Sjogren Syndrome is one of the most common autoimmune disorders, characterized by the body's immune system attacking its own exocrine glands, especially the lacrimal and salivary glands. As a result of reduction of tears and saliva, all affected patients display a characteristic form of dry eyes and mouth. Other clinical features of this condition include aqueous tear deficiency, dental cavities, enlarged parotid glands, fatigue due to lack of sleep, difficulty in swallowing, hoarseness, skin rashes, vaginal dryness, and dry cough. Apart from moisture glands, the disorder may also affect the joints, muscles, nerves, thyroid, and/or kidneys, resulting in arthritis, myositis, neuropathy, thyroiditis, and/or nephritis. Most patients are highly sensitive to spicy foods and alcohol, and are also highly susceptible to oral infections, especially with yeast. The syndrome can present itself in isolation (primary Sjogren syndrome), or in association with any of the other rheumatic autoimmune disorders, such as SLE or rheumatoid arthritis (secondary Sjogren syndrome).
The overall prevalence of Sjogren syndrome is estimated at 1 in 5,000 individuals. The condition is mostly seen in women over 40 years of age. In fact, women are 9 times more likely than men to have this disorder.
A battery of tests and examinations may be performed to diagnose the condition. These include the Schirmer test to assess the working of the tear glands, slit lamp examination to see the extent of dryness in the eye, mouth examination for signs of salivary gland swelling or dental cavities, and the most important of all, a lip biopsy to confirm the diagnosis. Patients' blood can also be checked for anti-thyroid antibodies, rheumatoid factors, antinuclear antibodies, and most importantly, anti-SS-A and anti-SS-B antibodies that are known to occur commonly in Sjogren syndrome. As of now, there is no cure for the condition. Treatment is only symptomatic in nature, and includes moisture therapy, use of non-steroidal anti-inflammatory drugs, and other immunosuppressive medications. Prognosis for the condition varies from one individual to another. The most seriously affected patients have to manage life-long fatigue, joint pain, blurred vision, and oral infections.
Nampoory et al. (1998) described two unrelated patients with Sjogren syndrome in Kuwait, in both of whom the condition resulted in renal tubular acidosis and severe hypokalemia leading to quadriplegia. The patients were 15 and 52-year old females. Both presented with severe muscle weakness and an inability to move. The onset of this paralysis was preceded by only a few hours of painful cramps. Laboratory investigations revealed severe hypokalemia, severe acidosis, and alkaline urine, leading to diagnoses of RTA with severe hypokalemia leading to paralysis, which was treated with bicarbonate and potassium supplements. Serological analysis revealed the presence of antinuclear antibody, and SS-A/Ro and SS-B/Ro precipitin in both patients, and C-reactive protein in the younger patient. Schirmer's test was positive, and parotid glands were inflamed. Renal biopsy performed in the younger patient showed the presence of interstitial nephritis. A diagnosis of Sjogren syndrome was made based on the Copenhagen and European preliminary classification criteria.
Al Tamimi (1999) evaluated the use of salivary gland ultrasonography as a means of diagnosing SS in a group of patients with arthritis (n=45), other connective tissue disorders, and primary SS (n=10), and 25 healthy individuals. Normal salivary glands were found to be homogenous, whereas those involved with SS were heterogeneous due to the presence of diffusely scattered focal hypoechogenic areas. SS was classified as definite (60% in RA patients and 10% in patients with other connective tissue disorders) or early (20% in RA patients and 25% in patients with other connective tissue disorders) based on the degree of this heterogeneity. Al Tamimi (1999) concluded that salivary gland US was a reliable screening method for the detection and monitoring of salivary gland involvement in SS.
[Al Tamimi M. Salivary gland ultrasonography as a screening method for Sjogren's syndrome. Kuwait Med J. 1999; 31(1):60-5.]
Hammoudeh and Mahdi (2005) reviewed the clinical and laboratory findings of all patients diagnosed with primary Sjogren syndrome in Qatar between 1977 and 2002. A total of 18 patients (16 females, 2 males) were found to meet the criteria for the condition. Of this group, 10 were Qatari nationals. Mean age at diagnosis was 40.6 years, while the average period between onset of symptoms and diagnosis was 2.3 years. This period was significantly shorter compared to those from studies in other countries. Hammoudeh and Mahdi (2005) suggested that this could be due to the free healthcare system and free access to subspecialty centers in Qatar. The most common clinical features were dry eyes and mouth (66%), joint pain (61%), fatigue (44%), skin rash of vasculitic nature (22%), and parotid swelling (18%). Less commonly seen features included renal tubular acidosis (3 patients), hypokalemic muscle weakness (2), elevated liver function tests (3), hypothyroidism (2), trigeminal neuralgia (1), peripheral neuropathy (1), and Non-Hodgkin's lymphoma (1). Laboratory investigations revealed elevated antinuclear antibosies in 83%, positive rheumatoid factor in 83%, SS-A antibodies in 71%, and SS-B antibodies in 50% of the patients. Hammoudeh and Mahdi (2005) concluded that findings of the disease in Qatar were similar to those reported from Western countries.
[Hammoudeh M, Mahdi S. The clinical and immunological picture of primary sjogren's syndrome in Qatar. Qatar Med. J. 2005; 14(1): 41-3.]
Elagib et al. (1999) characterized and sequenced the variable (V) region genes of the light (L) chains of 10 immunoglobulin (IgM) rheumatoid factor (RF) monoclonal antibodies (MoAb) derived from the peripheral blood of patients with primary Sj?gren's syndrome (pSS). Six out of 10 RFs used lambda (lambda) L chains, while four RFs used kappa (kappa) L chains. Five out of the six lambda RFs were encoded by Vlambda3 gene segments, the sixth one was encoded by a Vlambda1 gene segment. Elagib et al. (1999) commented that this preferential utilization of the Vlambda3 family genes suggests selective expansion of the B cell in pSS. Three of the kappa RFs used Vkappa3 gene segments, while the fourth used a Vkappa2 gene segment. Half of the RFs were found as unmutated copies of their closest germline (GL) gene. Interestingly these RFs were previously shown to use heavy (H) chains in GL gene configuration. Three RFs have very few mutations (2-3) and only two RFs have substantial numbers of mutations (6 and 11). This also correlated with the number of mutations in the respective H chains.
Rajani et al. (2013) reported a 50-year old woman with primary Sjogren syndrome. She presented with severe pulmonary artery hypertension and pericardial effusion, and also suffered from cardio-pulmonary system involvements. Immunosuppressive agents and other therapies for pulmonary hypertensions were used for managing her condition.