Defining organ involvement in sarcoidosis: the ACCESS proposed instrument

Background: Sarcoidosis is a multiorgan granulomatous disease of unknown ca use. Lack of an objective system for assessment of sarcoidosis to evaluate disease course and effectiveness of therapy is a major problem. Methods: Th e sarcoidosis assessment instrument was developed by the Steering Committee of A Case Control Etiologic Study of Sarcoidosis (ACCESS) which included i nvestigators at the ten ACCESS Clinical Centers, the Clinical Coordinating Center, and representatives of the National Heart, Blood, and Lung Institut e. This system was developed to assess sarcoidosis organ involvement in ACC ESS patients who would be followed over a two-year period. The system repre sents a consensus of opinions of members of the Steering Committee based on review of their experience and the medical literature. Results. Criteria f or involvement in patients with biopsy-confirmed sarcoidosis are presented for organs and systems that are commonly involved (lung, skin, eyes, liver, calcium metabolism), unusual but clinically important (nervous system, kid ney, heart) and other sites (non-thoracic lymph nodes, bone marrow, spleen, bone/joint, ear/nose/throat, parotid/salivary glands, muscles). Conclusion : The proposed instrument is partially subjective in that it depends upon t he clinician's diligence in pursuing evidence for sarcoidosis involvement o f various organs. It is hoped that this instrument will lead to increased s tandardization in the definition of sarcoidosis organ involvement to help c linicians and researchers better characterize patients with sarcoidosis.