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Table 35.1 Non-sicca manifestations suggestive of Sjögren syndrome
(a)Clinical features
Chronic fatigue
Fever of unknown origin Leukocytoclastic vasculitis
Parotid or submandibular gland swelling Raynaud phenomenon
Peripheral neuropathy Pulmonary fibrosis
Mother of a baby born with congenital cardiac block
(b)Analytical features
Elevated erythrocyte sedimentation rate Hypergammaglobulinemia Leukopenia, thrombocytopenia
Serum and/or urine monoclonal band
Positive antinuclear antibodies or rheumatoid factor in an asymptomatic patient
findings, ocular or oral test results, and immunological assays are frequently unclear. Risk management under these circumstances is a key feature of primary care. Having excluded an immediately serious outcome, the appropriate decision may be to review at a later time and await further developments (if any) [7].
35.3Symptoms That Aid Early Identification
A variety of clinical and laboratory features may indicate undiagnosed SjS (Table 35.1). Many SjS patients present with sicca symptoms, but the variability in SjS presentations partially explains the fact that delays in diagnosis of many years after the onset of symptoms are common in this condition [8]. The disease may occur at all ages but typically has its onset in the fourth to sixth decades of life. When sicca symptoms appear in a previously healthy person, the syndrome is classified as primary SjS. When sicca features are found in association with another systemic autoimmune disease, most commonly rheumatoid arthritis (RA), systemic sclerosis (SjSc) or systemic lupus erythematosus (SLE), it is classified as associated SjS.
35.3.1Keratoconjunctivitis Sicca
Dry eyes are the most prominent ocular manifestation of SjS, has a long differential diagnosis (Table 35.2). Symptoms of dry eye, which include sensations of pruritus, grittiness, or soreness, may occur when the appearance of the eyes is normal. Other
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Table 35.2 Causes of dry eyes
•Allergic conjunctivitis: burning eyes, mucoid secretion, and conjunctival erythema
•Blepharitis: eyelid margins are erythematous and thickened with crusts and debris within the lashes; usually worse in the morning and improves as the day goes on; does not respond to lubricant drops
•Blepharospasm: uncontrolled blinking due to an increased local neural reflex circuit
•Iritis/uveitis: in most cases associated with pronounced photosensitivity
•Herpetic keratitis: generally with ophthalmic distribution of shingles
•Environment: dryness caused by prolonged exposure to low humidity, dust, or sun
•Lifestyle: dryness caused by diminished blinking during long periods of reading, driving, or computer use
•Medications: diuretics and anticholinergic medications, including treatments for Parkinson disease, Alzheimer disease, multiple sclerosis, depression, allergic rhinitis, and incontinence
•Rosacea: ocular symptoms (e.g., itchy, burning, dry eyes with eyelid swelling and erythema) occur in 50% of patients with rosacea
Table 35.3 Causes of dry mouth
•Diabetes: dryness worsens with poor glycemic control
•Periodontal gingivitis
•Head and neck radiation: external beam radiation damages salivary glands
•Hepatitis C: sialadenitis results in dry mouth in 15% of persons with hepatitis C
•HIV infection: Oral candidosis, and HIV-related salivary gland disease exhibits clinical manifestations similar to Sjögren syndrome
•Medications: the same that cause dry eyes
•Obstructed nasal passages: dryness caused by mouth breathing
•Sarcoidosis: decreased salivary flow results from non-caseating granulomas in salivary glands
ocular complaints include photosensitivity, erythema, eye fatigue or decreased visual acuity, and the sensation of a film across the visual field [9]. Environmental irritants such as smoke, wind, air conditioning, and low humidity can exacerbate ocular symptoms. Diminished tear secretion may lead to chronic irritation and destruction of the corneal and bulbar conjunctival epithelium (keratoconjunctivitis sicca). In addition, because tears possess an inherent antimicrobial activity, SjS patients are more susceptible to ocular infections such as blepharitis, bacterial keratitis, and conjunctivitis. Severe ocular complications of SjS include ulceration, vascularization, and opacification of the cornea [10].
35.3.2Xerostomia
Xerostomia, a key feature in the diagnosis of primary SjS, occurs in more than 95% of patients [11, 12]. However, a broad differential diagnosis must be considered in the evaluation of dry mouth symptoms (Table 35.3). Dry mouth lasting
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longer than 3 months is one of the sicca symptoms reported most often. In the early stages of SjS, the mouth may appear moist. As the disease progresses, the usual pooling of saliva in the floor of the mouth becomes absent and lines of contact between frothy saliva and the oral soft tissue are seen. The surface of the tongue becomes red and lobulated, with partial or complete depapillation. Early restoration of salivary function can relieve dry mouth symptoms and prevent or slow the progression of oral complications, including dental caries, oral candidiasis, and periodontal disease. Reduced salivary volume interferes with basic functions such as speaking or eating, and the lack of saliva components (lysozyme, lactoferrin or lactoperoxidase) that inhibit bacteria and fungi may accelerate infections by Streptococcus mutans and Candida albicans. Dental care utilization is significantly greater among patients with pSjS compared with control subjects [13].
35.3.3Systemic Dryness
Reduction or absence of respiratory tract glandular secretions can lead to dryness of the nose, throat, and trachea, resulting in persistent hoarseness and chronic, nonproductive cough. Involvement of the exocrine glands of the skin also leads to cutaneous dryness. In female patients with SjS, dryness of the vagina and vulva may result in dyspareunia and pruritus [14].
35.3.4Extraglandular Manifestations
Patients with primary SjS often present with general symptomatology that includes fever, generalized pain, fatigue, weakness, sleep disturbances, and anxiety and depression, which may have a much greater impact on the quality of life of patients than do sicca features. Fatigue occurs in 50% of patients with pSjS, and fibromyalgia has been reported in 22% [15]. Joint disease in pSjS is typically an intermittent, polyarticular problem that affects small joints in an asymmetric manner. Arthralgias exist in 48% of patients and arthritis in 15% [2]. Dry skin, another exocrine manifestation of SjS, affects 55% of patients.
Two major forms of vascular disease occur in SjS: Raynaud phenomenon, which affects 20–30% of patients; and vasculitis of smallor medium-sized vessels, which affects 10%. Up to 30% of patients with SjS have subclinical pulmonary disease. Cough, the main manifestation of respiratory tract disease, is usually a symptom of xerotrachea. Other systemic involvement includes esophageal dysmotility, tubulointerstitial nephritis, and a peripheral neuropathy with principally sensory features.