An important part of Sjogren's
While Sjogren's is far more than a dryness (sicca) disease, sicca features are important. The term "sicca" comes from the latin word "siccus" which means dryness. Sicca occurs when the fluid secreting glands in the body (the exocrine glands) do not produce enough liquid. Sicca usually impacts tear and saliva production, causing dry eyes and dry mouth. Other areas impacted by sicca include the nose, throat, sinuses, airways (trachea, large and small bronchi), gastrointestinal tract, pancreas, vagina, and skin.
Sjogren's should never be called sicca syndrome. It is always systemic, even when sicca features predominate. While most people with Sjogren's have sicca, some do not. About 20 % of people with Sjogren's first present without sicca (2).
Sjogren’s is more than dryness, but even sicca symptoms are “more than dry.” Local inflammation, neuropathic pain, and changes in tear and saliva composition may all contribute to symptoms. Many good treatments are available for eye and oral sicca manifestations. All dentists and eye doctors should be familiar with the oral and ocular (eye) Clinical Practice Guidelines, available at the Sjogren's Foundation website.
While not every Sjogren's patient has sicca, the vast majority do, or eventually will. Ongoing clinical management may prevent serious outcomes such as severe dental caries, loss of teeth, visual impairment, or even blindness. Chronic sinusitis, vaginal irritation/ pain with sex, and chronic dry cough are common in Sjogren's patients. Clinicians should not assume that chronic cough is simply due to dryness, even if dryness contributes to the problem. This can be a symptom pointing to Sjogren's lung disease, a common and often overlooked systemic manifestation.
Glandular Features, Extraglandular Manifestations, and the Glandular Domain
1. Sicca features: often referred to as glandular features.
2. Extraglandular Manifestations (EGMs): often used to describe systemic features. It is important to know that rheumatologists vary in how they define EGM. See the Sjogren's is Always Systemic page for explanations of terms used to describe systemic Sjogren's features.
3. Glandular domain of the ESSDAI: A category of systemic disease and not caused by dryness. This is very confusing terminology because the glandular domain refers to enlargement of the major salivary gland tissue, typically the parotid glands.
Glandular Features (Sicca)
Sicca / dryness is present in the vast majority of people diagnosed with Sjogren's patients. However, 20%, and possibly more, do not have sicca when they first present (2). Not having sicca early on often results in delayed diagnosis. Even when sicca symptoms predominate, there is much more going on in the body.
The actual prevalence of clinically apparent sicca is unclear because Sjogren’s is rarely diagnosed in the early stages of disease.
Sicca is often, but not always, the first sign of Sjogren's.
Because dryness tends to come on slowly, patients may not think that these symptoms are important. Sometimes they don't notice the dryness, especially early on. Changes on dental and eye exam occur long after damage to the exocrine glands has started. Even when dryness is clinically obvious, lack of awareness by eye and dental providers frequently results in missed opportunities for timely diagnosis. Ophthalmologists treating dry eye disease often fail to consider Sjogren’s as a possible cause (34, 35).
Sicca is important but it is just one of many Sjogren's features. Because Sjogren's is always systemic, it should never be referred to as sicca syndrome.