A 59-year-old woman with a 7-year history of Sjögren’s syndrome treated symptomatically has had three episodes of cutaneous vasculitis, successfully treated with oral steroids. She now presents with complaints of a slowly progressive enlargement of her right parotid gland. Her physical exam reveals dry mucous membranes and an enlarged, firm, non-tender right parotid gland.
Laboratory data reveal low C4 levels, mixed monoclonal cryoglobulinemia, positive ANA, rheumatoid factor and anti-Ro/SSA antibodies. Her CBC is normal.
Key Supporting Information
Sjögren’s syndrome, characterized by keratoconjunctivitis sicca (dry eyes) and xerostomia (dry mouth), is a slowly progressive autoimmune condition that affects the exocrine glands. It also can be associated with systemic lupus erythematosus (SLE), rheumatoid arthritis (RA) or mixed collagen vascular disease. The condition is more common in women older than 40 years, with a female-to-male ratio of 9:1, and can improve with time with symptomatic care. This condition can have multisystem manifestations involving the thyroid, kidneys, liver, lungs and vascular system.
This disorder affects 0.3% to 0.6% of the U.S. population, with an incidence of 3.9/100,000. Approximately 5% of patients with RA and 15% of patients with SLE have manifestations of Sjögren’s syndrome.
Symptoms result from immune complex mediated inflammation as well as lymphocyte infiltration of epithelial tissue. The disorder is associated with distinct HLA-DQ and HLA-DR alleles, as well as B-cell activation with polyclonal gammopathy.
The clinical manifestations depend on the organ system involved, with dry eyes resulting in corneal ulcers, blurred vision, gritty sensations and corneal irritation. Dry mouth can result in altered sensation, oral candidiasis, dental caries and impaired digestion. One-third of patients may have parotid gland enlargement, while vaginal dryness, dry skin, and persistent cough also have been described. Lymphadenopathy occurs frequently, and 5% of patients may develop B-cell lymphoma, with mucosa-associated lymphoid tissue (MALT) being the most common.
Dry eye disease (DED) is a common, yet frequently underdiagnosed, clinical condition that has challenging etiology and management for health care professionals. DED has only recently been acknowledged as a public health concern. Advances in our understanding of the disease have been made during the past few years in areas of epidemiology, pathogenesis, clinical manifestation, and therapy. It is critical that clinicians keep up to date with the current literature and the most recent guidelines for diagnosis and treatment.
Dry eye prevalence is estimated to range between 5% to 30% of the population older than 50 years, and it is one of the most common reasons for ocular surface disease (OSD). Dry eye is a multifactorial condition involving ocular irritation and inflammation in cascades giving origin to symptoms and to the pathogenesis of the disease. Changes in tear composition, including increased tear osmolarity and increased or altered expression of cytokines, chemokines, metalloproteinases, and T cells in the conjunctiva, are responsible for the symptoms of irritation, ocular surface epithelial disease, and altered corneal epithelial barrier function in individuals with dry eye.
Although the American Academy of Ophthalmology (AAO) Preferred Practice Pattern for dry eye is widely used, a recent study has found conformance to certain elements of the guidelines varies significantly. According to a study conducted at Duke University, 77.3% of the initial physical examination key elements, 67.9% of patient education key elements, 66.4% of the initial history key elements, and 40% of care management key elements were documented. Subspecialty ophthalmologists’ physical exam scores were higher than comprehensive ophthalmologists’ scores.
Although the guidelines are detailed, there is still room for improvement and further clarification. For example, while the guidelines are helpful in initiating treatment, the stepwise approach may not be best for every patient.
New diagnostic modalities continue to surface. Clinicians should track not only individual symptoms but also a more quantifiable “dry eye symptom load.” Therefore, questionnaires that generate a numerical symptom score may aid the process. The Ocular Surface Disease Index (OSDI) and the Standard Patient Evaluation of Eye Dryness (SPEED) are questionnaires known to be effective in diagnosing dry eye.