E-News Exclusive

Unraveling the Web of Sjogren’s Disease

By Barbara A. Rubin MEd, RHIA, CHIP

One of the marketing points to replace ICD-9-CM, with ICD-10-CM, was that ICD-9 was running out of space to add diseases and conditions or even expand related additional digits to a current code. Sjogren’s disease and dry eye are one of those examples. They are two separate conditions and should not be classified as one condition. However, old habits are truly hard to break.

It became common knowledge, even to patients, that “dry eye,” which is not a standalone condition or disease but is a symptom of Sjogren’s, was placed in the same category. The result was all conditions, whether they were dry eye or more complex like Sjogren’s, became known simply as dry eye syndrome and hence fell under the dry eye symptom, not the more crucial disease of Sjogren’s.

This created many issues in regard to being in compliance with coding assignment guidelines. It was not addressed until 2017, when the Sjogren’s Foundation and their advocates took up the campaign to correctly separate those two conditions and educate people: Dry eye is no more than a symptom of Sjogren’s, and the crucial debilitating disease was truly Sjogren’s disease, which is a systemic, cyclic, devastating disease. Four years later, after overcoming many challenges, the Sjogren’s Foundation was finally awarded their request for Sjogren’s disease to be separated from one of its symptoms and recognized as a serious debilitating disease.

Following the public’s being made aware of this victory for change and compliance, the Social Security Administration (SSA) Department for Disability realized the devastating, incapacitating impact of Sjogren’s disease and recognized this condition as permanently debilitating and incurable; it is now on SSA’s application list as being a permanent disabling condition.

The accurate classification for Sjogren’s disease has, in a sense, created an unorthodox setting for recognition as an intense condition because symptoms for years were hidden behind the red herring—dry eye—which was accepted as a condition rather than a symptom. The hidden irony of this thinking is there is no mortality risk to human life from dry eye; however, there is with Sjogren’s disease. Up until October 1, 2022, when the Sjogren’s Foundation was awarded their request to permanently recognize Sjogren’s as a separate entity from “dry eye syndrome,” Sjogren’s was listed under dry eye.

Identifying Sjogren’s Disease
Sjogren’s is an incurable, rare, genetic, autoimmune disease that first attacks moisture-producing glands, attacking the salivary and tear glands first. It can and often does progress to the 11 body systems next. It is defined as an autoimmune disease because it also causes the immune system to mistakenly attack the body’s own healthy cells, tissues, and organs, treating them as foreign invaders. According to studies, the worst symptoms patients experienced were the following:

1. Incapacitating pain. At a single time, the patient could be experiencing pain in multifocal systems: musculoskeletal, nerve, and mental pain, just to mention a few of the 11 body systems that are affected. Many patients have described the pain as debilitating and perhaps the worst pain that they have ever experienced.

2. Incapacitating weakness and extreme tiredness. Sjogren’s can lead to the patient sleeping for more than 12 hours, at times. The depth of the fatigue can be overwhelming.

3. Extreme anxiety. Patients experience an insurmountable amount of anxiety, which often leads to a decrease in socialization. They experience changes in their voice, usually hoarseness when they converse with others, difficulty swallowing, frequently choking on food, difficulty speaking, and a chronic dry cough. Patients often find it more comforting to limit their socialization because not many social circles have the understanding that Sjogren’s is an incurable autoimmune disease.

Many Sjogren’s patients experience Sjogren’s anxiety, which is far different from the usual types of anxiety. Sjogren’s anxiety is far more difficult to tolerate. Studies show up to 74% of patients reported a substantial impact on their emotional well-being. This is likely due to living with a chronic, often invisible and unpredictable, disease that can affect daily life and causes fatigue, pain, and cognitive issues.

4. Hearing loss. The loss of hearing is particularly devastating and a struggle for many patients. According to research from the Hough Ear Institute, moisture and proper hydration are essential for the function of the inner ear, especially the cochlea, because it contains fluid. This fluid is useful in transmitting sound waves. It also houses hair cells. These cells are responsible for converting vibrations into nerve signals. Like the Sjogren’s Foundation, who saw the need to bring the autoimmune disease into compliance of reporting, the Hough Ear Institute is likewise trying to raise awareness of hearing loss as it relates to Sjogren’s disease. They have received approval from the FDA to go forward with a prescription treatment to restore some types of hearing loss by reviving the collapsed and damaged hair cells in the inner ear associated with the cochlea.

Why did it take so long to put all the pieces of the puzzle together to note that Sjogren’s was a condition well beyond dry eye? Probably one of the major reasons the disease has remained silent or hidden for so long is because symptoms may have been considered insignificant and not related to the reason for the office visit. Yet, since our bodies contain approximately 60% water, a dryness complaint is significantly valid.

The hallmark symptom that indicates a patient has Sjogren’s disease is a lack of spit. When the salivary glands are attacked, they can no longer generate moisture to the mouth. Often, a patient may have a severe dry mouth and may elect not to report it, thinking it is unrelated and unimportant, hence delaying the Sjogren’s diagnosis.

There are many more symptoms that are red flags. For example, nervous system symptoms may not be reported due to fear that they may not be taken seriously. The sensation of bugs crawling on the skin is a phenomenon called formication. It is seldom reported on clinical visits; therefore, there is often a delay in identifying it as a condition that should be coded. The Sjogren Foundation notes other frequently missed symptoms as well. Taking into consideration the effects that are mentioned from the above review, the reader can understand how dangerous the incurable, genetic autoimmune disease is and recognize that it is far more serious than a simple case of dry eye.

Coding Tips
Because of multiple, diverse symptoms associated with Sjogren’s, including dry eye, which was an acceptable code for decades and now is merely a symptom of the underlying Sjogren’s Syndrome, insurance carriers may question the validity of the claim and deny payment. There is a significant risk of denial, primarily stemming from the subjective impact of symptoms that at one time were allowed, such as pain, fatigue, and dryness. It is often a struggle to document the long-term, debilitating effects of Sjogren’s.

The best defense for denials of this nature is to always appeal a denial. Never let a denial pass without rebuttal based upon good clinical documentation in the patient record. This warrants a great deal of camaraderie between case managers and coders. This is where pathophysiology weighs in as a good defense.

Coders need to keep in mind that there are specified parts of the clinical record that codes may be extracted from and be sure only the allowable documentation is used to support the codes that are assigned. Coding for Sjogren’s Syndrome M35.0x, requires organ involvement, ie, M35.01-M35.0x, rather than using the unspecified code (M35.00 or Sicca syndrome, which is the old code for dry eye syndrome). This approach will decrease claim denials, appeals, and delays. Key issues include failing to document specific complications such as lung or heart involvement, documenting specific complications such as nerve involvement, and confusing these specific complications with dryness.

The best practice is following the documentation trail and making sure that all nonspecific Sjogren’s codes do not warrant a coding query for higher specificity, based upon clinical hints that the physician may want to revisit before submitting for payment. Remember, any new revision or additional code added to the acceptable new codes is a higher risk for audit focus for several years. This supports the need for an excellent case management and coding report.

— Barbara A. Rubin, MEd, RHIA, CHIP, is a retired AHIMA nationally registered health information administrator with 50 years of service in HIM. Prior to retiring, she taught for 25 years in AHIMA CAHIIM-accredited colleges. She currently writes about health care on various topics.