Do Doctors Have a Clue You Have Sjögren’s?

Your reaction to treatment may help diagnose the disorder.

By Kevin RR Williams

HEALTH You're 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 physicians have it in their head that this illness is in yours. This time you're placated with an antibiotic for which, through blurry vision, you try to read side effects you are quite likely to experience.

Since Sjögren's syndrome increases one's chance of developing non-Hodgkin's lymphoma (NHL), it is an appropriate ailment to discuss during National Breast Cancer Awareness month. A few celebrity battles have shed a dim spotlight on Sjögren's syndrome. Perhaps because of its rarity or the lack of a cure, most physicians who don't 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 over 80 years ago, in 1933. Relying on blood tests alone while ignoring a body of symptoms can delay diagnosis and prevent doctors from screening for NHL.

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 other organs can develop sicca symptoms, causing pain and/or inflammation. Such discomfort can make it more difficult for athletes, like Venus Williams, to optimally perform.

Rare Sjögren's Symptoms and Complications

  • Infection of the salivary glands.
  • Corneal ulcers: if not treated, this can lead to loss of vision.
  • Pancreatitis: noticed by severe pain in the upper abdomen.
  • 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/backache and at least one of the following: malaise, vomiting, dizziness, confusion or meningeal irritation. Hence, such reactions to pharmaceutical antimicrobials can assist doctors in diagnosing Sjögren's syndrome.

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. 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.

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 of this symptomatic 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.

Tags: ophthalmology, primsol, proloprim, rheumatology, trimpex, vertigo

References
  1. Venus Williams opens up about incurable Sjögren's Syndrome. dailymail.co.uk
  2. Sjögren's Syndrome. patient.co.uk
  3. Adverse drug reactions in Sjögren's syndrome…. Antonen JA, et al. nih.gov
  4. Neurologic complications of Sjögren's. hopkinssjogrens.org
  5. Photography by SixtyOne Productions licensed from iStock Photo.