Case of the Month

October 2016

SASPID case of the month

Nicolette du Plessis, Paediatric Infectious Diseases, University of Pretoria

A 9-year old girl was referred for evaluation with a 5-year history of recurrent parotid swelling. The episodes of parotid swelling occur once every 4-5 weeks, is mostly bilateral, and is not associated with fever. She does experience localised pain for 4-5 days. The swelling subsides between “attacks”. She also complains of infrequent, but intermittent cramping in both her hands.

She underwent a laparotomy at 1 year of age for intestinal obstruction (possible intussusception) with bowel resection.

Birth history: Nothing significant
Mom’s antenatal history: Normal
Immunizations: Up-to-date
Feeding: Normal family diet
Social history: 7 siblings, rest of the siblings are healthy.

Clinical examination

She is a well grown pre-pubertal girl.

She has visible bilateral parotid enlargement. No abnormalities could be visualised at the opening of the parotid ducts and no stones or masses were palpable.

She had no dysmorphic features, no features of neurocutaneous syndromes or other skin changes visible.

The rest of her systemic examination was normal.

Question 1 What would be your differential diagnosis to recurrent chronic parotitis in childhood?

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 Q2

Salivary gland investigations

Parotid duct evaluation: sialography, CT scan, scialendoscopy

Salivary gland biopsy

Infectious diseases investigations


TB investigations

EBV serology

Auto-immune / Immunology investigations

Auto-immune work up: Rheumatoid factor, ANA, Anti-SSA/Rho and AntiSSB/La

IgA, IgM, IgG with subclasses, IgE


Other investigations

Ophthalmological evaluation for Schirmer’s or Rose Bengal tests

Question 3 - Shortly discuss the condition “recurrent parotitis of childhood”?

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?

Answer to Q4

Sjögren syndrome is rare in pediatric patients. It was first described as a slowly progressive inflammatory disorder that involves the exocrine glands (keratoconjunctivitis and xerostomia), until 1933 when Sjögren recognized the association of this symptom complex with polyarthritis. It is now described as a syndrome with both exocrine glandular and extraglandular features. Sjögren’s can be primary or secondary to other autoimmune disorders such as systemic lupus erythematosus (SLE), rheumatoid arthritis, scleroderma, and biliary cirrhosis.

Clinical manifestations may vary more than those seen in adult patients. Children, compared to adults, have lower frequency of sicca syndrome, higher rates of parotid enlargement, and a higher prevalence of immunologic markers. There are many diagnostic criteria for the diagnosis of adult onset Sjögren syndrome that include ocular and oral symptoms, ocular signs, diagnostic features on histopathological and imaging studies, and the presence of auto-immune markers (RF, ANA, Ro/SS-A, La/SS-B). Some authors suggest that a revised set of diagnostic criteria is made for childhood onset Sjögren syndrome.

Patient outcome

Her results:
Parotid investigations
Parotid MRI and sonar – enlarged parotids with sialectasis (possible chronic sialo-adenitis / Sjögrens / benign lympho-epithelial lesions)
Parotid biopsy showed non-specific inflammation
Infectious diseases results
HIV negative – test repeated numerous times with RTK and ELISA.
Previous IgG and IgM was positive for mumps.
EBV serology indicated a previous infection (VCA IgG positive, VCA IgM negative).
EBV PCR – 100copies/ml
Immune results
Rheumatoid factor – 11 IU/ml (normal)
ANA – positive screen on IFA (titer 160)
Anti-SSA/Rho and AntiSSB/La – Anti-SSA/Rho positive 600 U/ml, anti SSB/La negative
CD4:CD8 ratio – CD4 802cells/mm, CD8 230 cells/mm, CD4:CD8 ratio 3.48
IgA/IgM/IgG and IgG subclasses – within normal limits
Other investigations
FBC values were within normal limits and ESR / CRP were not significantly elevated.
She is referred for ophthalomological evaluation.

Working diagnosis:
A preliminary diagnosis of childhood onset Sjögren syndrome was made.

She will be evaluated by ophthalmology for features of sicca syndrome.
The recurrent episodes of parotid swelling is managed conservatively with pain medication. No steroids are advised at this stage.
Lessons learned

Close attention must be paid to emotional and cognitive functioning of the adolescent coping with a chronic disease such as Sjögren syndrome. Medical care for children with primary Sjögren syndrome is primarily based on strategies used for adults. No controlled studies in children with this disorder have been reported.

General measures include:
Discourage patients from smoking.
Instruct patients to avoid windy and low-humidity environments.
The family dwelling should be well humidified.
Support normal school attendance and academic functioning in patients with juvenile Sjögren syndrome.


  1. Wilson KF, Meier JD, Ward PD. Salivary Gland Disorders. American Family Physicians. 2014;89(11)
  2. Cimaz R, Casadei A, Rose C, Bartunkova J, et al. Primary Sjögren syndrome in the paediatric age: a multicentre survey. European Journal of Pediatrics. 2003;162(10):661-665.
  3. Roby BB, Mattingly J, Jensen EL, Gao D, Chan KH. Treatment of juvenile recurrent parotitis of childhood: An analysis of effectiveness. JAMA Otolaryngology - Head and Neck Surgery. 2015;141(2):126-129.
  4. Leerdam CM, Martin HCO, Isaacs D. Recurrent parotitis of childhood. Journal of Paediatrics and Child Health. 2005;41(12):631-634.
  5. Baszis, K., Toib, D., Cooper, M., French, A., White, A. Recurrent parotitis as a presentation of primary pediatric Sjögren syndrome. Pediatrics. 2012;129(1):e179-e182.

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