Symptoms of Sjögren's Syndrome
Primary Sicca Manifestations
The hallmark symptoms of Sjögren's syndrome are severe dry eyes (keratoconjunctivitis sicca) and dry mouth (xerostomia), which occur due to lymphocytic infiltration and destruction of the lacrimal and salivary glands. 1, 2
Ocular Symptoms
- Sensation of foreign body or grittiness in the eyes 1
- Eye irritation, itching, or pain 1
- Light sensitivity (photophobia) 1
- Frequent need for artificial tears or eye drops 1
- Reduced tear production measurable by Schirmer test ≤5 mm/5 minutes 3, 1
Oral Symptoms
- Difficulty swallowing dry foods without liquids 1
- Frequent need to sip water throughout the day 1
- Burning sensation in the mouth 1
- Painful sores or red patches at corners of mouth (angular cheilitis) 1
- Frequent dental cavities despite good oral hygiene 1, 4
- Gum inflammation or receding gums (gingivitis) 1
- Altered taste sensation 4
Constitutional and Musculoskeletal Symptoms
Extreme fatigue and widespread pain are among the most debilitating symptoms, often having greater impact on quality of life than the sicca symptoms themselves. 3, 1
- Profound, persistent fatigue that interferes with daily activities 1, 4
- Joint pain (arthralgias) without significant inflammation 3, 1
- Muscle pain (myalgias) 1
- Non-inflammatory musculoskeletal pain affecting multiple sites 3
Systemic Manifestations
Mucosal and Skin Involvement
- Vaginal dryness causing painful intercourse 1
- Dry skin with itchiness or flaking (xerosis) 1, 5
- Generalized pruritus 5
Pulmonary Manifestations
- Chronic dry, nonproductive cough (xerotrachea) affecting up to 38% of patients 1
- Shortness of breath ranging from minimal dyspnea on exertion to severe respiratory compromise 1
- Small airway disease (bronchiolitis) 1
- Bronchiectasis with chronic sputum production 1
- Interstitial lung disease developing in 10% within first year, 20% after 5 years 1
Neurological Symptoms
- Peripheral neuropathy with numbness, altered sensation, or burning pain in feet, legs, or hands 1
- Cranial nerve involvement affecting facial sensation or function 1
Glandular Enlargement
- Parotid or submandibular gland swelling, which may be recurrent 6
- Lacrimal gland enlargement visible on examination 1
Serious Complications to Monitor
Approximately 5% of patients with Sjögren's syndrome develop lymphoma, representing a 320-fold increased incidence compared to the general population. 1
- Persistent or progressive glandular swelling may indicate lymphoproliferative disease 1
- Decreased C4 complement levels at diagnosis correlate with higher lymphoma risk 3, 1
- Cutaneous vasculitis can develop in some patients 5
- Scleritis, sterile keratitis, and uveitis as ocular immune manifestations 3
- Renal tubular acidosis 7
- Autoimmune thyroid disease 7
Clinical Pearls
- Women are affected 20 times more frequently than men, typically presenting in middle age 1, 6
- Approximately 10% of patients with clinically significant aqueous deficient dry eye have underlying Sjögren's syndrome 1
- Symptoms often develop insidiously and may be attributed to aging, medications, or environmental factors, leading to delayed diagnosis 8
- The profound impact on quality of life is frequently underappreciated by clinicians 8
- Unexplained chronic cough with dry eyes led to Sjögren's diagnosis in 36% of previously undiagnosed patients 1