New ICD-10 Codes in US, Specific for Sjögren’s Patients, in Effect

After a four-year effort led by the Sjögren’s Foundation, new health statistics codes in the U.S. — known as ICD-10 codes — recognizing Sjögren’s syndrome as a distinctive condition from “sicca” are now in effect.

The codes are officially known as the International Statistical Classification of Diseases and Related Health Problems — ICD for short — and are designated by the National Center for Health Statistics. The classification system is used globally by healthcare systems, government agencies, and researchers to track and report disorders, injuries, and other health conditions.

With dedicated codes, Sjögren’s specialists can more easily conduct investigations and determine disease prevalence, morbidity, and mortality rates. The coding changes will also benefit providers and address key Sjögren’s complications, including Sjögren’s with inflammatory arthritis or dental involvement, among others that were not included in the former code.

Before the changes, made last summer, Sjögren’s fell under “sicca,” which means dryness. While sicca is a chief Sjögren symptom  — the disease can cause dryness in the eyes, mouth, and elsewhere — the two are not interchangeable. Sjögren’s can affect multiple organs and body systems, the foundation, along with the American College of Rheumatology and a group of multi-specialty experts, had argued.

Sicca symptoms also can be seen in people without Sjögren’s. Such individuals, commonly referred to as non-Sjögren’s sicca patients, lack characteristic Sjögren’s features such as Ro/SSA and La/SSB autoantibodies and immune cells that infiltrate salivary glands.

“While dryness (sicca) certainly occurs in Sjögren’s, dryness alone does not represent the disease and its many other manifestations,” the foundation states in a press release.

Several countries use the ICD classifications, maintained by the World Health Organization, to document patients’ medical conditions for epidemiology and research, and for health insurance coverage and reimbursement.

Diseases and conditions are listed in a way that permit easy storage, retrieval, and analysis of medical information for evidence-based decision making. Health information can be shared and compared among clinical settings, regions, and countries.

“The current ICD-10 code creates confusion, contributes to misinformation and is potentially detrimental to patients, whose diagnosis carries an incorrect label,” the foundation wrote in the summer edition of its quarterly publication, before the Sjögren’s coding changes were made.

This “can influence subsequent difficulty with insurance reimbursement, clinicians, who may be unsure how to best code the disease, study investigators and researchers, who may have trouble accurately identifying Sjögren’s patients for clinical trials and data acquisition, and insurers, who grapple with inaccuracy, which can influence reimbursement to both patients and providers,” it added.

Efforts to gain the new coding began in 2017, when the foundation and a team of multidisciplinary experts convened and collaborated on the best course forward, culminating in a meeting with the federal ICD-10 Coordination and Maintenance Committee, which had accepted the group’s suggestions for code modifications. That led to a public comment period and, ultimately, official changes.