by Nathan Wei, MD, FACP, FACR
Nathan Wei is a nationally known board-certified rheumatologist and author of the Second Opinion Arthritis Treatment Kit. It's available exclusively at this website... not available in stores.
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From the American College of Rheumatology and the Arthritis Foundation
Sjögren’s disease (SD) is a chronic inflammatory autoimmune disorder characterized by infiltration of the exocrine glands (glands that secrete), by lymphocytes and plasma cells.
The classic signs of SD include enlargement of the salivary (saliva) and lacrimal (tears) glands with dryness of the mouth (xerostomia) and eyes (xerophthalmia).
The term, Sjögren’s syndrome (or sicca syndrome), refers to dryness of the eyes and mouth. This symptom complex can occur as a result of many conditions, such as medications, allergies, etc. Sjogren’s disease, on the other hand, refers specifically to the autoimmune disease.
Sjogren’s disease may either be primary- occurring by itself- or secondary to another autoimmune condition such as rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis or polymyositis.
The disease affects mostly middle-aged people, women more than men in a ratio of 9:1. However, it can be seen at all ages. In addition to the primary syndrome, 30% of patients with rheumatoid arthritis, systemic lupus erythematosus and systemic sclerosis suffer secondary Sjögren’s syndrome.
The majority of the patients with Sjögren’s syndrome have symptoms related to deficient lacrimal and salivary gland functions. The initial symptoms may be nonspecific. These include joint pains, Raynaud’s phenomenon (a condition where the fingers or toes blanch when exposed to cold), and fatigue.
Patients usually complain of dry eye symptoms, including burning, itching or foreign body (gritty, sandy) sensation and accumulation of thick, ropy secretions along the inner corner of the eye. With time, conjunctival injection, reduced visual acuity and photosensitivity develop. These symptoms, which occur because of decreased and altered tear production, result in the destruction of conjunctival cells.
Drynessof the mouth, xerostomia, is frequent. Patients usually complain of difficulty in eating dry foods, inability to speak continuously, mouth soreness, changes in taste and smell and fissures of the tongue and lips. An increase in dental caries (cavities) is due to decreased saliva volume and the loss of the antibacterial factors found in saliva.
A decrease in the mucous gland secretions of the upper and lower respiratory tract results in dry nose, throat and trachea. Mucosal gland involvement of gastrointestinal tract can be associated with dysphagia (difficulty swallowing), atrophic gastritis (atrophy of the lining of the stomach), esophageal mucosal atrophy, constipation and pancreatic insufficiency. Vaginal dryness can result in dyspareunia (painful intercourse).
Systemic features can complicate the course of Sjögren’s disease and are seen in one third of the patients. Most patients complain of easy fatigability, low-grade fever, myalgias (muscle aches) and arthralgias. An inflammatory arthritis is also common.
Dyspnea (shortness of breath) may be the presenting symptom of an underlying diffuse interstitial pneumonitis (lung inflammation) due to lymphocytic infiltration. Kidney involvement includes interstitial nephritis and defects in kidney function. Central nervous system involvement has been recognized only over the past decade and reported features include focal and diffuse defects, including multiple sclerosis, progressive dementia, cognitive dysfunction and spinal cord involvement (transverse myelitis).
Vasculitis (inflammation of blood vessels) affects small and medium sized vessels. The most common clinical features are nonthrombocytopenic purpura of the legs, skin ulceration, recurrent urticaria (hives) and mononeuritis multiplex (inflammation of large nerves). Raynaud’s phenomenon occurs in about 20% of the patients.
Malignant or pseudomalignant lymphoproliferation may be a prominent part of the illness. Lymphoma should always be suspected when persistent major salivary gland enlargement, lymphadenopathy (swollen lymph nodes) or lung nodules are noted.
Diagnosis of Sjögren’s syndrome is based on the presence of two of the three following manifestations: keratoconjunctivitis sicca (inflammation of the eyes due to dryness), xerostomia (dry mouth) and an associated connective tissue or lymphoproliferative disorder. Salivary or lacrimal gland enlargement may or may not be present.
Minor salivary gland biopsy serves as the cornerstone for diagnosis. Schirmer’s tear test is used for evaluation of tear secretion. The test is performed with strips of filtered paper slipped beneath the inferior lid of an unanesthetized eye. After 5 minutes the wetting length of the paper is measured. Wetting of less than 5 mm is a strong indication for diminished secretion. Keratoconjunctivitis sicca, the result of decreased tear production, is diagnosed with slit-lamp examination after using Rose Bengal staining of the corneal epithelium. Rose Bengal is a dye which stains the devitalized epithelium of both the cornea and conjunctiva.
Patients with primary Sjögren’s syndrome are at an increased risk of developing lymphoproliferative disorders, including non-Hodgkin lymphoma. Patients with splenomegaly (enlarged spleen), bilateral parotid enlargement, and a history of radiation treatment are at especially high risk.
The treatment of dry eyes is largely symptomatic and includes artificial tears and lubricant ointments. Occasionally, patients may require surgical punctual occlusions to block tear drainage. Managing the oral component of Sjögren’s syndrome requires the use of saliva substitutes, stimulation of salivary flow from functioning issue (pilocarpine hydrochloride or cevimeline hydrochloride) and aggressively fighting dental caries through both prevention and treatment.
Patients with severe extraglandular manifestations are usually treated with systemic corticosteroids and immunomodulatory drugs such as hydroxychloroquine, methotrexate, and azathioprine.
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