Response to antibiotics may help diagnose this autoimmune disorder and lead to proper treatment if properly differentiated from multiple sclerosis.
Difficult to See This Eye Problem
Patients are tired of the routine—another doctor visit, another lab test, another copayment—not just because of the lack of a diagnosis but because fatigue is a prominent symptom. It seems some physicians have it in their head that this illness is only in the patient’s head. This time the patient is placated with an antibiotic for which, through blurry vision, she attempts to read side effects.
What is Sjögren’s Syndrome?
A few celebrity battles have shed a dim spotlight on Sjögren's syndrome (pronounced SHOW-grins, sometimes spelled “Sjogren’s”). It increases one’s chance of developing non-Hodgkin’s lymphoma (NHL). Perhaps because of its rarity or the lack of a cure, most physicians who do not specialize in autoimmune disorders are quick to dismiss Sjögren’s syndrome – even though the thesis describing its effects was published by Swedish ophthalmologist Dr. Henrik Sjögren in 1933. Relying on blood tests alone while ignoring a body of symptoms can delay diagnosis and prevent doctors from screening for NHL.
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Sjögren’s syndrome is a systemic autoimmune disorder thought to be triggered by a virus that causes lymphocytes (white blood cells) to primarily target and attack two kinds of exocrine glands: lacrimal and salivary. Tears and saliva diminish in quantity and quality afterwards. But the lymphocytes may not stop there.
Other areas dependent upon certain fluids—joints, lungs, lymph nodes, vagina, skin, and brain can develop sicca symptoms, causing pain and/or inflammation. Such discomfort can make it more difficult for athletes, like Venus Williams, to optimally perform. Diuretics like caffeine and alcohol may exacerbate dry eyes.
Rare Sjögren’s Symptoms and Complications
- Infection of the salivary glands.
- Corneal ulcers: If not treated, this can lead to loss of vision.
- Peripheral neuropathy: Loss of sensation in fingers, hands, arms, toes, feet, legs.
- Cranial neuropathy: Loss of sensation in parts of the face.
- Kidney problems: Inflammation, disruption in body fluid balance, kidney stones and, if untreated, kidney failure.
- Pseudolymphoma: 1 in 10 Sjögren’s patients develop this condition, which can cause spleen enlargement or enlargement of lymph glands.
- Non-Hodgkin’s lymphoma: in 1 in 10 people who develop pseudolymphoma, the pseudolymphoma can progress to a lymphoma, a cancer of the lymph glands.
- Parotid gland tumors: Swelling in the cheek area.
- Recurrent miscarriage: Three or more miscarriages in a row because of a link between Sjögren’s syndrome and a condition called antiphospholipid syndrome.
- Raynaud’s phenomenon: The extremities of the body, usually the fingers and toes, temporarily discolor and may become painful usually due to exposure to the cold.
- Drug reactions: People with Sjögren’s syndrome may be more prone to developing side-effects when they take certain drugs - for example, antibiotics.
Diagnosing Sjögren’s Syndrome
Astute physicians take notice of bad drug reactions. For example, a clinical study comparing 85 primary Sjögren’s syndrome patients and 45 osteoarthritis patients revealed antimicrobial allergy was more common among Sjögren’s syndrome patients (46% vs. 27%).
Eleven Sjögren’s syndrome patients (13%), but no osteoarthritis patient had experienced at least a partial, non-allergic systemic reaction with trimethoprim. Of them five (6%) had had a full-blown systemic reaction, including both chills/fever and headache/
Dry eyes, dry cough, dental decay, and frequent respiratory infections should be investigated. More than 50% of patients with neurological manifestations of Sjögren’s may not have autoantibodies. In patients who have neuropathy and compelling glandular symptoms of dry eyes and dry mouth, negative blood tests for SS‑A and SS‑B antibodies do not exclude the diagnosis of Sjögren’s syndrome.
Because symptoms overlap, patients with Sjögren’s syndrome may erroneously receive a Multiple Sclerosis diagnosis.
Among 3297 participants, 2061 (63%) had negative anti-SSA/anti-SSB, 1162 (35%) had anti-SSA with or without anti-SSB, and 74 (2%) anti-SSB alone in a clinical study published in 2015 by Baer, et al. SS‑A/Ro, SS‑B/La, RNP, and Sm are autoantigens commonly referred to as extractable nuclear antigens (ENAs). Antibodies to ENAs are thought to be common in patients with connective tissue diseases (systemic rheumatic diseases).
Beyond blood tests for rheumatoid factor, primary physicians should perform a Schirmer’s test, among others, to measure tear output from lacrimal glands and be alert to additional symptoms that may suggest Sjögren’s syndrome that are reported by the patient. Vigilance is particularly important if there is a family history of this or other autoimmune disorders, such as rheumatoid arthritis, systemic lupus erythematosus, or scleroderma.
Oral and dermatological findings that should alert the practitioner to suspect the diagnosis of Sjögren’s syndrome include the following:
- Sensitivity to acids (68% of patients)
- Difficulty eating dry foods (66%)
- Sensitivity to spicy foods (58%)
- Higher incidence of caries
- Fissured red tongue
- Difficulty swallowing solid food without drinking water
- Frequent oral candidiasis (74%), especially angular chielitis
- Lack of salivary pool in the mouth floor
- Dry skin and rough, coarse scalp hair
- Vaginal dryness
Vitamins A, D, and K deficiencies manifest dry eyes. Neurologically, Sjögren’s should be differentiated from multiple sclerosis (MS). Myelitis and optic neuritis are syndromes can occur in patients with MS and Sjögren's syndrome. Because symptoms overlap, patients with Sjögren’s syndrome may erroneously receive a diagnosis of MS.
Because MS treatments are not only ineffective for Sjögren’s syndrome, but may actually precipitate flares of Sjögren’s disease, accurate distinction between MS and Sjögren’s syndrome is crucial to remain A Bit More Healthy. April is Sjögren’s Awareness Month.
- Sjögren’s Syndrome. health.harvard.edu
- Venus Williams opens up about incurable Sjögren’s Syndrome. dailymail.co.uk
- Sjögren’s Syndrome. patient.co.uk
- The SSB-positive/SSA-negative antibody profile is not associated with key phenotypic features of Sjögren's syndrome. Baer AN, et al. nih.gov
- Adverse drug reactions in Sjögren’s syndrome…. Antonen JA, et al. nih.gov
- SS-A and SS-B Antibodies, IgG, Serum. mayomedicallaboratories.com
- Which vitamins are good for dry eyes? medicalnewstoday.com
- Neurologic complications of Sjögren’s. hopkinssjogrens.org
- Sjögren’s Syndrome. dermatologyadvisor.com
- Photography by SixtyOne Productions licensed from iStock Photo.