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Granulomatous diseases in ENT

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Granulomatous conditions in ENT are rare conditions that we come in contact with, we tend to overlook them because they are so rare, however some of the conditions like TB and syphillis and Mucormycosis of the Nose and PNS are seen in our clinics
this is a good summary from scotts brown chapter

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Granulomatous diseases in ENT

  1. 1. Granulomatous conditions in ENT
  2. 2. Granuloma formation • Response of immune system to an indigestible agent • Usually neutrophils remove agents by phagocytosis & digestion • Macrophages phagocytose indigestible agents, loose their mobility & accumulate at site of injury • Undergo structural changes: Larger with more cytoplasm  epitheloid cells • Epitheloid cells fuse  Multinucleated giant cells • Nuclei arrange in horse shoe pattern  Langerhans giant cells • All these cells are surrounded by a collar of lymphocytes
  3. 3. Granuloma
  4. 4. Granuloma • Noncaseating or Caseating – Central area of necrosis – “Cheese-like” appearance – Often visible macroscopically
  5. 5. Granulomatous diseases • Infective • Inflammatory • Neoplastic • Forms part of a spectrum of conditions in which vasculitides play a role • Many are systemic conditions – Preferentially target or present in the upper respiratory tract
  6. 6. Granulomatous diseases
  7. 7. INFLAMMATORY CONDITIONS
  8. 8. Wegener’s Granulomatosis • Relatively common disease of upper airway • Friedrick Wegerner – 1939: Necrotizing granulomas and vasculitis of upper & lower airway, occurring either together or as separate components • Coexistence of vasculitis & granulomas • Classically involves triad: – Airway – Lung – Renal disease
  9. 9. Wegener’s Granulomatosis • Aetiology = unknown • Hypersensitivity reaction to an unknown stimulus • Affects all ages and both males & females
  10. 10. • 3 main forms of WG – Type 1 • Limited form • Symptoms of URTI persisting for weeks & not responding to A/b • Pain over the dorsum of the nose • Epistaxis & variable degree of nasal obstruction • Very large crusting of the nasal cavities & nasopharynx bilaterally • When crusts are removed – mucosa is very friable • Septal perforations & eventual nasal collapse in advanced disease Wegener’s Granulomatosis
  11. 11. Wegener’s Granulomatosis • Early changes – Angry mucosa – Mucous hypersecretion
  12. 12. Wegener’s Granulomatosis • End result of advanced disease
  13. 13. Wegener’s Granulomatosis • End result of advanced disease
  14. 14. Wegener’s Granulomatosis • End result of advanced disease – Infiltration of orbit – Sclerosis and opacification of nasal cavity – Septal erosion
  15. 15. Wegener’s Granulomatosis • End result of advanced disease – Infiltration of orbit – Septal destruction – Hyperostosis – Opacification of sinuses
  16. 16. • 3 main forms of WG – Type 2 • Sicker patient with more systemic symptoms • Pulmonary involvement – Cough & pleuritic pain – Haemoptysis & cavitating lesions on CXR – Encapsulated lung abscess Wegener’s Granulomatosis
  17. 17. • 3 main forms of WG – Type 3 • Widely disseminated with involvement of multiple organs – Airway » Short segment of subglottic/upper tracheal stenosis – Pulmonary – Renal » Hematuria & abn urinary sediment & segmental/diffuse glomerulonephritis – Cutaneous » Tick-bite like lesions on the distal limbs – Oral » Hyperplastic granular lesion of gingiva; ulcerative stomatitis – Otological » OME ± mastoiditits & profound SNHL – Eye » Conjunctivitis; Dacrocystitis; Episcleritis; Orbital pseudotumour Wegener’s Granulomatosis
  18. 18. Wegener’s Granulomatosis
  19. 19. Wegener’s Granulomatosis • Diagnosis – cANCA • 95% sensitive in generalized disease • 60% sensitive in localized disease – FBC, ESR, CRP, CXR, Urine analysis for casts – Histology: Tissue from septum & all turbinates – CT nose & paranasal sinuses • Non-specific mucosal thickening; bony destruction; new bone formation
  20. 20. Wegener’s Granulomatosis
  21. 21. Wegener’s Granulomatosis • Main histological features: – Vasculitis involving medium & small vessels – Granulomatous necrosis (can be non-necrotic); Large epitheloid cells; lined with histiocytes – Scattered multinucleated giant cells
  22. 22. Wegener’s Granulomatosis • Treatment: – Prednisone 1mg/kg/day + Cyclophosphamide 2mg/kg/day x 1/12 • Prednisone tapered to alternate days x 2/12 & then discontinued once complete response achieved • Cyclophosphamide continued x 6/12 to 1 year & then tapered over a few months – Other • Azathioprine • Methotrexate • Plasma exchange Ig infusion – Nose • Topical steroids, nasal douching & irrigation – Surgical reconstruction • Wait until disease has been in remission for some time
  23. 23. Sarcoidosis • Systemic condition of unknown aetiology • Can involve any organ including many H&N structures • Incidence = 64/100,000 in Scandinavia • Condition of young adults between 3rd & 5th decade • Female preponderance = 2:1 • Ratio of black to Caucasian = 12:1 • Nasal manifestation almost always part of multisystem sarcoid which have been present for some years
  24. 24. Sarcoidosis
  25. 25. Sarcoidosis • Histology – Epitheloid cells surrounded by lymphocytes & fibroblasts, but devoid of caseation – Crystalline or calcified inclusion bodies sometimes seen (Schaumann bodies)
  26. 26. Sarcoidosis • Nasal involvement – Presenting complaints
  27. 27. Sarcoidosis • Nasal involvement – External • Lupus pernio • Predilection for cold sensitive areas, such as tip of nose
  28. 28. Sarcoidosis • Lupus pernio of the cervical skin
  29. 29. Sarcoidosis • Nasal involvement – Mucosa • Granular appearance (“Strawberry skin”) – Tiny pale granulomas against a erythematous mucosa • Very friable mucosa • Crusting, nasal congestion, mucopurulent discharge • Anterior septal perforation & later nasal bridge collapse • Paranasal sinus involvement & infection
  30. 30. Sarcoidosis • Nasal involvement – Sclerosis & osteolysis of nasal bones – Soft tissue mass in nose
  31. 31. Sarcoidosis • Salivary glands – Bilateral non-tender, firm, smooth parotid swelling • Lymphoid hyperplasia – Adenoids – OSA; OME • Supraglottic larynx – Dyspnoea; dysphonia – Sub sites • Epiglottis  arytenoid  aryepiglottic folds  false cords
  32. 32. Sarcoidosis • Laryngeal involvement
  33. 33. Sarcoidosis • Laryngeal involvement
  34. 34. Sarcoidosis • Diagnosis – Combination of histology, imaging & haematology – Exclude other causes for non-caseating granulomatous changes – ↑ serum ACE (83% of patients with active sarcoid) • Also in TB, Leprosy, 1ᴼ biliary cirrhosis –  ESR, CRP & urinary calcium – Mild anaemia, leucopenia, thrombocytopenia, eosinophilia
  35. 35. Sarcoidosis • Treatment – Oral steroids, Methotrexate ± Hydroxychloroquine – Topical intranasal steroids (spray/drops) – Nasal douching/irrigation – Surgery contraindicated for both cosmetic & functional indications
  36. 36. Churg-Strauss Syndrome • 1st described 1951 by Churg & Strauss – Syndrome of systemic vasculitis & asthma • Eosinophil-rich & granulomatous infl involving the resp tract & necrotizing vasculitis affecting small to medium sized vessels & associated with asthma & eosinophilia
  37. 37. Churg-Strauss Syndrome • 3 phases – Prodromal phase • May persist for years • Allergic disease (allergic rhinitis, nasal polyposis, asthma) – Peripheral blood & tissue eosinophilia • Chronic eosinophilic pneumonia & eosinophilic gastroenteritis – Life threatening systemic vasculitis
  38. 38. Churg-Strauss Syndrome • Histo – Necrotizing giant cell vasculitis, interstitial granulomas & eosinophilic pulmonary infiltrates
  39. 39. Churg-Strauss Syndrome • Nasal involvement – Usual polyposis – May also have crusting & septal perforation – Doesn’t display diffuse mucosal destruction like with WG
  40. 40. Churg-Strauss Syndrome • Treatment – Oral steroids – Polyposis • Topical steroids ± surgery
  41. 41. Giant cell Granuloma • “giant cell reparative granuloma” or “giant cell reaction of bone” • Benign condition of jaws & other craniofacial sites • Commonly occur in children & young adults
  42. 42. Giant cell Granuloma • Clinical features – Maxilla & mandible most commonly affected –  sphenoid bone  temporal bone – Pain & swelling over affected bone – Diplopia; frontal headache; hearing loss; vertigo; tinnitus
  43. 43. Giant cell Granuloma • Imaging – Expansile lytic lesions – “Soap bubble” centre – Well demarcated edges
  44. 44. Giant cell Granuloma • Cherubism – Bilateral symmetrical involvement of jaws – Rare inherited childhood condition
  45. 45. Giant cell Granuloma • Cherubism – Bilateral symmetrical involvement of jaws – Rare inherited childhood condition
  46. 46. Cholesterol granuloma • Granulomatous reaction to cholesterol crystals precipitated in tissues • Presumed 2ᴼ to haemorrhage and/or trauma • Lesions may affect maxilla, frontal sinus, temporal bone • Produce expansion of bone, cosmetic deformity & displacement of adjacent structures
  47. 47. Cholesterol granuloma • Imaging – Cyst-like expansion of bone/sinus – Opaque on CT & not contrast enhancing • Histo – Granulation tissue of giant cells surrounding clefts created by cholesterol granulation • Treatment – Surgical excision & complete removal of granulation
  48. 48. INFECTIOUS SYSTEMIC DISEASES BACTERIAL
  49. 49. Rhinoscleroma • Chronic granulomatous disease of the resp tract – Nose; larynx; trachea & bronchi • Klebsiella rhinoscleromatis • Occur @ any age in either sex – > common in middle aged females • Central & South-eastern Europe; North Africa; Indian subcontinent; Indonesia; South America
  50. 50. Rhinoscleroma • 3 stages – Catarrhal stage • Foul-smelling purulent rhinorrhoea (weeks – months) – Atrophic stage • Large, foul-smelling nasal plaques or crusts • Same as lesions in atrophic rhinitis – Granulomatous or proliferative stage • Multiple granulomatous, non-ulcerative nodules throughout nose, pharynx, larynx, trachea & bronchi • Bluish red & rubbery and later paler & harder – Cicatrizing stage • Scaring, adhesions, stenosis & distortion of normal anatomy
  51. 51. Rhinoscleroma
  52. 52. Rhinoscleroma • Histo – Scattered large foam cells (Mikulicz cells) • Vacuolated cells with a central nucleus • Contain the indigestible bacilli & Russel bodies • Diagnosis – Clinical features – Microbiology – Histological features
  53. 53. Rhinoscleroma • Treatment – Treatment must be intense & prolonged – Streptomycin 1g/day + Tetracycline 2g/day x 4/52 – Continued till 2 x consecutive negative cultures – Rifampicin, Bactrim & Ciprofloxacin – Surgical debridement prior to A/b in granulomatous stage – Surgery to “core out” scar tissue in Cicatrizing phase
  54. 54. Tuberculosis • Mycobacterium tuberculosis • Uncommon in nose • More common in cervical LN, pharynx, larynx & ear
  55. 55. Tuberculosis • Nasal involvement – Lupus Vulgaris • Begins in vestibule, then extends to adjoining skin & mucosa • Apple jelly nodules (Does not blanch) • May form ulcers with undermined edges
  56. 56. Tuberculosis • Lupus vulgaris
  57. 57. Tuberculosis • Nasal involvement – Lupus Vulgaris • Begins in vestibule, then extends to adjoining skin & mucosa • Apple jelly nodules (Does not blanch) • May form ulcers with undermined edges – Ulcerative form • Involves cartilaginous nasal septum or inferior turbinate • Nasal floor is spared • Nasal obstruction, pain, discharge, crusting, epistaxis • Septal perforation, but not dorsal saddling – Sinus granuloma • Soft tissue mass with/without bone destruction
  58. 58. Tuberculosis • Sinus granuloma – Sino-nasal mass – Surrounding infiltration – Bone destruction
  59. 59. Tuberculosis • Laryngeal involvement – Occurs in association with pulm TB – Dysphonia, Dysphagia, Otalgia – Diffusely oedematous & reddened larynx • Predominantly affects post ⅓ of larynx • May have ulceration (confused with Sq cell CA)
  60. 60. Tuberculosis • Laryngeal involvement
  61. 61. Tuberculosis • Diagnosis – Histology (AFB) – CXR – PCR & BACTEC • Treatment – As other TB
  62. 62. Nontuberculous Mycobacteria • Mycobacterium fortuitum • Mycobacterium scrofulaceum • Mycobacterium szulgai • Mycobacterium xenopi • Mycobacterium bovis
  63. 63. Syphilis • Spirochaete: Treponema pallidum • Can affect all ages and both sexes equally • Primary – Infectious!! – Chancre / hard non-painful ulcerated nodule – Self limiting (6 – 10 weeks) – Diff: furunculosis / malignant neoplasm
  64. 64. Syphilis • Secondary – Most infectious!! – Persistent catarrhal rhinitis – Persistent crusting or fissuring of vestibule – Mucous patches on tongue, gingiva, pharynx – Rarely larynx • Diffuse erythematous papules on larynx – Epiglottis & aryepiglottic folds
  65. 65. Syphilis • Secondary
  66. 66. Syphilis • Tertiary – Luckily much less infective…. – Nose • Pain (always worse @ night), swelling & obstruction • Tenderness over nasal bridge (characteristic sign) • Gumma – Begins as a subcut nodule  punched out destructive ulcer • Bony portion of septum most comonly involved
  67. 67. Syphilis • Tertiary – Ulcerated gumma – Cicatrization of bony nasal dorsum
  68. 68. Syphilis • Tertiary – Luckily much less infective…. – Larynx • Nodular infiltrates coalescing to painless ulcers – Epiglottis & aryepiglottic folds • Appearance = similar to TB & CA – Ear • SNHL, OM, Meniere’s symptoms
  69. 69. Syphilis • Diagnosis – TPHA/VDRL / RPR – Biopsy in doubtful cases • Treatment – Parenteral Penicillin
  70. 70. Leprosy • Hansen’s disease • AFB: Myrobacterium leprae • Epidemiology – 12 – 15 million people affected world wide – Brazil, India, Indonesia, Myanmar, Nigeria: 82% of cases – Commonly manifests ages 10-20 years – Nasal d/c = principal route of transmission
  71. 71. Leprosy • 7th WHO Expert Committee on Leprosy – Hypopigmented or reddish skin lesion w definite loss of sensation – Involvement of peripheral nerves (thickening or loss of sensation – Skin smear + for AFB,s
  72. 72. Leprosy • 2 distinct polar forms of disease w spectrum in between – Tuberculoid Leprosy: • Strong host resistance • Non-infective • Localized – Lepromatous Leprosy • Poor host resistance • Infective • Systemic with widespread involvement of tissues
  73. 73. Leprosy • Nasal involvement – Tuberculoid Leprosy • Solitary skin lesion w cutaneous anaesthetic patches • Vestibule skin involvement, but no mucosal involvement – Lepromatous Leprosy • Skin, nerve & mucosal involvement • Nasal obstruction, crust formation & blood-stained d/c – Nasal d/c contain AFB’s • Advanced: Atrophic Rhinitis, septal perforation & dorsal saddling
  74. 74. Leprosy • Laryngeal involvement – Only with concurrent systemic illness – Principal area = supraglottis • Epiglottis – nodular oedema & ulceration – Present w dysphonia (muffled voice)
  75. 75. Leprosy • Diagnosis – Clinical – Bacteriological examination: Nasal d/c or scrapings of nasal mucosa – Histology • Treatment – Dapsone (Single drug treatment  resistance) – Rifampicin & clefozamine – acts rapidly – Triple therapy = best results
  76. 76. Actinomycosis • Anaerobes: Actinomycoses bovis / israeli • Chronic suppurative disease • Poor dental hygiene • Soft tissue involvement of submandibular or cervical regions – Abscess & sinus tract formation • Secondary laryngeal involvement – Erythematous, swollen wooden larynx
  77. 77. Actinomycosis • Diagnosis: – Microbiology – Classic “Sulphur Granules” on biopsy • Treatment – Penicillin – Tetracycline
  78. 78. INFECTIOUS SYSTEMIC DISEASES FUNGAL
  79. 79. Histoplasmosis • Histoplasmosis capsulatum • Granulomatous fungal disease • Soil from chicken houses / bat & bird faeces • Extremes of ages • Mostly Larynx & tongue, but also nose & paranasal sinuses
  80. 80. Histoplasmosis • Always accompanied pulm involvement – Cough, chest pain, hoarseness Diffuse miliary Chronic cavitating
  81. 81. Histoplasmosis • Tongue & Larynx more commonly affected • Either nodules or ulcers
  82. 82. Histoplasmosis • Tongue & Larynx more commonly affected • Either nodules or ulcers
  83. 83. Histoplasmosis • Diagnosis – Biopsy • Epitheloid / histiocytic granuloma • Organism seen with methenamine silver nitrate stain • Treatment – Amphotericin B
  84. 84. Rhinosporidiosis • Rhinosporidium seeberi • Asia & Africa • Immersion in contaminated waters • Nasal mucosal lesions – Initial: flat & sessile lesion – Later: Painless polypoid growth filling the nasal cavity
  85. 85. Rhinosporidiosis • Clinical manifestation – Painless slow growing nasal mass • Friable, polypoid and vascular • Strawberry appearance – Nasal obstruction, watery rhinorrhoea
  86. 86. Rhinosporidiosis • Diagnosis – Histology • Pseudoepitheliomatous squamous metaplasia overlying multiple globular cysts (sporangia) • Prominent accompanied granulomatous reaction of fibrosis tissue • Treatment – No medical management – Complete surgical excision
  87. 87. Aspergillus/Mucor • Fungal Rhinosinusitis • Usually in immune-compromised individuals • Non-invasive fungal sinusitis / rhinosinusitis • Invasive fungal rhinosinusitis
  88. 88. Aspergillus/Mucor • Non-invasive fungal sinusitis / rhinosinusitis – Fungus ball • Only described in adults in the literature • Immunocompetent patients • Aspergillus most common • Tangled mats of hyphae in one or more sinuses • Extramucosal disease • Maxillary sinus  sphenoid sinus  other sinuses • Symptom = Post-nasal d/c , asymptomatic • CT: heterogeneous opacification • Surgical removal by endonasal under endoscopic guidance
  89. 89. Aspergillus/Mucor • Non-invasive fungal sinusitis / rhinosinusitis – Fungus ball
  90. 90. Aspergillus/Mucor • Non-invasive fungal sinusitis / rhinosinusitis – Allergic fungal sinusitis • Immunocompetent patients • Younger patient group < 30years • Hypersensitivity to fungus residing in mucous • Dematiaceous species • “allergic mucin” – eosinophilic mucin with or without fungus • Unilateral/Bilateral polyps with complete opacification of sinus cavity on CT – Frequently bony expansion • Diagnosis – Presence of hyphae in mucin • Treatment – Prednisone, nasal steroids & removal of all mucin
  91. 91. Aspergillus/Mucor • Non-invasive fungal sinusitis / rhinosinusitis – Allergic fungal sinusitis
  92. 92. Aspergillus/Mucor • Invasive fungal rhinosinusitis – Chronic or indolent invasive fungal rhinosinusitis • Rare • Mostly in immuno-competent patients • Aspergillus most frequent • Pain = Main symptom • Chronic headache, proptosis, cranial nerve deficits • Maxillary sinus most commonly affected • Bony erosion extending to orbit or skull base
  93. 93. Aspergillus/Mucor • Invasive fungal rhinosinusitis – Acute fulminant fungal rhinosinusitis • Immuno-compromised patients • Fatal outcome without prompt treatment • Mycotic infiltration of mucosa & sinuses • Initially: Fever of unknown origin or rhinorrhoea • Later: Proptosis, opthalmoplegia, CN palsies • Most common site: near middle turbinate, septum • Thrombosis & necrosis
  94. 94. Aspergillus/Mucor • Invasive fungal rhinosinusitis – Acute fulminant fungal rhinosinusitis
  95. 95. NEOPLASTIC
  96. 96. T/NK cell Lymphoma • “midline destructive granuloma” • Classical destruction of the midface • Similar appearance of aggressive midfacial destruction by cocaine abuse • All decades of life (mean = 50years) • Both sexes • Association with EBV
  97. 97. T/NK cell Lymphoma • 3 stages: – Prodromal • Persistent nasal obstruction & rhinorrhoea – Second stage • Necrosis around nasal cavity • Purulent nasal d/c, crusting, tissue loss – Terminal stage • Haemorrhage & gross mutilation of face • Eventually lead to death
  98. 98. T/NK cell Lymphoma
  99. 99. T/NK cell Lymphoma • Diagnosis – Good quality representative biopsy beneath the slough & crusts • Imaging – Dramatic destruction of midline soft tissue & bone without a gross tumour mass – Treatment • Radical full course radiotherapy 55 Gy • Wide field coverage incl nose, sinuses & palate

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Granulomatous conditions in ENT are rare conditions that we come in contact with, we tend to overlook them because they are so rare, however some of the conditions like TB and syphillis and Mucormycosis of the Nose and PNS are seen in our clinics this is a good summary from scotts brown chapter

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