of 15 years who may present with lung disease due to pulmonary infiltrates, bilateral hilar lymphadenopathy and skin or eye lesions.

 

 

The eyes may also be affected in sarcoidosis in respect of:

-conjunctival follicles of characteristic appearance especially in the lower

fornix fold which may be a useful source of tissue for biopsy.

-calcifications of the cornea and conjunctiva due to a raised serum calcium

associated with calciferol sensitivity .

-kerato-conjunctivitis sicca from tear deficiency resulting from lacrimal gland infiltration.

-vasculitis retinae mainly affecting veins, causing irregularity in calibre and

peri vascular cuffing and pigment epithelial defects, both of which may be

revealed by fluorescein angiography even when not clinically obvious.

-orbital masses which may cause proptosis, and paralytic squint.

-meningeal sarcoidosis where granulomas may also cause extraocular muscle palsies, diabetes insipidus, and affect the optic nerves. There may be raised intracranial pressure and papilloedema.

 

Treatment of ocular sarcoidosis

Posterior uveitis and anterior uveitis of marked degree and those with vasculitis

require systemic steroids. Topical steroids and mydriatics are used in

mild anterior uveitis cases. Steroid therapy will have to be continued in the

absence of resolution, although the side effects of steroids have to be weighed carefully against the degree of ocular inflammation and decisions can be dif ficult. Azathioprine is then sometimes used as an alternative medication. Fortunately in most patients the condition gradually resolves allowing treatment to be reduced progressively so that most patients emerge with minimal impairment of ocular function; very rarely the inflammation may be severe and fail to respond to treatment leading to secondary glaucoma, cataract, optic nerve involvement and loss of vision.

 

Phakomatoses

The phakomatoses are a group of conditions in which a variety of structures

derived from neuroectoderm are affected by hamartomas (congenital overgrowths of tissue which is normally found in that location). They are frequently inherited and ocular involvement is common

 

 

 

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