Sjogren's Quarterly: Why Language Matters

 I enjoy all of the Sjogren's Syndrome Foundation's publications. The most recent edition of Sjogren's Quarterly, The Professionals' Resource on Sjogren's lead article was entitled "Why Language Matters" and written by Florian Kollert, MD and Benjamin A. Fisher, MD. It was a very interesting read in which the authors voiced their concerns over the use of 'primary' and 'secondary' terms to classify Sjogren's Syndrome. Here's an excerpt: 

Historically, Sjogren's has been classified into 'primary' and 'secondary' disease. 'Primary Sjogren's is defined as a standalone entity occurring in the absence of another systematic autoimmune disease, whereas 'secondary' disease is associated with the presence of rheumatoid arthritis (RA), systemic lupus erythematosus (SLE) or systemic sclerosis (SSc), for example. Notably, the presence of a coexistent autoimmune disease is very common in Sjogren's (approximately 30% overall) when organ specific autoimmunity is also included.

In our recently published article (Kollert & Fisher, Rheumatology) we reviewed the historical justification for the distinction of 'primary' and 'secondary' Sjogren's based on genetics, clinical presentation, chronology, histology and serology, and found it difficult to justify the dichotomy based on existing evidence. We therefore recommend further research, and advocate abandoning the term 'secondary' unless strong evidence emerges of a pathological difference between these subsets. We further argue for a nomenclature including the associated disease (Sjogren's in association with...) to not only emphasize the second autoimmune disease but also Sjogren's itself. In our perspective, Sjogren's is an under-researched disorder, a situation that is even worse for patients with so called 'secondary' disease. This stands in sharp contrast to the potential impact of Sjogren's on quality of life, even when compared to other systemic  rheumatological disorders..............

.........Patients with 'secondary' Sjogren's are often excluded from clinical trials and were not incorporated in the development of the most recent classification criteria for Sjogren's. They were considered in the widely used 2002 criteria but neither histopathology nor autoantibodies were necessary for classification as 'secondary' Sjogren's. Thus, recent clinical trials investigating new compounds for patients with Sjogren's typically recruit patients with primary Sjogren's only. So it seems unclear if a drug which is eventually proven to have efficacy for these 'primary' Sjogren's patients; will be accessible to patients with 'secondary' disease also. Conversely, studies of other systemic autoimmune diseases such as lupus or rheumatoid arthritis have typically not excluded patients with concomitant Sjogren's. This provides a largely unexplored opportunity to derive additional early signals of potential efficacy in Sjogren's.......

.......Taken together, as there is currently no evidence for a major difference between the phenotype of 'secondary' and 'primary' Sjogren's we argue in favor of using the same set of classification criteria for both. Moreover, we take the side of abandoning the term 'secondary' in favor of 'Sjogren's in association with' to emphasize not only Sjogren's but also the associated autoimmune disease. The overlap between different systemic autoimmune diseases should be regarded as an opportunity to foster drug development and to further stratify our available treatment modalities and personalize our therapies. 

Well said. I agree on all points. 

Has your rheumatologist diagnosed you with 'secondary' Sjogren's syndrome? Has your care been affected as a result?