26th January 2024

Presentation

• Systemic Autoimmunity: Thyroid dysfunction and/or other co-existing autoimmune conditions are found in approximately 45% of patients with primary SS. 
• Fatigue: Extreme fatigue despite getting more rest, occurs in approximately 50% of patients and is sometimes caused or made worse due to underlying hypothyroidism. 
• Cutaneous manifestations: Approximately 55% of patients complain of skin dryness, which is a main characteristic feature of SS. Some patients complain of a rash that is vasculitic in nature and sometimes may be due to underlying vasculitis. Raynaud phenomenon may be observed in about 30% of cases. However, it is usually very mild. 
• Pulmonary manifestations: Up to 30% of patients have subclinical pulmonary disease, and lung function tests may show small airway obstruction. Other pulmonary complications include lymphocytic alveolitis, lymphocytic interstitial pneumonitis and fibrosis, and pseudolymphoma. 
• Gastrointestinal manifestations: Oesophageal dysmotility is the most common GI presentation and is treated symptomatically with prokinetic drugs. Rarely, mild pancreatitis or autoimmune hepatitis may occur. 
• Renal manifestations: Some patients with SS may have tubulointerstitial involvement and glomerular lesions leading to symptoms such as haematuria, proteinuria and renal insufficiency. 
• Neurological involvement: The most common significant manifestation is sensory peripheral neuropathy, which is found in approximately 22% of patients. 

Image Source: Wikimedia Commons (2022). Sjogrens Syndrome, Licensed under a creative commons licence. Available for free at:  https://upload.wikimedia.org/wikipedia/commons/5/54/Sjogrens_Syndrome.jpg (Accessed: 10 July  2022).

Image Source: Wikimedia Commons (2022). Secondary Raynaud’s in Sjögren’s syndrome 1, Licensed under a creative commons licence. Available for free at: https://upload.wikimedia.org/wikipedia/commons/8/8b/Secondary_Raynaud%27s_in_Sj%C3%B6gren%27s_syndrome_1.JPG (Accessed: 10 July 2022).