Sjogren’s Syndrome

Sjogren syndrome is a chronic inflammatory disorder of the body’s exocrine glands in which white blood cells attack the glands and infiltrate them. It is primarily a disease of old age and mainly affects women. The destruction of exocrine glands leads to “sicca” symptoms: dry mouth, dry eyes, and dry skin with parotid gland enlargement. Extra-glandular manifestations also develop as the disease progresses.

The cause of Sjogren syndrome is not very well understood. It can occur on its own (primary disease) or in conjunction with other autoimmune disorders. In both forms, an auto-immune mechanism against exocrine glands develops and destroys these glands causing symptoms of dryness. As the disease progresses, virtually all the organs can get involved. There is some genetic predisposition for this auto-immune mechanism to occur in individuals that destroy their exocrine cells.

Sjogren syndrome can occur in isolation, also called primary Sjogren syndrome or secondary Sjogren syndrome, which occurs with other rheumatic diseases such as systemic lupus erythematosus, rheumatoid arthritis, and scleroderma. In these conditions, the clinical signs overlap, and often, it is difficult to determine if Sjogren syndrome occurred first, leading to these conditions or vice versa. Viral infections such as HIV. Epstein-Barr virus, herpes, etc., act as environmental triggers.

Sjogren syndrome is one of the most common rheumatic diseases after SLE. It occurs worldwide with around 1-2 million cases yearly. It occurs most commonly in the middle age of life during the fourth and fifth decade. The female to male ratio is 9 to 1. Risk factors for Sjogren syndrome include:

• Genetic predisposition with major histocompatibility complex (MHC) genes on exocrine cells of various glands.
• Viral infections such as HIV, EBV, herpes, hepatitis C, etc.
• Menopause as estrogen has some role in disease causation and progression
• Female gender
• Middle age (40 to 50 years)

Clinical manifestations of Sjogren syndrome include:

• Dryness of eyes
• Dryness of mouth
• Redness of tongue
• Dental caries
• Cracks at the corner of the mouth
• Parotid gland enlargement with inflammation
• Skin manifestations include dry skin, rash, and urticaria.
• Dry vagina, vaginitis, and pruritus
• Extra glandular manifestations include arthritis, arthralgia, Raynaud phenomenon, myalgias. Pulmonary disease, renal disease, anemia, leukopenia, lymphoma, neuropathy, gastrointestinal manifestations, vasculitis, and muscle pains.
• In secondary Sjogren syndrome, symptoms of the primary disease dominate.

Complications of Sjogren syndrome include:

• Recurrence of autoimmune disorders such as SLE and rheumatoid arthritis
• Infection of the parotid gland with pain, redness, and warmth
• Parotid tumors
• Complications during pregnancy include fetal loss, fetal heart block, and neonatal lupus syndrome in the newborn
• Pseudo lymphomas and non-Hodgkin lymphoma

 Sjogren syndrome is diagnosed based upon clinical signs. The diagnostic criteria are met with the presence of any three of the following four objectives:

• Ocular symptoms include dry eyes for more than three months, foreign body sensation, use of artificial tears for more than 3 months
• Ocular signs- Schirmer test without anesthesia (<5mm in 5 min), positive vital dye staining test
• Oral symptoms include dry mouth, swollen salivary glands, use of liquids to aid swallowing
• Oral signs include abnormal sialography and abnormal sialometry findings, abnormal scintigraphy finding

Serologic abnormalities can be detected by:

• Complete blood count
• ESR
• Anti-nuclear antibodies (ANAs)
• Rheumatoid factor (RF)
• Creatinine clearance
• Liver function test
• Total protein and total albumin levels
  Minor salivary gland biopsy is currently the best single test to establish the diagnosis of Sjogren syndrome. Minor salivary glands are removed from inside the lips and taken for histologic examination.

Differential diagnoses of Sjogren syndrome include:

• Sarcoidosis
• Host vs. graft disease
• Keratoconjunctivitis sicca
• Bell palsy
• Parkinson disease
• Alzheimer’s disease
• Lymphoma
• AIDS
• Rheumatoid arthritis
• Bulimia nervosa
• Chronic pancreatitis
• Scleroderma
• Tuberculosis

Currently, no cure for Sjogren syndrome exists, and only symptomatic relief can be provided to manage it.

• Skin and vaginal dryness can be treated with ointments, lotions, and lubricants. Vaginal dryness can increase the risk of developing yeast infections.
• Dryness of the eyes can also be managed with artificial tears and lubricants.
• For pain associated with joint inflammation, NSAIDs can be used. If they cannot provide pain relief, such as in patients with rheumatoid arthritis, anti-inflammatory agents such as disease-modifying anti-rheumatic drugs (DMARDs) can be used.
• Immunosuppressive agents are used in cases of major organ involvement, such as lungs.
• Antiphospholipid antibody syndrome that can develop due to Sjogren syndrome requires anticoagulants.
• Surgical intervention is usually done for diagnostic purposes where parotid gland biopsy or minor salivary gland biopsy can be done for definitive diagnosis and to rule out other causes. Lacrimal occlusion can also be corrected surgically.

Patients with primary Sjogren syndrome who do not develop any associated disorder such as SLE or rheumatoid arthritis have a good prognosis. These patients can have a normal life expectancy. Patients with infiltration of exocrine glands with lymphocytic cells can have considerable morbidity. They are at increased risk of developing non-Hodgkin lymphoma. During pregnancy, the complications can worsen that can cause fetal wastage and other disorders.

The exact mechanism by which Sjogren syndrome is caused is unknown, so there are no definitive ways of preventing it. However, once it is diagnosed, patient education is essential for self-care for symptoms such as drying of mouth, skin, eyes, vagina, etc.

• Oral hygiene is important to prevent dental infections such as caries.
• Any swelling in the face or anywhere in the body should not be ignored and assessed accordingly as it could be a sign of lymphoma.
• Pain and redness in the eyes should be evaluated as they could be due to an underlying infection.
• Compliance with prescribed treatment is important to prevent complications.