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Health Protocols

Sjögren Syndrome

Signs And Symptoms

“Sicca syndrome” is the term used to describe the main clinical features of Sjögren syndrome: dry eyes (keratoconjunctivitis sicca) and mouth (xerostomia). Sjögren syndrome can affect other lubricating secretions as well, resulting in dryness in the throat, nose, skin, and vagina (Table 1).

Chronic inflammation and other aspects of Sjögren syndrome can further lead to involvement of organs and tissues throughout the body including joints, lungs, kidneys, liver, stomach, and nervous system (Table 2).

Table 1. Signs and Symptoms of Sjögren Syndrome – Sicca Features

 

Eyes

Mouth

Other

Symptoms

sensation of dry eye

sandy/gritty, itchy, burning sensation

redness; blurred vision; eye fatigue

mucinous discharge, “filmy” sensation

intolerance of contact lenses; need for frequent eye drop use

dry mouth/lips

need to drink fluids often to help swallow food

difficulty chewing/swallowing dry foods

difficulty speaking continuously

sore/burning sensation in mouth

difficulty wearing dentures

decreased saliva production

distortion of taste

dry nose, throat, trachea causing dry cough or hoarseness

symptoms of gastritis or pancreatitis

dry skin

vaginal dryness

painful intercourse

Signs

inflammation of cornea

red eye

absent or cloudy saliva

dry, reddened tongue and oral mucosa

sticky oral mucosa

fissures of the tongue

periodontal disease

increased risk of dental cavities

parotid (salivary) gland inflammation or enlargement, with possible fever, tenderness, redness, or infection

oral candidiasis (yeast infections)

halitosis (bad breath)

skin rashes

photosensitivity

recurrent hives

skin ulcerations

low stomach acid

(Rischmueller 2016; Hajj-ali 2013; Catanzaro 2014; Ichikawa 1981; D'Souza 2014; Pokorny 1991)

Table 2. Extraglandular Manifestations of Sjögren Syndrome

General

  • -Fatigue
  • -Low-grade fever, night sweats, weight loss, fatigue

Lungs

  • -Inflammation and fibrosis of the lungs
  • -Thickening or inflammation of the walls of the bronchial airways

Musculoskeletal

  • -Multiple joint pain
  • -Muscle inflammation
  • -Muscle weakness

Kidneys and Urinary Tract

  • -Inability to produce concentrated urine
  • -Systemic acidosis with high urine pH
  • -Weakness caused by potassium loss
  • -Urinary urgency
  • -Kidney stones

Liver

  • -Enlarged liver
  • -Autoimmune hepatitis

Neurologic

  • -Peripheral neuropathy
  • -Brain white matter lesions
  • -Lesions that resemble multiple sclerosis
  • -Pain or tingling sensation (paresthesia)

Skin and Vascular

  • -Dry skin related to nervous system impairment
  • -Venous blood clots

Gastrointestinal

  • -Acid reflux
  • -Gastritis
  • -Symptoms of irritable bowel syndrome (eg, abdominal pain, diarrhea, constipation, bloating)

(Mariette 2016; Rischmueller 2016; Tobon 2012; Kurien 2017; Kim-Lee 2015; Stalenheim 1997; Vrethem 1990; Colafrancesco 2015; Aasarod 2000; Arman 2017; Kaplan 2002; Chung 2014; Volter 2004; Catanzaro 2014)

Sjögren Syndrome and Risk of Lymphoma

The risk of non-Hodgkin’s lymphoma in people with primary Sjögren syndrome is 10 to 15 times higher than in the general population (Ferro 2016; Nezos 2015). Approximately 5% of primary Sjögren patients develop lymphoma, and most cases are B-cell non-Hodgkin’s lymphoma (Barone 2016).

The reasons for increased lymphoma risk in those with Sjögren syndrome are not precisely known. However, more severe Sjögren syndrome appears to be associated with greater lymphoma risk (Baecklund 2014; Yadlapati 2016).

Most non-Hodgkin’s lymphoma in Sjögren patients develops as mucosa-associated lymphoid tissue (MALT) lymphoma. This type of lymphoma frequently affects organs where Sjögren syndrome is active, especially the salivary glands. Other mucosal surfaces can be affected, including those in the eyes, stomach, and lungs (Rischmueller 2016; Mariette 2016).

Sjögren patients should be carefully evaluated and monitored for lymphoma. Recurrent or persistent swelling of the salivary glands should be brought to the attention of a healthcare provider right away (Andola 2016; Jonsson 2012). Other signs and symptoms include unexplained fever, night sweats, fatigue, unexplained weight loss, itchy skin, and reddened areas on the skin (NIH 2016a; Ferro 2016; Aktan Kosker 2013).

More information is available in the Lymphoma protocol.