In early 2018, the ACR and the Sjögren’s Syndrome Foundation joined forces to request a code change in the ICD-10 classification for Sjögren’s syndrome, the second most common autoimmune rheumatic disease. An advisory team, comprising specialists in rheumatology, as well as representatives from eight additional specialties involved in managing Sjögren’s syndrome patients, was assembled. The team helped craft the proposal presented to the ICD-10 Coordination and Maintenance (C&M) Committee during its September 2018 meeting at the Department of Health and Human Services (HHS) Centers for Medicare & Medicaid Services (CMS) office in Baltimore and again in March 2019. Public comment periods followed each presentation, and in July 2019, the C&M committee approved the Sjögren’s syndrome diagnosis code change request, which becomes effective October 2020.
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Explore This IssueOctober 2019
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The Need to Update Sjögren’s Syndrome Coding
The groundwork to revise the Sjögren’s syndrome diagnosis code was started specifically to separate it from the tabular listing of M35.0 in the ICD-10 coding manual, which is linked to sicca syndrome. The advisory team (see list below) determined the current ICD-10 code is inaccurate and doesn’t contribute to the highest level of specificity of coding for Sjögren’s syndrome. The existing code, M35.0, sicca syndrome [Sjögren], is misleading, because sicca syndrome, in current parlance, refers to the symptom complex of dry eyes and dry mouth without consideration of etiology. There are many potential causes of sicca syndrome, including side effects of commonly used medications and inflammatory diseases of the saliva and tear-producing glands. In the past, sicca syndrome was used synonymously with Sjögren’s syndrome, but now, with a better understanding of Sjögren’s syndrome, we know they are distinct terms and should not be used interchangeably.
Sjögren’s syndrome is a systemic autoimmune disease that affects the entire body. In Sjögren’s syndrome, the immune system targets the mucous membranes and moisture secreting glands, leading to decreased moisture throughout the body and resulting in decreased tears and saliva, dry skin, dry vagina and decreased moisture in the esophagus, lungs and digestive tract. Along with symptoms of extensive dryness, other serious complications include profound fatigue, chronic pain, major organ involvement, neuropathies and lymphomas.
With the implementation of this revised tabular modification for Sjögren’s syndrome, it is important to review the code changes and begin educating coders, clinical documentation professionals and providers about the changes. Missing pieces are the designations for the final complications and comorbidities, and major comorbidities, which will be published with the Inpatient Prospective Payment System (IPPS) Final Rule for fiscal year 2020 (see Table 1).