Sarcoidosis
Sarcoidosis derives from the Greek sarco, meaning “flesh,” eidos, meaning “like,” and osis, meaning “condition.” In 1877, Jonathan Hutchinson described the first case at King’s College Hospital in London. Later, Caesar Boeck advanced the description of sarcoidosis by emphasizing the granulomatous inflammation characteristic of this disease. He was the first to use the term sarkoid (sarcoid) because he thought the lesions resembled sarcoma but were benign. In 1953, Sven Löfgren described the acute sarcoidosis syndrome of bilateral hilar adenopathy and erythema nodosum, frequently associated with arthritis, fever, and uveitis. Over the past century, sarcoidosis has become recognized as a multisystem granulomatous disorder with protean manifestations that may affect any organ.
- Sarcoidosis is a systemic inflammatory disorder of unknown cause characterized by noncaseating, granulomatous inflammation that can affect virtually any organ.
- Acute and chronic forms of inflammatory arthritis particularly involve the ankles and knees.
- Cystic bone lesions may produce dactylitis or rarely involve the skull, vertebrae, ribs, or pelvis.
- Muscle involvement is usually asymptomatic but may cause acute or chronic myopathies or nodular masses.
- Nonmusculoskeletal features are common and include bilateral hilar adenopathy, pulmonary infiltrates, uveitis, cardiac and neurologic involvement, and skin lesions such as erythema nodosum and lupus pernio.
The clinical manifestations are diverse, ranging from abnormal chest radiographic findings in an asymptomatic individual (up to 50% of patients) to severe multiorgan involvement. Because there is no specific test for sarcoidosis, the diagnosis is established when well-recognized clinical and radiographic findings are supported by histologic evidence of widespread noncaseating epithelioid granulomas in more than one organ system. Other granulomatous diseases must be excluded.
Clinical features of sarcoidosis
Manifestation |
Presenting (%) |
Cumulative (%) |
Respiratory tract |
25-50 |
95 |
Constitutional |
25 |
33-70 |
Adenopathy |
10-20 |
40 |
Joint disease |
1-14 |
4-38 |
Uveitis |
5 |
10-20 |
Hepatosplenomegaly |
4 |
5-20 |
Cutaneous |
3 |
15-30 |
Other |
4 |
|
Heart |
<1 |
5-10 |
Neurologic |
<1 |
5-10 |
Muscle |
<1 |
1-5 |
Bone |
<1 |
1-13 |
Web: