The WASOG Sarcoidosis Organ Assessment Instrument: An update of a previous clinical tool.

TLDR

The ACCESS instrument, used for over a decade to standardize sarcoidosis organ involvement probability, has become outdated and incomplete, lacking coverage of all possible organs. WASOG created a new sarcoidosis organ assessment instrument to evaluate the probability of organ involvement based on expert assessment of clinical manifestations. The instrument applies two criteria—histologic evidence of unknown‑cause granulomatous inflammation in an unassessed organ and exclusion of alternative causes—and classifies manifestations as highly probable (≥90 %), probable (50–90 %), or possible (<50 %) using Delphi consensus. Expert Delphi voting yielded consensus classifications of manifestations into highly probable, probable, possible, or indeterminate, producing an instrument that clinicians and researchers can use to establish sarcoidosis organ involvement criteria.

Abstract

A Case Control Etiology of Sarcoidosis Study (ACCESS) sarcoidosis organ assessment instrument has been used for more than a decade to establish uniform standards for the probability of sarcoidosis organ involvement. The ACCESS instrument has become increasingly outdated as new technologies have been developed. Furthermore, the ACCESS instrument failed to address all possible organs involved with sarcoidosis. For these reasons, the World Association of Sarcoidosis and Other Granulomatous Diseases (WASOG) developed a new sarcoidosis organ assessment instrument.Clinical sarcoidosis experts assessed various clinical manifestations for the probability of sarcoidosis organ involvement. Two criteria were required to apply this assessment: 1) histologic evidence of granulomatous inflammation of unknown cause in an organ that was not being assessed; 2) the clinical manifestation being addressed required that alternative causes other than sarcoidosis had been reasonably excluded. Clinical manifestations were assessed as either: a) highly probable: likelihood of sarcoidosis causing this manifestation of at least 90%. ; b) probable: likelihood of sarcoidosis causing this manifestation of between 50 and 90%; c) possible: likelihood of sarcoidosis causing this manifestation of less than 50%. The sarcoidosis experts voted on the likelihood of sarcoidosis causing each manifestation using Delphi study methodology where at least 70% agreement of the experts was needed for consensus.Various clinical manifestations were classified as highly probable, at least probable, possible, or indeterminate when no consensus could be reached.An instrument was developed by expert opinion that may be useful for the clinician and researcher in establishing criteria for sarcoidosis organ involvement.