Approach to Parotid Gland Enlargement Iraj Salehi-Abari MD Approach to Parotid Gland Enlargement Iraj Salehi-Abari MD., Internist Rheumatologist salehiabari@sina.tums.ac.ir

In the name of God the merciful the compassionate

The Parotids are one of salivary glands The causes of parotid gland swelling # 43

Approach to Parotid gland enlargement: Acute versus Chronic Swelling Unilateral versus Bilateral Swelling Painful versus Painless Swelling Obstructive versus non-Obstructive Swelling Granulomatous versus non-Granulomatous Isolated Swelling versus Swelling with systemic features Childhood versus Adulthood Swelling Febrile versus non-Febrile Swelling Suppurative versus non-Suppurative Swelling

Classification of Parotid gland enlargement: Infective Inflammatory Immune mediated (autoimmune) Neoplastic Non-inflammatory Non-neoplastic Congenital

Important Comments: Unilateral parotid swelling is more common than Bilateral The causes of Bilateral swelling: numerous The causes of Unilateral swelling: a few

Unilateral parotid enlargement:

Bilateral parotid enlargement: Chipmunk-like cheeks

Important Comments: In Bilateral parotid swelling with systemic features, for diagnosis usually: Do not require Imaging of parotid gland Do not require Biopsy of Parotid gland In Unilateral parotid swelling without systemic features, for diagnosis usually: require Imaging of parotid gland require Biopsy of Parotid gland

Parotid gland Imaging: MRI CT scan Ultrasonography Scintigraphy Sialography

Unilateral Parotid gland swelling: Acute: Bacterial (Suppurative) parotitis Stones (Sialolithiasis) Chronic: Chronic bacterial parotitis Tumors (Neoplasms) Stones Actinomycosis

Bilateral parotid gland swelling: Painful, Inflammatory: Acute Chronic Painless, non-Inflammatory, non-Neoplastic: Sialadenosis

Acute painful, inflammatory bilateral parotid gland swelling: Viral Parotitis: Mumps Coxsackie virus Echovirus EBV CMV

Chronic painful (or little painful), inflammatory bilateral parotid gland swelling: Sjogren’s syndrome Chronic granulomatosis diseases: Sarcoidosis (Heerfordt’s syndrome) Tuberculosis Leprosy Cat scratch fever Granulomatosis with polyangiitis (WG)

Chronic painful (or little painful), inflammatory bilateral parotid gland swelling: IgG4 related systemic disease IgE related systemic disease: Kimuras’S disease Chronic viral disease HCV HIV Recurrent parotitis of childhood

Chronic painless, non-inflammatory Non-neoplastic bilateral parotid gland swelling: Eating disorder Anorexia Bulimia Malnutrition and Alcoholism Hyperlipoproteinemia Malabsorbtion: Celiac Cirrhosis Chronic Pancreatitis

Chronic painless, non-inflammatory Non-neoplastic bilateral parotid gland swelling: Amyloidosis Diabetes mellitus Acromegaly Myxedema Gonadal hypofunction Cushing’s syndrome

Bilateral parotid swelling with dry eyes: Sjogren’s syndrome Sarcoidosis Amyloidosis IgG4-related systemic disease

Bilateral parotid swelling with dry mouth: Sjogren’s syndrome Sarcoidosis Amyloidosis IgG4-related systemic disease Diabetes mellitus HCV HIV

Bilateral parotid swelling with dry mouth and dry eyes: Sjogren’s syndrome Sarcoidosis Amyloidosis IgG4-related systemic disease

Bilateral parotid swelling with Macroglossia: Amyloidosis Sarcoidosis Acromegaly Hypothyroidism

Parotid swelling with Facial n. paralysis: Unilateral parotid swelling: Malignant neoplasm of parotid Bilateral parotid swelling: Sjogren’s syndrome Sarcoidosis

Parotid glands swelling with lacrimal glands enlargement: Sjogren’s syndrome Sarcoidosis: Scintigraphy using Gallium-67 citrate may produce the classic “Panda sign” owing to bilateral increased uptake by the lacrimal and parotid glands

Parotid G. enlargement + bihilar adenopathies: Sarcoidosis Tuberculosis Lymphoma Parotid G. enlargement + ILD: Sjogren’s syndrome Parotid G. enlargement + bihilar adenopathies + ILD:

Heerfordt’s syndrome: (Uveoparotid fever) Uveitis Parotid swelling Facial nerve paralysis Fever

Causes of parotid glands swelling in Sjogren’s syndrome: Parotid stones Bacterial infection of parotid Viral (Mumps, EBV, CMV) trigger Non-Hodgkin’s lymphoma Hypothyroidism Pancreatitis Cirrhosis HCV

There are three key findings: Salivary gland swelling Dry eyes Amiralam Hospital Guideline for General Practitioner to approach to patients suspected to have primary Sjogren’s syndrome: There are three key findings: Salivary gland swelling Dry eyes Dry mouth If there are at least 2 items of above in a patient, he/she has to be referred to a Rheumatologist by GP.

In a patient with parotid G. enlargement: If there is chronic or recurrent bilateral parotid enlargement and at least one of below features: Pathologic dry eyes Pathologic dry mouth He/she has to be evaluated for sjogren’s syndrome

Items must be done for patients suspected to have primary Sjogren’s syndrome: (I) Hx. and Ph. Ex. By Rheumatologist Schirmer test and Slit lamp examination (Rose Bengal test) by Ophthalmologist At least one test to quantifying the amount of saliva CBC, ESR, CRP, FBS, SGOT, SGPT, BUN/Cr, U/A Anti-Ro, Anti-La, RF and FANA PA-CXR

Items must be done for patients suspected to have primary Sjogren’s syndrome: (II) Labial Gland Biopsy (LGB) by ENTman or General Surgeon Pathologic evaluation and looking for Focus score > 1 by Pathologist In suspected states: Plasma IgG4 level, ACE In suspected states: HIV, HBsAg, Anti HCV If we need: CT scanning or MRI, Scintigraphy or Sialography of salivary glands and Biopsy of them by ENTman may be done

Diagnosis of Sjogren’s synd.: American-European classification criteria: Ocular symptoms Oral symptoms Ocular signs: Schirmer’s test Rose bengal staining Pathology: > 1 focus score(50 lymphocytes /4 mm2) Salivary gland involvement Salivary flow < 1.5 ml in 15 min Parotid sialography Salivary scintigraphy Autoantibodies: Anti-Ro & or Anti –La 4 of 6 ; Definite Sjogren’s syndrome

Diagnosis of Sjogren’s synd.: American-European classification criteria: Ocular signs: Schirmer’s test Rose bengal staining Pathology: > 1 focus score(50 lymphocytes /4 mm2) Salivary gland involvement Salivary flow < 1.5 ml in 15 min Parotid sialography Salivary scintigraphy Autoantibodies: Anti-Ro & or Anti–La 3 out of 4  Sjogren’s syndrome

Diagnosis: Secondary Sjogren’s syndrome: A connective tissue disease plus Ocular or Oral dryness and At least 2 items out of below 3 items: Ocular sign Positive LGB (Biopsy) Abnormal tests of salivary gland function

In a patient with bilateral Parotid G. enlargement: If no dry eyes and no dry mouth:  very low probability of Sjogren’s If no lung involvement and no skin involvement:  low probability of Sarcoidosis

Chest radiograph in Sarcoidosis: Stage 0: Normal: 5% Stage I: Bilateral hilar adenopathy: 50% Stage II: Bihilar adenopathy + ILD: 25% Stage III: ILD: 15% Stage IV: Fibrosis: 5%

Evaluation of the patients with suspected Sarcoidosis: Hx and Ph. Examination Ophthalmologic Examination PA-CXR, PFTs CBC, ACE, LFT, serum Ca, U/A PPD HRCT of Lungs Biopsy: noncaseating Granulomas Lofgren’s and Heerfordt’s syndrome: no Biopsy

A patient with Bilateral Parotid G. enlargement and : Black eyes sign Shoulder pad sign Symmetric CTS Hepatosplenomegaly Cardiomyopathy Proteinuria Skin petechia and purpura is a case of Amyloidosis Biopsy and Congo red staining are necessary for documentation of DX.

A patient with benign tumor of Parotid G.: Chronic unilateral diffuse swelling of parotid No skin involvement No lymphadenopathy No facial n. paralysis

A patient with malignant tumor of Parotid G.: Chronic unilateral local swelling of parotid gland with rapid growth Skin retraction Lymphadenopathy Facial n. paralysis

A student of school of medicine has: Acute painful bilateral parotid swelling Sore throat Fever Maculopapular rash Diagnosis: Infectious mononucleosis

Typical feature of Mumps: A children with Acute painful bilateral parotid swelling Fever

Chronic bilateral parotid enlargement with Dactylitis: Sarcoidosis Tuberculosis

Chronic bilateral parotid enlargement with lacrimal gland enlargement and facial n. palsy without dry eyes and without dry mouth: Diagnosis: Sarcoidosis

A boy of 8 years old with history of recurrent parotid gland stones has an older brother with the same history. Recently his parotid has been fistulized with yellow creamy discharge: Diagnosis: HGPRT deficiency  Hyperuricemia