Promoting optimal care for Sjogren's, a common, serious, systemic disease.
SICCA (MORE THAN DRYNESS)
Key Concepts
What Is Sicca? - Sjogren's-related sicca is often equated with dry eyes and dry mouth, but it is much more than that. Sjogren's sicca is a combination of dryness (a loss of secretions), inflammation, neuropathy, dysfunction of the autonomic nervous system, and changes in the compositions of secretions and microbiota in the body.
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Sicca And Sjogren's - Not everyone with Sjogren’s has sicca, although most do by the time they are diagnosed. Not everyone with sicca has Sjogren’s. Sjogren’s is always systemic, even in patients who have sicca-dominant disease.
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Oral Sicca - When not managed aggressively, oral sicca may lead to severe dental decay (caries) or even loss of teeth.
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Sicca Of The Airways - Severe airway dryness damages the mucosal surfaces of the airways and may damage nerves.
What Is Sicca?
Sicca in Sjogren's disease is a combination of a loss of secretions (dryness), inflammation, neuropathy, dysfunction of the autonomic nervous system, and changes in the compositions of secretions and microbiota in the body.
One of the most well-known symptoms of Sjogren's is sicca, informally described as dry eyes and mouth. But sicca is much more than a loss of secretions (dryness). Sicca impacts the eyes, mouth, airways, gastrointestinal (GI) tract, skin and vagina. Non-rheumatology clinicians usually manage sicca of the GI tract, skin and genitals. Click here to learn more about Sjogren's and skin health.
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Sjogren's And Sicca
Not everyone with Sjogren’s has sicca, and not everyone with sicca has Sjogren’s. Sjogren’s is always systemic, even in patients who have sicca-dominant disease. About 20% of people with Sjogren’s present with systemic (non-sicca) features and no obvious dryness (3, 8, 232). For most patients, sicca symptoms help point to a Sjogren’s diagnosis. Even though sicca is the most obvious and visible feature of Sjogren's, most Sjogren's patients identify fatigue and pain as their most burdensome symptoms (169).
Every Sjogren’s patient should be followed and given the proper care for both sicca and non-sicca Sjogren’s features. Without adequate care, sicca may cause permanent harm, including loss of teeth, visual impairment, or even blindness.
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Most people seek a diagnosis of Sjogren's after sicca has far progressed. Even then, most people don't think to mention sicca symptoms to their PCPs (234), because they think they are unimportant or unrelated to their systemic symptoms.
Although Sjogren's may present in many different ways, common early presentations of Sjogren's include extreme fatigue, cognitive impairment, joint and muscle pain, "irritable bowel syndrome", persistent cough, and neuropathy/dysautonomia features. Early Sjogren's symptoms are often misattributed to fibromyalgia, anxiety, depression, or functional neurological disorder.
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Sicca is just one of many Sjogren's features.
Sicca is present in most, but not all, Sjogren's patients.
It is important to monitor and manage sicca, even when symptoms are mild.
Resources For Managing Sicca In Sjogren's
Sjogren’s Foundation Resources On Sicca
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National Patient Conference Recordings - Excellent presentations about sicca.
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Sjogren’s Clinical Practice Guidelines, Oral - Share these guidelines with your dentist.
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Sjogren’s Clinical Practice Guidelines, Ocular (eye) - Share these guidelines with your eye doctors. Note: The Ocular Guidelines do not include some of the newest prescription treatments.
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Brochures and Resource Sheets - Information on a variety of topics including dry eyes and dry mouth
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Other Resources On Sicca
The Sjogren’s Book (214) - ​See the Citations page for information about this book.
Sjogren’s Syndrome: A Clinical Handbook (151) - ​See the Citations page for information about this book.
Smart Patients - ​The Sjogren's patient group is a great online support group for practical tips
Treating Dry Eye - A detailed, but easy to understand, overview of Sjogren's dry eye management. Good for both patients and clinicians.
HOW DRY ARE YOUR EYES? - Blog post with link to dry eye video
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Ocular (Eye) Sicca / Dry Eye Disease Caused By Sjogren's
On average, it takes 10 years from a diagnosis of ocular sicca to a diagnosis of Sjogren’s (69). About 10% of ocular sicca patients have Sjogren’s. Eye doctors often treat ocular sicca without considering Sjogren’s as a possible cause, “Screening for SS (Sjogren’s) by ophthalmologists caring for patients with dry eye is not common practice, partly because serious ocular complications secondary to SS are not widely recognized.” (35)
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Time to Sjogren’s diagnosis could be improved if eye doctors asked about systemic (non-sicca) symptoms in ocular (eye) sicca patients. Likewise, other clinicians should ask about about ocular sicca symptoms in patients with common systemic features.
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Because Sjogren's sicca may not be interpreted as dryness, clinicians should ask patients about the symptoms listed in the blue box below, if patients answer "no" to when asked about dry eyes.
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Every Sjogren’s patient should be monitored by an eye doctor who is familiar with Sjogren’s eye disease at least once a year. The eye specialist is often an ophthalmologist (MD eye doctor) because patients taking hydroxychloroquine (HCQ) need to undergo routine retinal screening. An optometrist is often a good choice for managing dryness but they will need to refer to an ophthalmologist for HCQ screening or if signs and symptoms of systemic (non-sicca) eye disease occur.
Ocular sicca symptoms are usually caused by a loss of tear volume as well as meibomian gland disease. The meibomian glands secrete lipids that stabilize the eye surface. This keeps tears from evaporating quickly. Many patients need to treat both aspects of dry eye disease.
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People with Sjogren's may have decreased functional vision even if they have normal vision based on reading a standard eye chart. Blurring, difficulty reading, difficulty looking at a TV, computer, or smartphone screen, and poor night vision may be caused by a disrupted tear film.
Ocular sicca may cause serious damage. Sicca caused by Sjogren's is often more damaging than sicca not caused by Sjogren's. People with mild sicca symptoms caused by Sjogren's may be accruing serious damage (235). Ocular sicca caused by Sjogren's is not trivial; when ignored it can lead to corneal ulcers, altered vision, or even blindness (69).
Not all eye diseases caused by Sjogren’s are related to dryness. Systemic (non-sicca) eye manifestations such as uveitis, scleritis, and corneal melt may occur (69, 156). These vision-threatening emergencies require urgent treatment. Sudden onset of eye pain, vision changes, and redness may indicate one of these conditions.
Symptoms Of Ocular (Eye) Sicca
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Dryness/inability to produce tears
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Eye irritation, pain, or burning
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Blurry vision, needing to blink to correct vision
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Redness, sensitivity to light, poor night vision
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A gritty feeling or foreign body sensation in the eyes
Note: It is safe to assume that people who use prescription treatments for dry eye (Restasis, Cequa, Xiidra, etc.) or artificial tears more than two times a day have sicca.
Oral Sicca / Dry Mouth Caused By Sjogren's
When not managed aggressively, oral sicca may lead to severe dental decay (caries) or even loss of teeth. People with Sjogren's often do not mention oral health concerns to anyone other than their dentist. Primary care clinicians and others should ask about eye and oral sicca any time that a patient presents with systemic symptoms that suggest Sjogren’s.
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Because sicca caused by Sjogren's may not be interpreted as dryness, clinicians should ask patients about a variety of sicca symptoms if they answer "no" when asked about dry mouth.
Symptoms Of Oral Sicca Caused By Sjogren's
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Dry mouth sensation
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Difficulty swallowing food without liquids
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Problems with food or pills getting stuck
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Severe, unexplained caries despite good oral hygiene
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Burning, irritated tongue and mouth
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Recurrent candida infections (thrush) and angular cheilitis
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Heartburn [gastroesophageal reflux disease (GERD)]
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Difficulty tasting food
Sjogren’s patients may develop severe caries despite practicing good dental hygiene.
Dental caries do not occur in proportion to measured saliva flow. Other factors such as microbiota, lack of mucin secretion, and inflammatory changes contribute to dental decay and sometimes loss of teeth.
Oral candidiasis and angular cheilitis
Oral candidiasis (thrush) often causes bright red mucous membranes, rather than the typical “cottage cheese” appearing film in the mouth. These conditions often require 10-14 days of treatment with antifungal medication.
Burning mouth and tongue
Burning mouth and burning tongue have multiple causes including candidiasis, neuropathy, nutritional deficiencies, and more. Salivary gland blockages (by stones or mucus) and infections may contribute to this and often require treatment by an ear, nose, and throat (ENT) doctor (151, Ch. 3).
Oral sicca contributes to gastroesophageal reflux disease (GERD) (148).
GERD is more likely with oral sicca because saliva buffers stomach acid and loosens mucus. GERD may cause chronic cough. Any Sjogren’s patient with a chronic cough should be evaluated for lung disease.
Difficulty swallowing (dysphagia) may be caused by dryness, autonomic neuropathy, or both (148).
Loss of taste and smell may occur.
This, combined with impaired swallowing often lowers the quality of diet and may result in weight loss and lower quality of life.
Dysautonomia may contribute to ocular and oral sicca symptoms.
Saliva and tear glands are directly damaged by Sjogren’s.
However, the loss of function frequently does not match the amount of damage.
Severe oral dryness has been documented with only a 50% loss of glandular structure, which should be enough to make adequate saliva (233). This data suggests that the autonomic nervous system, which stimulates saliva and tear production, may play an important role in sicca.
Dysautonomia is becoming more widely acknowledged as a substantial contributor to multiple Sjogren’s symptoms.
Sicca Of The Airways Caused By Sjogren's
The moist mucous membranes that line the nose, sinus, and airways provide the first line of defense against respiratory infections. Dryness and inflammation disrupt this important part of the innate immune system. This is part of the reason that Sjogren’s patients tend to experience severe or repeated respiratory infections (39).
People with Sjogren’s may experience recurrent sinusitis, sometimes starting many years prior to diagnosis.
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Chronic cough, productive or non-productive, may be the first sign of Sjogren’s disease (79).
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Any patient with chronic cough should be evaluated for Sjogren’s lung disease according to the Pulmonary Clinical Practice Guidelines (159). Sjogren’s lung disease is common and underdiagnosed. Chronic cough should not be attributed to dryness until lung disease, GERD, and/or laryngopharyngeal reflux (LPR) have been ruled out.
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Severe airway dryness damages the mucosal surfaces of the airways and may damage nerves.
This may lead to bronchiectasis (dilation and inflammation of the medium sized airways) and contribute to compromised lung function and/or pneumonia (214, Ch. 28). Bronchiectasis and other Sjogren’s airway diseases are sometimes misdiagnosed as asthma/ bronchitis.
Skin and vaginal sicca are also common in Sjogren's.
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Updated 11-29-2023