Special Forms of Rhinosinusitis - orl-hno.ch · or aerobic/anaerobic) or invasive fungal sinusitis...

Special Forms of RhinosinusitisVasculitis, Autoimmune Disease, Immuno-deficiencies…

Patrick Dubach, Chefarzt HNO Klinik Buergerspital Solothurn

Konsiliarus Universitätsklinik HNO, Kopf- und Halschirurgie Inselspital Bern

Klinik für HNO-, Kopf- und Halschirurgie

Team Approach for Special Cases!

AUTO? IMMUNO? Factor X?

• Alternative Diagnosis: Auto-Immune,

Immundeficency, atypical rare infections,

Neoplasm……

Nose, Ear, Larynx:

Polychondritis?Sinus, Middle

Ear, Mastoid:

Vasculitis?

Oral mucosa:

Lupus, Crohn?

Salivary Glands:

Sjögren,

Sarcoidosis?


SUMMARY:

• …in case of abnormal prolongated or repetitive

rhinological disease

• RED FLAGS: Think of special forms of RS and

check

–Patients History

–? Autoimmune diseases (rheumatologic disease,

immunosupressants, disorder of immune system)

–? Infections (atypical organism, co-infection (HIV))

–? Neoplasia


Schedule

• The BIG THREE of autoimmune GRANULOMATEOUS

diseases for the rhinologist

Not in detail:

• Atypical Infection and Fungal RS

• The immuno-supressed patient

IATROGENIC IMMUNOSUPPRESSION

(transplantion or rheumatologic patients)

UNCONTROLLED DIABETES, renal and

hepatic insufficiency


•40 year old male patient

•suffering from stuffy nose and nasal crusts

since years

•presented with discrete bilateral epistaxis

and intermittant unilateral epiphora

•Chronic cough and fatigue

Case 1: Bleeding Crusts


Circular Stenosis


Ulcerated Crusts


Granulomatosis with Polyangiitis GPA(AKA Wegener Granulomatosis)

• 50-100 per million Europeans

• „Autoimmune disease“ with histological triad:

–Necrosis (46%), granuloma (42%), vasculitis (16%)

–Cave: ENT biopsy in 50% suggestive/in 10% classical

• Staging possibilities

–ELK staging system (organs with high vascularization)

–E: ENT, L:Lung, K: Kidney

–„Limited“ vs Generalized/Systemic Disease

–„Vasculitic“ (GN, pulmonary hemorrage) vs

„Granulomatous“ (ENT, pulmonary nodules)


American College of Rheumatology GPA Criteria

• 2 out of 4 criteria – separates GPA from other vasculitis with Sensitivity of 88% and Specitiy of 92%

• Nasal or oral inflammation

• Abnormal chest radiograph (nodules, fixed infiltrates or

cavities)

• Abnormal urinary sediment (microscopic hematuria with or

without red blood cell casts)

• Granulomatous inflammation in perivascular areas on

biopsy

• Leavitt RY et al. The American College of Rheumatology 1990 criteria for the

classification of Wegener‘s granulomatosis. Arthritis Rheum 1990;33:1101


0

10

20

30

40

50

60

70

80

90

100

Clinical Presentation (n=62): ENT Bern

WG

non-WG

GPA Leading Symptoms

Borner U et al Am J Rhinol Allergol 2012:475-480.


Saddle nose: 28% (Holle et al 2010)

• 29.9.


Septal Perforation:

24% (Holle et al 2010)

Biopsy if focal inflammed target

i.e. directed and deep (i.e. bleeding)

Biopsies… you want vessels…


SUBGLOTTIC STENOSIS


Midline Destructive Lesions: What disease is this?

1….

2……

3……


•48y old nurse suffering from nasal

obstruction and anosmia since 2 years

•Polypectomy and sphenoethmoidectomy but

recurrent nasal block by polyps within 3

months

•Since 10y: Bronchial asthma

•Blood eosiniophilia >10%

Case 2: The hydra of nasal polyps


Churg Strauss


–1956 : 13 mysterious cases

sharing the triad:

Eosinophilic infiltrations,

granuloma and necrotizing

vasculitis of small and

medium size arteries

–Asthma followed by blood esinophilia and fever

(Localization: pneumonia, mononeuritis multiplex, carditis)


American College of Rheumatology EGPA:Eosinophilic Granulomateous Polyangiits AKA Churgh Strauss Disease

• Four out 6 criteria gives EGPA diagnosis with sensitivity

85% and specificity of 99.7%

• Asthma

• Transient pulmonary opacities

• Blood eosinophilia >10% or 1500/mm3

• Mononeuritis (multiplex)

• Paranasal sinus abnormalities

• Biopsy with eosinophilic paravasal infiltrates

• Masi AT et al. The American College of Rheumatology 1990 criteria fo the Classification

of Churg Strauss Syndrome. Arhtritis Rheum 1990;33:1094

Alternative Lanham Criteria with Asthma, Eos1500/ul and vasculitis in 2 or more extrapulmonary organs


EGPA: Clinical Stages

• Prodromal Phase: 2nd and 3rd decade

–Atopic disease: Allergic rhinitis (50% with polyps) and asthma

• Eosinophilic Phase:

–Peripheral blood esinophilia and inflitration of multiple organs

• Vasculitic Phase: 3rd and 4th decade

–Vasculitis with paravasal eosinophilic granuloma and

necrosis

• Red flags: Eosinophilic Myocarditis, Glomerulonephitis


EGPA:

• 10 bis 15 per million Europeans

• No gender preference

• Third frequent ANCA associated vasculitis after

GPA and microscopic polyangitis

• Histological Trias: Vasculitis, esosinsophilc tissue

infiltrates and granuloma


EGPA Manifestationen

• Pulmonary 90% (Nodules, migrating infiltrates)

• Skin 66%: Subcutaneous nodules (granuloma) and

palpable purpura (vasculitis), livedo reticularis

• Upper Airway (and ear) 48%: Chronic rhinosinusits (60%

with polyps in case series n=29)

• Heart 15-30%: CARDITIS responsible for 50% of deaths by

EGPA: → echocardiographia and ECG (heart failure and

arrythmia)

• Mononeuritis (multiplex) 75% and Glomerulonephritis 22%


Case 3: „GRANULATING“ NODULES

• 60 y old female

farmer‘s wife

• with blocked nose,

anosmia,

• chronic otitis

media

• stigmatized in the

village by her

nose


Chronic Otitis media


Sarcoidosis

• DIAGNOSIS OF EXCLUSION of the granulamtous

diseases (GPA, specific infection (fungi, Tbc/MAI,

Leishmaniosis, foreign body granuloma)

Can involve every organ

• Rhinosinusitis, dacryostenosis 10%

• Sialadenosis e.g. Heerford Syndrom (febris uveo-

parotidea)

• Cutaneous: Lupus Pernio, Erythema nodosum (25%)


§

Granulomatous Dacryoadenitis


Palatal Fistula


oAffection of Olfactory Cleft: Hyposmia


Sarcoidosis

• 100 to 200 per million(blacks 3-4x more frequent)

• Mulitsystemic granulomatous disease of unknown origin

Histologically characterized by non-caseating granulomas

• Typically manifested in young adults

• Lung >90% affected (50% subclinical presentation)

–Hilar lymphadenopathia

–Pulmonary reticular opacities

• Extrapulmonary sarcoidosis:

–Mostly Skin, eyes, reticulendothelial and musculosceletal

system, 10% also nervous system 5% heart disease.


Extrapulmonary Sarcoidosis

• Reticuloendothelial system commen: Lymphadenopathia

40%, hepato-splenomegalia 20%

• Skin 25%: Papules, plaques, erythema nodosum with e.g.

Löfgren Syndrome

• Eyes 20%:Uveitis, iritis

• URT involvement rare 10%: CRS and Laryngitis

• Renal (50% Calci-uria) and Neurological 4% (basal

meningitis)

• Exocrine glands 4%: Xerostomia, Xerophtalmia

• Bone: Zysts


Synopsis

• Sarcoidosis

• No ANCA

• Urine Ca ↑

(up to 40%)

• WBC normal

• Hilar

Lymphadeno

• ACE↑ in 50%

• Serum-Ca↑ in

10%

• EGPA

• p-ANCA

• Urine may be

abnormal

• > 10% Eos

• Pulmonary

infiltrates

• ACE normal

• Serum-Ca

normal

• GPA

• c-ANCA

• Urine red

casts and

proteine

• Tc and Lc ↑

• Pulmonary

nodules

• ACE normal

• Serum-Ca

normal


Therapy:

• Treatment of underlying systemic diseasse

(rheumatologist, nephrologist), rule out specific infections

(fungi, tbc…) and tumor

• Nasale irrigations (NaCl, Babyshampoo,…)

• Bactrim prophylaxis

• Topical steroids

• Systemic: Prednison, cyclophosphamide

• Azathioprine or methotrexat

• Biologicals….rituximab (moab to deplete B-cells)

• Surgery only if not avoidable (DCR,saddle nose correction)


Differential Diagnosis: Spezific

Rhinitis on infectious grounds

• Bilder aus www.google.ch/images


Spezific Rhinitis-Rhinosinusitis

Diagnose:



Spezifische Rhinitis-Rhinosinusitis



Spezific Rhinitis-Rhinosinusitis

• Eg Leshmaniasis

–Protozoon (transmitted by sandflies, host rodents,

marcrophages, endothelial cells)

– Lesions are defined by affected tissue types and host

response

•Leishmaniasis of the old world (Mediterranian area,

Alepobeule): superficial cutaneous form

•Viszerale form (Kala-Azar in India)

•Mukocutenous form (Espundia in Central- and South

America)


Spezifische Rhinitis-RhinosinusitisDiagnostics: microscopic

visualization of protozoon in

macrophages and endothelial

cells

Th: Antimon, Amphotericin B

Bild aus www.google.ch/images


• 50 000 species, only 300 pathogenic in humans

• Acute vs Chronic (longer than 12 weeks) infections

Non-invasive

• FUNGUS BALL («Truff», Aspergillom, Mycetom)

• ALLERGIC FUNGAL RHINOSINUSITIS: RARE (moist and

hot regions)

Invasive ((vs non-allergic eosinophilic RS))

• ACUTE INVASIVE: MUCOR (Aspergillus in case of acute

leucemia)

• CHRONIC INVASIVE: RARE (Granulomatous in dry hot

regions) and non-granulomatous form

Fungi


Chronic Mycetoma

• Left maxillary sinus: Typical brown fungus ball and

radiopaque inclusion on Rx (Mn, Mg, Hg,.. )

• Aspergillus fumigatus (75%), flavus (7%), Chrysosporium


ACUTE INVASIVE FUNGAL SINUSITIS

Cavernous sinus thrombosis by Mucor


Mucormycosis

• Immuno-compromised host (reduced innate immune

system)

–Ketoacidosis, severe neutropenia

• Molds on plants, angiotropism (invasion, thrombosis)

• Clinical signs:

• Pain, Necrosis (Eschar of the palate)

• GET TISSUE FOR HISTOLOGY: Angio-invasion and

thrombosis by Mucor (culture only in 50% later positive)

• Therapy: «Face-ectomy», Amphotericin B iv, correction of

underlying disease


Allergic fungal Rhinosinusitis

• Warm and hot climate (Saudi Arabia, Southern US)

• First detected in: Early recurrent polyposis, peanut butter

mucus (ie. eosinophilic mucin) in combination with Allergic

bronchopulmonary Aspergillosis with similar mucus plugs

in the lung.

• Diagnostic criteria

– Gell & Coombs type I hypersensitivity (history, skin, or serology)

– Polyposis

– CT findings

– Eosinophilic mucus;

– Positive fungal stain and no fungal invasion

Bent, Kuhn, Oto-HNS 1994;111:580-88

Meltzer, Hamilos, Hadley, Lanza, Marple, et. al. JACI 2004;114:S155-S212.


AFR: Serpigineous opacifications and polyps

source:

www.radiopaedia

Hyperdense

mass

in the sinus

and classical

serpiginous

hypodens rim


Allergic Mucin (Peanut butter)

• Gross findings -indistinguishable from ABPA

– Thick viscosity

– Tan, black, green

• Histology– Non-invasive fungus

• Grocott

• GMS

• H&E stains show

– Inflammatory cells

– Eosinophils

– Charcot-Leyden Crx


Treatment: (Caveat high recurrency rate)

• Establishing diagnosis

• Extensive surgery removing all «mucus plugs» and

creating large sufficient fenestration for gravity dependent

self clearance

• Aggressive post-op care with saline irrigation, office based

cleaning

• Long time steroids (duration controversial)

• Controversial: Antifungal (Azole) rinsing, immunotherapy


Defects of the Immune System: Primary Disease

• Innate system: Mucosal barriers, natural killer cells,

complement system

–Cystic fibrosis, primary cilliary dyskinesia

• Humoral System: 2/3 of defects of the immune system are

Immunglobuline associated diseases:

–Common Variable Immunodeficiency CVID (IgGs): variable

weakness for URT and GIT infections

–Selective IgA deficiency: 70% URT infections, otitis and

parotitis (rarely life threatening)

–Transiente Agammaglobulinemia of the newborne 6-

12months (3-6m to 2-3y) explains propensity to URT and GIT

infections

• Cellular and Combined Cellular/Humoral Defects


Secondary (Aquired) Immunodeficiencies

• Diabetes Mellitus, Hypothyreosis, Malnutrition

• Bone Marrow Transplantation: Brief but very serious

immunosuppression with bone marrow ablation

–Extremly sensitive to acute bacterial sinusitis (gram negative

or aerobic/anaerobic) or invasive fungal sinusitis

–Mucositis, lympadenopathia (tuberculosis)

• Solid Organ Transplantation: Delayed suppression of cell-

mediated immunity: Mostly established at 4w to 6m

–Viral infections EBV, CMV, HIV, Hep B and C

–Sinusitis (invasive fungal sinusitis is much rarer)

–Pneumonia


Aquired Immunodeficiencies

• Radiotherapy: Disruption of mucosal barriers and impaired

cellular immune response:

–Stomatitis (Candida in Xerostomia, reactivated HSV 1)

–Susceptibility to dental caries and gingivitis

• Chemotherapy: Direct effects on immune cells

–Stomatitis

–Leucocytes <500/cc

–Fungal infection especiall following anibiotic therapy

(Candida sepsis, Angioinvasive or pulmonary Aspergillosis,

Mucor rhino-orbito-frontal invasion)


Iatrogenic Immunosuppressant

• Mostly due to treatment of patient with vasculitis and

rheumotologic patients

• Prednisone 40-60mg/daily longer than 4 to 6 weeks

• Antiproliferative agents for B- and T- lymphocytes:

Methotrexat, Cyclophosphamide, Azathioprine,

Mycophenolate, Biologicals

• Immunophilin antagonist interfering with intracellular signal

transduction: Cyclosporin


Defects of the immune system: HIV Infection

• Look for untreated patients or acute infection

• 41-68% ENT manifestations (especially in acute infection)

• 70% generalized Lymphadenopathia and Waldeyer ring

hyperplasia

• Oral: 10% Ulcera (VZV with prodromal itching, CMV)

• HPV Papillome, mucosal Kaposi Sarkoma painfull blue-

livid nodules (Th: Surgery, Vinblastin injections, radiation)

• Oral hairy leukoplakia

• Candida mucositis (various forms: pseudomembranous,

erythematous, angular cheilitis, leukoplakia)


Acquired defects of immune system: HIV Infection

• Sinusitis only in 11-17%

–Adenoidhyperplasia and nasal obstruction (early stage)

–Sinunasal Kaposi Sarcoma DD: bacilliary angiomatosis

• External otitis with Pseudomonas, Aspergillus

• Ear canal Polyps by Tbc, Pneumocystis

• Parotid gland: Lymphoepithelial Cyst(s) DD: Sjögren

• Cave CD 4 Helper Cell Count <200/cc

• Laryngooesophagal Candidiasis, high indicence of chronic

RS, baciliary angiomatosis (Bartonella related)


SUMMARY AGAIN:

• ….in case of extremly prolongated or repetitive

disease

• RED FLAGS: THINK OF IT…AND CHECK

–Patients history: COCAINE, travel history, medication

–Autoimmune:

•c-/p-ANCA, urine sediment, creatinine, glucose

–Infections:

•Low threshold for biopsy: CULTURE AND HISTOLOGY

–Neoplasia (NK- T cell) lymphoma

•Deep biopsies in OR

Special Forms of Rhinosinusitis - orl-hno.ch · or aerobic/anaerobic) or invasive fungal sinusitis...

Documents

Transcript of Special Forms of Rhinosinusitis - orl-hno.ch · or aerobic/anaerobic) or invasive fungal sinusitis...