Chronic sclerosing sialadenitis

Chronic sclerosing sialadenitis (CSS) is a rare, chronic inflammatory disease that primarily affects the salivary glands, particularly the parotid gland. The condition is characterized by progressive fibrosis (scarring), inflammation, and hardening of the salivary glands.

Etiology

The exact cause of CSS is unknown. However, it is believed to be an autoimmune disease, where the body's immune system mistakenly attacks its own tissues. Some researchers suggest that it may be associated with IgG4-related disease, a systemic condition characterized by tumefactive lesions and often elevated serum IgG4 levels.

Clinical Presentation

Patients with CSS typically present with a firm, painless swelling of the salivary glands. The swelling is often unilateral and can progressively increase in size. Other symptoms may include xerostomia (dry mouth), dysphagia (difficulty swallowing), and recurrent salivary gland infections.

Diagnosis

Diagnosis of CSS is often challenging due to its rarity and non-specific symptoms. It is typically confirmed through a combination of clinical examination, imaging studies such as ultrasound or computed tomography (CT) scan, and histopathological examination of a biopsy specimen. The histopathological findings typically show dense lymphoplasmacytic infiltrate, fibrosis, and acinar atrophy.

Treatment

Treatment of CSS is primarily aimed at managing symptoms and preventing complications. This may include the use of corticosteroids to reduce inflammation, sialogogues to stimulate saliva production, and antibiotics to treat or prevent infections. In severe cases, surgical removal of the affected gland may be necessary.

Prognosis

The prognosis of CSS is generally good with appropriate management. However, the condition can significantly impact the patient's quality of life due to persistent symptoms and potential complications such as recurrent infections and sialolithiasis (salivary stones).

See Also

• Sjögren's syndrome
• Mikulicz's disease
• Salivary gland diseases