Authors' Response to Silicosarcoidosis: Do We Really Need Hybrid Labels That May Undermine Occupational Disease Recognition?

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We thank Akgün et al. [1] for their thoughtful responses to our article on silicosarcoidosis [2]. We agree that a “sarcoidosis-first reflex” is often counterproductive, and that the persistent lack of priority given to occupational history-taking leads to missed opportunities for exposure control and prevention. These points are in fact the primary reason why we think the term silicosarcoidosis, rather than creating more complexity, underscores the causal link between silica exposure and the granulomatous reaction that characterizes sarcoidosis. As the authors note, beryllium exposure can be distinguished from sarcoidosis based on a careful exposure history in combination with the beryllium lymphocyte proliferation test. In contrast, efforts to develop a clinically valid and widely available silica lymphocyte proliferation test to help distinguish silicosis from idiopathic sarcoidosis remain unrealized. Until a sensitive and specific biomarker for silicosarcoidosis is developed, the only feature distinguishing these two entities, in the absence of an adequate biopsy, is the exposure history. We disagree that the term silicosarcoidosis hinders compensation systems or compromises workers' rights to benefits for occupational disease. In our experience, a diagnosis of sarcoidosis implies an idiopathic, non-work-related disease for which compensation becomes problematic if not impossible. For example, radiologists hired by mine operators to interpret chest radiographs for coal workers' pneumoconiosis may decline to provide profusion scores for images they conclude show sarcoidosis [3]. Linking etiology with clinical findings via the “hybrid” term silicosarcoidosis should help both clinicians and legal/regulatory systems recognize the medical and public health implications of this diagnosis. Our article points out that when the term ACO (Asthma-COPD Overlap) entered the lexicon, the field took a major step forward. Previously, patients with ACO often were excluded from clinical trials and studies of asthma or COPD alone. Creating a diagnostically accurate term that was simple to articulate and remember provided the foundation enabling a multinational group to develop consensus definitions. Asthma and COPD are now recognized as distinct diseases with differing genetic predispositions that may coexist in an individual. As the ACO example illustrates, the term silicosarcoidosis, even if imperfectly defined at this time, creates the long overdue foundation needed to develop more detailed consensus case definitions, characterize clinical phenotypes (including frequency of extra-pulmonary involvement and predictors of disease progression), improve patient outcomes through combined pharmacologic and exposure management, and expand research into pathogenetic mechanisms. Whether or not the term silicosarcoidosis stands the test of time, Akgün et al. and other exposure- and prevention-oriented clinical investigators [4] will be important voices in helping take these vital next steps. Cecile S. Rose drafted the initial manuscript with input from Jeremy T. Hua. All authors critically reviewed and approved the final manuscript. The authors have nothing to report. The authors received no specific funding for this work. The authors have nothing to report. The authors declare no conflicts of interest. The authors have nothing to report.

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