Answer to Q1

Saliva consists of a complex mixture of fluid, electrolytes, enzymes, and macromolecules

1. Lubrication to aid in swallowing and digestion
2. Digestion of starches with salivary amylase
3. Modulation of taste
4. Protection against dental caries
5. Defense against pathogens

Major salivary glands are the paired parotid, submandibular, and sublingual glands.
Minor salivary glands line the mucosa of the lips, tongue, oral cavity, and pharynx.

Infectious parotitis

Acute bacterial parotitis

Parotitis is now more common in elderly patients because many take medications with an atropine effect that retards salivary flow and predisposes to ascending infection. Many psychotropic drugs are relatives of antihistamines. Acute parotitis in neonates The characteristic clinical picture was of a sick premature infant with unilateral parotid swelling and inflammation. Seventy-five percent of the cases were in male infants. Pus expressed from the duct cultured S aureus in more than half of the cases. Most all of the cultured bacteria were from organisms present in the oral cavity, which suggests an ascending infection from the mouth.

Treatment is prompt administration of gentamicin and antistaphylococcal antibiotics plus adequate hydration, with a cure in approximately 80% of cases. Failure to improve after 24-48 hours of treatment necessitates surgical drainage. Recurrence is uncommon. Acute bacterial parotitis in children between one year of age and adolescence is extremely rare and only a few have been reported. The etiology and treatment is the same as for adults. Chronic bacterial parotitis Chronic bacterial parotitis may exist in the presence of calculi or stenosis of the ducts secondary to injury. In most instances, the chronic disease is either autoimmune or of unknown etiology with superimposed bacterial infections and should not be designated as a chronic bacterial infection.

Acute viral parotitis (mumps)

Mumps, one of the classic childhood infections, is spread by droplets or by direct spread from oropharyngeal secretions that contain the paramyxovirus. The disease was characterized by grossly enlarged and modestly tender parotid glands. Parotid stimulation caused pain in the gland and ear. Mumps was a benign disease in the vast majority of cases but was occasionally complicated by meningoencephalitis, pancreatitis, orchitis, or deafness especially in young adults. Treatment was and is symptomatic and supportive.

HIV parotitis
The course of the disease is different enough between children and adults to warrant a separate description.

HIV parotitis in children
Salivary gland involvement in children with HIV is well recognized and is much more common than involvement in adults. Characteristically, the gland is firm, nontender, and chronically enlarged (unilateral or bilateral) and usually causes few symptoms. Lymphoepithelial cysts are less common than in adults. Xerostomia with decreased salivary flow rates occurs in adults but is infrequent in children. Infiltration of CD8-positive lymphocytes, possibly as a result of HIV, Epstein-Barr virus (EBV), or an interaction between the 2, enlarges the gland. The diagnosis of HIV parotitis is usually clinical with the typical findings.

Parotitis in tuberculosis
Tuberculosis is an uncommon cause of parotitis.Patients have enlarged, nontender, but moderately painful glands. Involvement is most frequently confined to the parotid lymph nodes, but the gland may become diffusely involved with the disease.

Non-infectious parotitis

Chronic punctate parotitis (chronic autoimmune parotitis)
Although acute bacterial parotitis is fairly well understood, chronic enlargement of the salivary glands with recurring infection has caused confusion for more than a century. Numerous terms found in the literature, such as Mikulicz disease, Sjögren syndrome, benign lymphoepithelial lesion of Godwin, chronic punctate sialectasis, and recurrent parotitis of childhood.

Sjögren syndrome
Most authors classify the disease as definite Sjögren syndrome, which includes (1) objective evidence of keratoconjunctivitis sicca or (2) characteristic pathologic features of the salivary glands. The probable Sjögren syndrome requires 2 out of 3 of the following: (1) recurrent chronic idiopathic salivary gland swelling, (2) unexplained xerostomia, and (3) connective tissue disease.The involved parotid gland is enlarged and tender at times. Massage of the gland produces clear saliva with flocculated clumps of coagulated proteins.

Diseases of uncertain etiology

• Salivary stone (sialolithiasis)
• Chronic recurrent parotitis (chronic nonspecific parotitis)
• Recurrent parotitis of childhood
• Sarcoidosis
• Pneumoparotitis
• Miscellaneous causes of inflammation and enlargement of the parotid

Question 2 - What investigations would you request?

Answer to Q3

Recurrent parotitis of childhood is an inflammatory condition of unknown etiology, that leads to recurrent episodes of parotid swelling and pain. The recurrent episodes of acute or subacute parotid gland swelling are associated with fever, malaise, and pain; and are usually unilateral. Episodes can last days to weeks and occur every few months.

Treatment is supportive and include adequate hydration, gland massage, warm compresses, and sialagogues. Sialendoscopy has been shown to decrease the frequency and severity of episodes.

Recurrent parotitis of childhood usually resolves spontaneously with puberty. Surgery is rarely required.

Question 4 - What other diagnostic criteria is necessary to diagnose childhood onset Sjögren’s?