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Chapter-21 Sarcoidosis

BOOK TITLE: Uveitis: Text and Imaging

Author
1. Khairallah Moncef
2. Mochizuki Manabu
3. Matsui Kyoko Ohno
4. Takase Hiroshi
5. Attia Sonia
ISBN
9788184484915
DOI
10.5005/jp/books/11002_21
Edition
1/e
Publishing Year
2009
Pages
16
Author Affiliations
1. Fattouma Bourguiba University Hospital; University of Monastir, 5019 Monastir, Tunisia, University of Monastir, Tunisia
2. Tokyo Medical and Dental University, Graduate School of Medicine, Tokyo, Japan, Tokyo Medical and Dental University Graduate School of Medicine, Tokyo, Japan
3. Tokyo Medical and Dental University, Graduate School of Medicine, Tokyo, Japan
4. Tokyo Medical and Dental University, Graduate School of Medicine, Tokyo, Japan
5. University of Monastir, Tunisia
Chapter keywords
Sarcoidosis, granulomatous disease, bilateral hilar lymphadenopathy, pulmonary infiltrates, skin lesions, fluorescein angiography, Indocyanine green angiography, ocular sarcoidosis, keratoconjunctiva sicca, conjunctival nodules, lacrimal gland, systemic cellular immunity

Abstract

Sarcoidosis is a chronic multisystem granulomatous disease of unknown etiology characterized by the presence of noncaseating granulomas in involved organs. It predominantly affects the lungs and intrathoracic lymph nodes, but any organ or system can be involved. The disease can involve the orbit, lacrimal gland, anterior and posterior segments. The etiology of sarcoidosis is unknown, and it is assumed that systemic cellular immunity against exogenous infectious agents initiates the disease and causes the granuloma formation. The putative causative infectious agents of sarcoidosis include mycobacterium tuberculosis, mumps, and propionibacterium acnes. Sarcoidosis generally presents with bilateral hilar lymphadenopathy (BHL), pulmonary infiltrates and skin or eye lesions. Symptomatic patients with pulmonary sarcoidosis present with nonproductive cough, dyspnea, chest pain, fatigue, weakness, malaise, fever, and weight loss. Extrapulmonary sarcoidosis can involve any organ or system. Skin lesions may be found in at least 15% of patients. Ocular involvement occurs in 20 to 80% of patients with sarcoidosis at any time during the course of the disease. Common manifestations of orbital sarcoidosis include palpable mass, proptosis, palpebral swelling, ptosis, diplopia, keratoconjunctiva sicca, exposure keratopathy, and myositis. Lacrimal gland infiltration has been noted in up to 30% of patients with ocular sarcoidosis. Conjunctival nodules have been reported in 6. 9 to 70% of patients with ocular sarcoidosis. Anterior segment involvement is observed in nearly 85% of patients with ocular sarcoidosis. Anterior uveitis is the most common ocular manifestation of sarcoidosis. It is usually bilateral, chronic, and granulomatous, characterized by mutton fat or small keratic precipitates, iris nodules, and posterior synechiae. Intermediate uveitis has been reported in 16 to 38% of patients with posterior segment involvement. Fluorescein angiography (FA) is a useful tool for the diagnosis and follow-up of posterior segment involvement in sarcoidosis. It is especially useful in the evaluation of retinal vascular involvement. Indocyanine green angiography (ICGA) provides useful informations for choroidal involvement in the eyes with ocular sarcoidosis.

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