Screening Sarcoidosis Patients for Occult Disease

Abstract

As sarcoidosis may involve any organ, sarcoidosis patients should be evaluated for occult disease. Screening for some organ involvement may not be warranted if it is unlikely to cause symptoms, organ dysfunction, or affect clinical outcome. Even organ involvement that affects clinical outcome does not necessarily require screening if early detection fails to change the patient's quality of life or prognosis. On the other hand, early detection of some forms of sarcoidosis may improve outcomes and survival. This manuscript describes the approach to screening sarcoidosis patients for previously undetected disease. Screening for sarcoidosis should commence with a meticulous medical history and physical examination. Many sarcoidosis patients present with physical signs or symptoms of sarcoidosis that have not been recognized as manifestations of the disease. Detection of sarcoidosis in these instances depends on the clinician's familiarity with the varied clinical presentations of sarcoidosis. In addition, sarcoidosis patients may present with symptoms or signs that are not related to specific organ involvement that have been described as parasarcoidosis syndromes. It is conjectured that parasarcoidosis syndromes result from systemic release of inflammatory mediators from the sarcoidosis granuloma. Certain forms of sarcoidosis may cause permanent and serious problems that can be prevented if they are detected early in the course of their disease. These include (1) ocular involvement that may lead to permanent vision impairment; (2) vitamin D dysregulation that may lead to hypercalcemia, nephrolithiasis, and permanent kidney injury; and (3) cardiac sarcoidosis that may lead to a cardiomyopathy, ventricular arrhythmias, heart block, and sudden death. Screening for these forms of organ involvement requires detailed screening approaches.

From Karl Wurm and Guy Scadding's staging to 18F-FDG PET/CT scan phenotyping and far beyond: perspective in the evading history of phenotyping in sarcoidosis

“…Prognosis is excellent in Löfgren's, and indeterminate in the Heerfordt's syndrome ( 1 , 8 ). However, despite the fact that the abovementioned phenotypes are narrow in terms of clinical manifestations and are relatively homogeneous, nothing is known about other silent systemic sites of active disease; better detection and definitive identification of these silent systemic sites are possible with the application of new technologies ( 7 ). As disease evolved, technology and research followed its lead.…”

“…The etiologically implicated antigen “still eludes us,” and the disease is considered a dysregulated, immune-mediated response due to its presence, persistence, and failure to clear, leading to tissue granulomas formation ( 4 , 5 ). The histological hallmark of the disease indeed constitutes well-formed, non-caseating granulomas that may localize in any organ or tissue without boundaries and with any combination patterns of active sites of involvement in most of the cases, notably, even in the clinically silent ( 6 , 7 ) cases. The detection of granulomas with the abovementioned characteristics is never pathognomonic for the disease ( 8 ).…”