Granulomatous Diseases

Question 1

What is a granuloma?

what are the macrophages termed in a granuloma?

Answer 1

localised cluster of macrophages characteristic of some forms of chronic inflammation, classically TB or foreign body reactions

• macrophages have a particular appearance and are termed epitheliod cells
• may aggregate to form ‘giant cells’ e.g. langerhans giant cells
• Sometimes accompanied by multinucleated giant cells
• Solitary granulomas may form in response to persistent local inflammatory stimuli e.g. foreign body

Question 2

Symptoms, diagnosis and treatment of TB

Answer 2

• caused by mycobacterium tuberculosis
• Symptoms:
  Malaise, reduced appétits, weight loss, fever, cough with purulent or blood stained sputum
• Diagnosis:
  biopsy, chest RX, specimen of sputum stained with Ziehl-Neelson
• Tx:
  Rifampicin, isoniazid, Ethambutol (+pyrazinamide)

Question 3

How does oral tuberculosis occur? what is the presentation

Answer 3

• Secondary to active pulmonary disease - the mucosa is infected from the sputum
• affects elderly with pulmonary infection and chronic cough, or impaired immune function
• presentation: ulcer on the mid-dorsum of the tongue; non-healing extraction socket

Question 4

What are the characteristics of each stage of syphilis?

Answer 4

1. Congenital syphilis
   - diagnostic triad: interstitial keratitis of cornea, hearing loss, dental anomalies
2. Primary syphilis
   - Oral chancre appears 2-8 weeks after infection on lip or tongue - initially firm nodule which breaks down into painless ulcer
   - chancre heals without scarring
3. Secondary syphilis
   - 1-4m after infection
   - mild fever with malaise, headache, sore throat and lymphadenopathy
   - rash and stomatitis
   - flat ulcers mainly affecting tonsils, lateral border of tongues and lips
4. Tertiary syphilis
   - 3+y after infection
   - leukoplakia of tongue, aortitis
   - characteristic oral lesion: gumma usually on palate, tongue or tonsils, starts as welling that undergoes necrosis leaving deep ulcer
   - heals with scarring that can distort soft palate/tongue

Question 5

Clinical features of orofacial granulomatosis

Answer 5

• used to describe oral mucosal granulomatous inflammation without an identifiable cause (likely to be related to food allergy
• similar to oral lesions of Crohn’s
• lip and buccal mucosa are main sites involved with marked oedema and cobblestone appearance

Question 6

Treatment of orofacial granulomatosis

Answer 6

• dietary restriction
• topical steroids
• Intra-lesional corticosteroid injections
• immunosuppressants: prednisolone
• anti TNF-alpha inhibitors

Question 7

what is the pathology and clinical features of sarcoidosis?

Answer 7

• Autoinflammatory disease - inflammation is enhanced and immune reactions are supressed
• small non-caveating granulomas found in affected tissues containing multinucleate giant cells surrounded by lymphocytes
• symptoms:
  fever, weight loss, fatigue, breathlessness, cough, arthralgia
• primarily a diesease of lungs and lymph nodes but can affect gingivae and lips, followed by palate and buccal mucosa
• some cases can get bilateral swelling of major salivary glands almost always parotid

Question 8

Treatment for sarcoidosis

Answer 8

• Often no tx required
• If needed: NSAIDs and usually spontaneous resolution follows
• Those with extrapulmonary or extensive lung involvement maybe given corticosteroids, methotrexate or azathioprine
• sarcoidosis carries a risk of lung cancer or leukaemia later in life

Question 9

Crohn’s disease clinical features

Answer 9

• Inflammatory bowel disease involving granulomatous inflammation in ileocaecal region
• abdominal pain, constipation, diarrhoea, sometimes obstruction and malabsorption
• lips - tense swelling or mucosal thickening
• buccal mucosa- cobblestone appearance
• gingival - erythematous
• ulcers- resembling aphthae
• mucosal tags
• glossitis: due to iron, folate or vitamin B12 deficiency

Question 10

Treatment for Crohn’s disease

Answer 10

• dietary adjustment
• corticosteroids
• antibiotics
• sulfasalazine
• immunosuppressants

Question 11

What is Melkersson-Rosenthal syndrome

Answer 11

rare syndrome of unilateral recurrent facial paralysis, lip/facial swelling, and fissured tongue
- clinical features and treatment same as OFG

Question 12

What are the clinical features of granulomatosis with polyangiitis (old name: Wegener’s granulomatosis)?

Answer 12

multi systemic chronic granulomatous disease of unknown cause consisting of:

• necrotising granulomatous lesions of respiratory tract
• systemic vacuities of small arteries and veins
• necrotising glomerulonephritis

oral features:

• Hyperplastic gingivae
• tooth mobility
• poor post-extraction healing
• parotid gland swelling
• palatal swelling

Question 13

What is the management of granulomatosis with polyangiitis (old name: Wegener’s granulomatosis)?

Answer 13

• Induction of remission (cyclophosphamide and prednisolone)
• Maintenance (azathioprine or methotrexate) for long term control
• relapse is common