Introduction to Sjogren's Syndrome
Signs and Symptoms of Sjogren's Syndrome
- Discharge or secretions from the eyes due to abnormal mucus production
- Feeling a film or lack of acuity in the visual field
- Corneal ulceration
- Blurred vision
Some patients have reported that ocular symptoms associated with Sjogren's syndrome are often exacerbated by:
- Low humidity
- Dry climate
- Exposure to cigarette smoke
- Anticholinergic drugs
Systemic Manifestations
Sinus Symptoms
Nasal or sinus symptoms are attributable to Sjogren's syndrome partly because up to 50% of patients experience atrophy of the mucus lining of the nose. Symptoms include:
- Nasal crusting
- Epistaxis (nose bleeds)
- Perforation of the septum
- Hyposmia (diminished sensitivity to smell)
- Hypogeusia (diminished sensitivity to taste)
Musculoskeletal Symptoms
Approximately 55% of patients with primary Sjogren's syndrome experience musculoskeletal symptoms including:
- Arthralgia (non-inflammatory joint pain) or arthropathy (diseases of the joint) that occurs in up to 50% of patients
- Arthritis (inflammatory joint pain) is thought to occur in at least 30% of patients with Sjogren's syndrome and tends to be relapsing and remitting. Some patients exhibit signs of arthritis 5-10 years before they are diagnosed with Sjogren's syndrome. Arthritis tends to occur symmetrically on the right and left sides.
- Myositis - inflammation of muscle tissue
- Myalgia - noninflammatory muscular pain - it is estimated that up to 20% of patients with Sjogren's syndrome suffer from fibromyalgia
- Muscle weakness
Fatigue
One of the most debilitating systemic features of Sjogren's syndrome is fatigue which many individuals with Sjogren's syndrome describe as more distressing than any other symptom. Fatigue occurs in approximately 50-70% of patients with Sjogren's syndrome.
Sleep disorders are also common in patients with Sjogren's syndrome which may be a factor in fatigue. Patients report that they sleep but do not feel well rested when they wake up. Some clinicians are of the opinion that the excessive fatigue experienced by these patients may be related to subclinical hypothyroidism which is also associated with Sjogren's syndrome.
Cutaneous Symptoms
Estimates of the percentage of Sjogren's syndrome patients who experience skin problems range from 10-40%. Cutaneous vasculitis (inflammation of the blood vessels in the skin) is one of the most characteristic extraglandular manifestations of Sjogren's syndrome and is thought to be due to lymphocytic infiltration into the walls of the blood vessels. Typically, small blood vessels are affected more than large ones.
The most common forms of cutaneous vasculitis seen in Sjogren's syndrome patients (typically patients who have anti-Ro and anti-La antibodies) are:
- Palpable or nonpalpable purpura (purple spots on the skin after blood "leaks" underneath; similar to a bruise) which can cause raised, red skin lesions
- Urticarial lesions (hives)
- Erythematosus micropapules - red spots
Patients with cutaneous vasculitis also may develop non-vasculitic lesions including:
- Petechiae - pinpoint dots
- Photosensitive cutaneous lesions - lesions which are sensitive to light (also seen in patients with systemic lupus erythematosus)
- Livedo reticularis - marbled appearance of the skin
- Lichen planus - small itchy pink or purple spots on arm and/or legs
- Thrombocytopenic purpura - purple areas on the skin related to a decrease in blood platelets
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