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In 2015, pulmonary sarcoidosis and interstitial lung disease affected 1.9 million people globally and they resulted in 122,000 deaths. [10] [11] It is most common in Scandinavians, but occurs in all parts of the world. [14] In the United States, risk is greater among black people as opposed to white people. [14]
Löfgren syndrome is a type of acute sarcoidosis, [1] an inflammatory disorder characterized by swollen lymph nodes in the chest, tender red nodules on the shins, fever and arthritis. [2] It is more common in women than men, and is more frequent in those of Scandinavian, Irish, African and Puerto Rican heritage.
The disease newly occurs in about 12 per 100,000 people per year. [4] Those in their 60s and 70s are most commonly affected. [4] Males are affected more often than females. [4] Average life expectancy following diagnosis is about four years. [1]
Conglomerate masses can also develop in sarcoidosis, [5] but usually near the hilae and with surrounding paracicatricial emphysema. The disease arises firstly through the deposition of silica or coal dust (or other dust) within the lung , and then through the body's immunological reactions to the dust.
Only 1 percent of people with sarcoidosis will have neurosarcoidosis alone without involvement of any other organs. Diagnosis can be difficult, with no test apart from biopsy achieving a high accuracy rate. Treatment is with immunosuppression. [1] The first case of sarcoidosis involving the nervous system was reported in 1905. [2] [3]
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The differential diagnosis includes other types of lung disease that cause similar symptoms and show similar abnormalities on chest radiographs. Some of these diseases cause fibrosis, scarring or honeycomb change. The most common considerations include: chronic hypersensitivity pneumonitis; non-specific interstitial pneumonia; sarcoidosis
Granulomatous–lymphocytic interstitial lung disease (GLILD) is a lung complication of common variable immunodeficiency disorders (CVID). It is seen in approximately 15% of patients with CVID. [ 1 ] It has been defined histologically as the presence of (non-caseating) granuloma and lymphoproliferation in the lung. [ 1 ]