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Sarcoidosis (also called sarcoid or Besnier-Boeck-Schaumann disease) is an acute or chronic inflammatory disease of unknown origin that can affect various organs. The condition is characterised by nodules (granuloma), which can develop at any site in the body and disrupt the functioning of the affected organs. The lungs are among the most commonly affected areas.
While in more than half of sarcoidosis patients the condition presents a benign clinical course, concluding into remission in less than 3 years, it can also develop into a chronic form. This is accompanied by an increased mortality risk, often in connection with pulmonary or cardiac impairment.
Chronic forms of sarcoidosis can persist over multiple decades, which is why monitoring of the disease progression and prediction the disease course play an important role in treatment.
Immunosuppressive therapy is recommended in those cases where the disease progresses into clinically significant tissue damage or in case of quality-of-life degradation.
Sarcoidosis is difficult to diagnose because the symptoms are manifold and widely vary from person to person. As a result, the diagnostic workup is poorly standardized and involves several steps.
Diagnosis is usually based on
Multiple imaging procedures as well as pulmonary function tests are commonly employed in the assessment of the disease. Tissue biopsy is a diagnostic corner stone, as it enables to establish the presence of non-necrotizing granulomas.
Approximately 60 % of patients with sarcoidosis exhibit increased concentrations of angiotensin converting enzyme (ACE) in serum or heparin plasma. Additionally, ACE levels tend to be reduced after treatment with immunosuppressive agents.
Serological determination of the ACE concentration is therefore particularly useful in the follow-up and monitoring after the initiation of therapy.
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