Sjögren Syndrome

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Introduction

Sjögren’s syndrome is a human autoimmune disease affecting 4 million Americans today, which causes disruption in the exocrine glands (Mayo, Rhodes). Salivary and lacrimal glands are among the most adversely affected, which leads to characteristic symptoms such as dry mouth (xerostomia) and dry eyes (keratoconjunctivitis sicca, or KCS) (Trujillo). In turn, xerostomia and KCS lead to high susceptibility to opportunistic pathogens such as Candida albicans and Pseudomonas aeruginosa (Mun, Anil). Until recently, limited public awareness has prevented focused research and treatments efforts (Rhodes).

History

Figure 1. Dr. Henrik Sjögren around the age of 35.

The first known documented case of Sjögren’s syndrome was in 1888 in London, England. It is theorized that the 65 year old woman had Sjögren’s syndrome because she presented with dry eyes and xerostomia (Parke). As these cases became more prevalent in the early 1900s, Johann von Mikulicz-Radecki determined that any patient with enlarged salivary and lacrimal glands would be diagnosed with Mikulicz’s disease. However, there are many different disorders that present with enlarged salivary and lacrimal glands, so only patients with chronic inflammation of these exocrine glands were diagnosed with Miculicz’s disease (Parke). After this change, incidences of patients with the specific subset of symptoms associated with Sjögren’s syndrome were documented, but the symptoms were not characterized until 1930 (Parke).

In 1930, a Swedish ophthalmologist by the name of Henrik Sjögren performed a study on a series of patients presenting with these symptoms (Fig. 1). Ultimately, Sjögren published a thesis describing the characteristics of the disease in 19 females, describing the syndrome as “keratoconjuctivitis sicca” (Parke). Interestingly, during this time, many other physicians had written and published papers describing this unique set of symptoms, but Sjögren had the most widely accepted thesis. Additionally, there was widespread confusion regarding the difference between Mikulicz’s disease and Sjögren’s syndrome, but it was ultimately decided that the two conditions are the same. Because of this, the syndrome carries Sjögren’s namesake still today (Parke).

Overall, the history of Sjögren’s syndrome can be divided into three prominent subsections. First, the basic characterization of the symptoms of Sjögren’s syndrome. Next, the mechanistic characterization of the associated immune system phenomena. Finally, beginning in the 1980s, a deeper understanding of the overall process of the disease and related symptoms through the expanding field of molecular biology (Parke).

Symptoms

Figure 2. Tongue of a patient with Sjögren's syndrome.

Inflamed lacrimal and salivary glands are the most common symptoms of Sjögren’s syndrome (Mayo). This, in turn, can create a wide variety of secondary symptoms. The inflammation of lacrimal and salivary glands prevents the lubrication of the mouth and eyes, which gives rise to KCS and xerostomia (Mayo). This dryness can be extreme, in some cases causing the tongue to look like crocodile skin (Fig. 2) (Parke). Other prominent symptoms which result from limited bodily lubrication include vaginal dryness, dry skin, skin rashes, and persistent dry cough (Mayo).

Additionally, symptoms resulting from a weakened immune system include joint pain and fatigue (Mayo). A weak immune system causes a general weakening of the body, which has been found to damage multiple tissues in the human body, including the kidneys, thyroid, liver, lungs, and nerves (Mayo).

Causes

Figure 3. Lymphocytic infiltration in bronchial glands. Lymphocytes are dyed dark purple, and infiltration rate increases from grade 0 to grade 3.

Diagnosis

Figure 4. Image showing the location of many symptoms of Sjögren's syndrome.

Complications

Figure 5. A patient with oral thrush.

Microbial Consequences

Yeast Infection

Figure 6. Scanning EM photo of Lactobacillus paracasei.

Dry Eyes (keratoconjunctivitis sicca)

Figure 7. Scanning EM photo of Pseudomonas aeruginosa.

Dry Mouth (xerostomia)

Figure 8. Scanning EM photo of Candida albicans.

Public Awareness

Figure 9. Venus Williams during a tennis match.

References

Authored for BIOL 238 Microbiology, taught by Joan Slonczewski, 2023, Kenyon College