History
Clinical manifestations of pediatric Sjögren syndrome may vary more than those seen in adult patients. [17] The constellation of symptoms seen in children (eg, lower frequency of sicca syndrome, higher rates of parotid enlargement, higher prevalence of immunologic markers) may be similar to those found in young adult patients (ie, < 35 y). [20, 21]
Sicca syndrome
Symptoms of keratoconjunctivitis include dry eyes with reduced tear production, gritty or sandy sensation under the lids, red eyes, and photosensitivity. Keratoconjunctivitis is less prominent in primary juvenile Sjögren syndrome. Lacrimal gland enlargement appears to be a feature in primary and secondary pediatric Sjögren syndrome. The management of keratoconjunctivitis includes the use of artificial tears and conservation of natural tear flow. [9, 10, 22]
Symptoms of xerostomia include decreased saliva production and difficulties with chewing, swallowing, and even speech; abnormality in taste and smell; dental caries; mucosal burning sensation; sensitivity to spicy and acidic foods and beverages; increased risk for oral candidiasis; hoarseness of voice, and dysphonia (common in adults). Recurrent parotitis appears to be the most common oroglandular manifestation in pediatric populations. [9, 10]
Musculoskeletal symptoms
Symptoms include the following:
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Arthralgia (often noninflammatory), morning stiffness, and nonerosive arthritis
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Myalgia and muscle weakness
Cutaneous findings
Symptoms include the following:
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Raynaud phenomenon
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Nonthrombocytopenic purpura, especially of lower extremities
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Nasal, vaginal, and cutaneous dryness
Gastrointestinal symptoms
Symptoms include the following:
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Dysphagia, nausea, and epigastric and abdominal pain
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Achalasia (in children)
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Achlorhydria and chronic atrophic gastritis (adult patients)
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Primary biliary cirrhosis
Pulmonary findings
Symptoms include the following:
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Dyspnea due to mild interstitial disease
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Dry cough
Renal symptoms
Symptoms include the following:
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Interstitial nephritis
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Renal tubular acidosis [23]
Additional findings
The following symptoms may also be seen in Sjögren syndrome:
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Fatigue
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Depression
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Insomnia
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Cognitive impairment
Physical Examination
The following may be noted on physical examination:
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Upper lid prominence due to lacrimal gland enlargement
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Corneal ulceration, vascularization, and uveitis
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Vasculitic lesions - Purpura and erythema nodosum
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Lymphadenopathy
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Autoimmune thyroiditis
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Nervous system manifestations - Peripheral sensorimotor neuropathy; central nervous system (CNS) disorders such as cognitive impairment, movement disorder, transverse myelopathy, encephalopathy, aseptic meningitis, dementia, optic neuropathy, and cranial neuropathies (in both adult and pediatric patients)
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Musculoskeletal manifestations - Intermittent synovitis, chronic nonerosive oligoarticular or polyarthritis (Jaccoud arthropathy has been observed in adults), and myalgias
Oral cavity manifestations of Sjögren syndrome may include the following:
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Poor or absent pooling of saliva underneath the tongue.
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Mild erythema and thinning of the mucosa
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Dental caries
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Traumatic erosions and ulcers, angular cheilitis, and chapped lips
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Frothy, ropey, and thickened saliva
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Erythema, fissuring, coating, and depapillation of the dorsal tongue
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Halitosis
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Gingivitis/periodontitis
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Superficial mucoceles
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Lower facial appearance of a 14-year-old adolescent girl with Sjogren syndrome. She exhibits both parotid and submandibular gland enlargement and chapped lips.
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Intraoral view of a 14-year-old adolescent girl with Sjogren syndrome. Hyposalivation results in erythema of the mucosa, gingivitis, decalcification or white spot lesions of the teeth at the cervical margin, and dental caries with extensive restorations of the posterior teeth.
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Erythema of the labial mucosa with enlargement of the minor salivary glands and superficial mucoceles.
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The dorsal surface of the tongue demonstrates generalized atrophy of the filiform papillae, mild fissuring, and median rhomboid glossitis.
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A 14-year-old adolescent girl with Sjogren syndrome with painful unilateral swelling of the knee and hyperpigmentation of the overlying skin.
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The dorsal tongue demonstrates hyperplastic candidiasis with focal erosions and a brown hairy tongue. Ulcerated fissures are observed on the corners of the mouth that represent angular cheilitis.
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Biopsy of the minor salivary glands of the lower lip may be useful in the diagnosis of Sjögren syndrome. A 1.5- to 2-cm incision of normal-appearing mucosa allows for the harvesting of 5 or more salivary gland lobules.
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Low-power photomicrograph of a minor salivary gland lobule showing multiple lymphocytic foci that are replacing the acinar structures (hematoxylin-eosin, 40 X).
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Intermediate-power photomicrograph demonstrating a chronic inflammatory aggregate of more than 50 lymphocytes and plasma cells with a periductal pattern. The inflammatory focus is adjacent to normal appearing acini (hematoxylin-eosin, 200 X).
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High-power photomicrograph of the chronic inflammatory aggregate consists of lymphocytes and plasma cells around a ductal structure (hematoxylin-eosin, 400 X).
