Glandular (10) | |
|
Oral dryness (7) | 90 |
Difficulty chewing or swallowing, sore mouth Oral infections and dental caries, fungal infections (pseudomembranous or erythematous mucosal lesions, fissured tongue, atrophy of filiform papillae and angular cheilitis)
|
Parotid gland enlargement (7) | 50 |
|
Ocular dryness (7) | 95 |
Sandy feeling or itchiness of the eyes Reduced tear secretion, chronic irritation and destruction of the corneal and bulbar conjunctival epithelium (keratoconjunctivitis sicca)
|
Dry skin | ~10 |
|
Dryness of the upper respiratory tract (7) | 20 |
Dry nasal mucosa, xerotrachea, bronchitis sicca Chronic dry cough, shortness of breath (xerotrachea/bronchitis sicca)
|
Dyspareunia (11) | 40 |
|
Extraglandular (systemic) |
Nonspecific |
Musculoskeletal (7) | 70 |
Jaccoud arthropathy (reversible hand deformities, absence of erosive lesions); (7), (25), (26) radiographs of the hands show soft-tissue calcification (especially in association with Raynaud phenomenon) (12)
|
Raynaud phenomenon (12) | 30 |
|
Fatigue (13) | 70 |
|
Periepithelial |
Bronchial (7) | 20 |
Peribronchial and/or peribronchiolar mononuclear inflammation Dry cough and shortness of breath on exertion Pulmonary function testing: predominantly small airway obstructive pattern Chest radiography: usually normal or ill-defined pattern of infiltrates High-resolution computed tomography: thickened bronchial walls; pure interstitial involvement is less frequent (common types: lymphocytic interstitial pneumonia characterized by thin wall cysts on imaging, lymphocytic bronchiolitis (29), (30))
|
Liver | |
|
Hepatomegaly (14) | 25 |
Antimitochondrial antibodies and Sjögren syndrome: two-thirds of patients show liver enzyme abnormalities Liver histopathology: mild intrahepatic bile duct inflammation reminiscent of primary biliary cirrhosis (stage I) (14) Progression of Sjögren syndrome–associated primary biliary cirrhosis: very slow in clinical, serologic and histopathologic terms (31)
|
Presence of antimitochondrial antibodies (14) | 5 |
Kidney (15) | 2.5 |
Interstitial nephritis-lymphocytic infiltration around renal tubular cells Subclinical course Distal renal tubular acidosis (hypokalemia, low specific gravity and alkaline pH of the urine, nephrocalcinosis) (15), (32), (33) Mild chronic renal compromise (34)
|
Endocrine glands | |
|
Autoimmune thyroid disease | |
|
Antibodies to thyroid antigens (16) | 10–20 | |
Clinical hypothyroidism (16) | 1.5–16.5 |
Autoimmune adrenal disease | |
Antibodies to adrenal antigens (21 hydroxylase) (17) | 17 |
|
Overt adrenal failure (17) | 0 | |
Autoimmune ovarian disease | |
Antibodies to ovarian antigens (18) | 27 |
Immunocomplex-associated disease | |
Deposition of immunocomplexes in small vessels of the skin, nerves, kidney, brain as a result of B-cell hyperactivity
|
Cutaneous vasculitis (7), (19), (20) | | |
Palpable purpura (7) | 10 |
Most common manifestation of cutaneous vasculitis Associated with hypocomplementemia and cryoglobulinemia conferring increased risk of lymphoma Flat purpura can also occur in the setting of hypergammaglobulinemia
|
Peripheral neuropathy (21), (22) | 2–10 | |
Sensory axonal neuropathy | |
|
Small fibre neuropathy | |
|
Sensorimotor neuropathy | |
|
Glomerulonephritis (15) | ~2 |
Associated with systemic vasculitis, hypocomplementemia and cryoglobulinemia; adverse predictor of lymphoma and survival Membranoproliferative and membranous glomerulonephritis are the most commonly occurring histopathologic types Immunofluorescence showing IgM and complement deposition in renal tissues; hypertension, mild proteinuria and hematuria are the most common manifestations (36)
|
Central nervous system vasculopathy (23), (24) | 3–20 |
Associated with antibodies against SSA (Ro/SSA) Multiple sclerosis-like features (24), (37) Antibodies against aquaporin-4 have been detected among patients with lupus and Sjögren syndrome with evidence of longitudinally extensive transverse myelitis or optic neuritis (38)
|
Lymphoma (6) | 5–10 |
Chronic antigenic stimulation, genetic aberrations Sites of involvement include minor and/or major salivary glands, stomach, lungs, nodes; involvement of bone marrow is rare Adverse predictors of lymphoma include peripheral neuropathy, glomerulonephritis, lymphopenia, vasculitic or purpuric lesions, low C4 levels, cryoglobulinemia, germinal centres in salivary gland biopsy (7), (9), (39), (40)
|
Mucosa-associated lymphoid tissue (most common); nodal marginal zone lymphoma; diffuse large B-cell lymphoma | |