musculoskeletal and connective tissue disorders
Sjögren Syndrome
Diagnosis of Sjögren Syndrome
Clinical criteria Eye and salivary gland testing Autoantibodies Sometimes salivary gland biopsy Sjögren syndrome should be suspected in patients with gritty or dry eyes or dry mouth, enlarged salivary glands, peripheral neuropathy, purpura, or unexplained renal tubular acidosis. Such patients should receive diagnostic tests that can include evaluation of the eyes and salivary glands and serologic tests. ; Diagnosis references ). Not every patient who receives a clinical diagnosis of Sjögren syndrome fulfills the proposed criteria, but the criteria provide useful guidance for evaluation and are applied to patients who have at least 1 symptom of eye or oral dryness: Eye symptoms:≥3 months of daily, persistent, troublesome dry eyes, recurrent sensation of sand or gravel in the eyes, or use of tear substitutes≥3 times a day Oral symptoms:>3 months of daily dry mouth sensation or daily use of liquids to aid in swallowing dry food To fulfill the criteria, patients must have at least 1 symptom of eye or oral dryness, must also have a score of ≥ 4, and must not have any of the following exclusion criteria: History of radiation treatment to the head and neck Active hepatitis C infection (confirmed by polymerase chain reaction) AIDS Sarcoidosis Amyloidosis Graft-vs-host disease IgG4-related disease The most common causes of dry eyes and dry mouth (sicca symptoms) are aging and medications, but when parotid enlargement occurs in addition to sicca symptoms, diseases such as Hepatitis C, Chronic , 5 mm, although about 15% of test results are false-positive and 15% are false-negative. Ocular staining with an eye drop of rose bengal or lissamine green solution is highly specific. The total ocular staining score (OSS) for each eye is recorded ( Diagnosis references ). Slit-lamp examination showing a can be confirmed by abnormally low saliva production (≤ 0.1 mL/minute) as measured by salivary flow, sialography, or salivary scintiscanning, although these tests are used infrequently. Saliva production can be qualitatively evaluated by several methods ( Diagnosis references ), including looking for normal pooling of saliva under the tongue. Alternatively, a tongue blade can be held against the buccal mucosa for 10 seconds. If the tongue blade falls off immediately when released, salivary flow is considered normal. The more difficulty encountered removing the tongue blade, the more severe the dryness. In women, the lipstick sign, where lipstick adheres to the front teeth, may be a useful indicator of dry mouth. have limited sensitivity and particularly low specificity. They include antibodies to Ro (SSA autoantibodies—see Systemic Lupus Erythematosus (SLE) ) or to nuclear antigens (termed La or SSB autoantibodies), antinuclear antibodies, or an elevated level of antibodies against gamma-globulin. Rheumatoid factor is present in Histopathologyis assessed by biopsy of minor salivary glands in the buccal mucosa. Salivary gland biopsy is usually reserved for patients in whom the diagnosis cannot be established by autoantibody testing or when a major organ is involved. Histopathologic involvement is confirmed if labial minor salivary glands show multiple large foci of lymphocytes with atrophy of acinar tissue. Biopsy can be complicated by protracted dysesthesias. 1.Shiboski CH, Shiboski SC, Seror R, et al: 2016 American College of Rheumatology/European League Against Rheumatism classification criteria for primary Sjögren's syndrome: A consensus and data-driven methodology involving three international patient cohorts.Arthritis Rheumatol69(1):35–45, 2017. doi: 10.1002/art.39859 2.Whitcher JP, Shiboski CH, Shiboski SC, et al: A simplified quantitative method for assessing keratoconjunctivitis sicca from the Sjögren's syndrome international registry.Am J Ophthalmol149(3):405–415, 2010. doi: 10.1016/j.ajo.2009.09.013 3.Navazesh M: Methods for collecting saliva.Ann N Y Acad Sci694:72–77, 1993. doi: 10.1111/j.1749-6632.1993.tb18343.x 