CHAPTER 11: Laryngeal and Tracheal Manifestations of Systemic Disease Flashcards
A multisystem inflammatory disease characterized by necrotizing granulomatosis and necrotizing vasculitis of small arteries, arterioles, capillaries, and venules
Granulomatosis with polyangitis (GPA)/ Wegener granulomatosis (WG)
Areas affected in GPA
- Upper and lower respiratory tracts
- Larynx
- Trachea
- Kidneys
- Oral cavity
- Skin
- Joints
- Orbit
Parts of airway commonly affected in GPA
- Subglottis
2. Trachea
TRUE or FALSE
Most patients with GPA have a positive antineutrophil cytoplasmic antibody (ANCA) test result, with cytoplasmic ANCA proteinase 3 staining being positive with greater frequency than perinuclear ANCA myeloperoxidase staining
TRUE
First line treatment of GPA
Steriods + cyclophosphamide
Treatment of airway obstruction in GPA
- Tracheostomy
- Dilation
- Carbon dioxide laser
- Resection and reanastomosis (mature stenoses and inactive disease)
A rare inflammatory disease of unknown etiology that causes episodic inflammation of cartilaginous structures throughout the body
Relapsing polychondritis
Commonly affected areas in relapsing polychondritis
- Ears
- Nose
- Eyes
- Larynx
- Bronchi
- Costal cartilages
- Articular joints
Peak onset of RP
4th to 5th decade
Most common finding in RP
Bilateral auricle chondritis
Respiratory distress in RP may occur by either of 2 mechanisms:
- Collapse of the central airway from destruction and fibrosis of the laryngeal and tracheal rings
- Peripheral airway narrowing from inflammation and cicatricial fibrosis
CT findings in RP
- Subglottic stenosis
- Tracheobronchial luminal narrowing
- Dense calcification and thickening of tracheal cartilages
- Peripheral bronchial narrowing
- Bronchiectasis
Most common CT finding in RP
Increased attenuation and smooth thickening of the airway walls
Histologic examination in RP
Chondritis with a mixed inflammatory cell infiltrate composed of polymorphonuclear leukocytes, lymphocytes, plasma cells, and eosinophils
Maintenance therapy in RP
- Methotrexate
2. Low-dose corticosteroid
TRUE or FALSE
In addition to surgical measures, RP is managed with high-dose corticosteroids
TRUE
Used in refractory cases of RP
- Azathioprine
- Cyclophosphamide
- Cyclosporine
- Dapsone
- Infliximab (TNF-alpha)
Is a chronic granulomatous disease of unknown etiology, characterized by noncaseating granuloma formation with clusters of tubercles in the same stage of developement, and it can affect any organ system within the body
Sarcoidosis
TRUE or FALSE
Although rare, laryngeal sarcoidosis can be the only manifestation of the disease
TRUE
Progression of RP…
Slow and is characterized by many relapses and remissions
TRUE or FALSE
The supraglottic larynx is affected most often, followed by the subglottis
TRUE
TRUE or FALSE
In RP, the epiglottis is the most common subsite, followed by the arytenoids, aryepiglottic folds, and false vocal folds
TRUE
Classic appearance of the larynx affected by sarcoidosis
“Turbanlike thickening”
Due to the pale, pink, diffuse induration and swelling of the supraglottis
Pathologic findings in RP
Lymphocytic infiltration and noncaseating epitheliod granulomas
Treatment of the laryngeal manifestations of sarcoidosis
Systemic corticosteroid
An idiopathic disorder characterized by extracellular deposition of insoluble fibrillar proteins
Amyloidosis
The deposits in amyloidosis were initially described by…
Rokitansky in1873
TRUE or FALSE
Laryngeal amyloidosis is usually localized and associated with immunoglobulin localized light-chain amyyloidosis (LLA) and rarely found in association with an underlying disorder, such as multiple myeloma
TRUE
Amyloidosis is classified on the basis of 3 parameters:
- Fibrillar protein making up the deposits
- Precursor protein from which the amyloid protein was derived
- Clinical description of the associated disease process
Most common location of deposits within the larynx
True and false vocal folds and the ventricles
Amyloidosis: light microscopy
Acellular, amorphous, homogenous, eosinophilic appearance after H&E staining
Amyloidosis: Congo red
Bright green birefringence under polarizing light microscopy
Treatment of amyloidosis
Maintaining airway patency, improving hoarseness and/or pain while minimizing the effects on voice quality
Amyloidosis treatment: symptomatic
Excision (suspension microlaryngoscopy)
Treatment: Recurrence of airway in amyloidosis
Reexcision and/or radiation therapy
A common autoimmune disease that involves inflammation of the synovial tissue
Rheumatoid arthritis
Commonly involved area in RA
Larynx (25 to 30%)
RA affects the larynx in 2 forms
- Active form
2. Chronic form
RA: larynx is acute with tenderness and erythema
Active form
Laryngoscopic examination in RA active form
Red, swollen arytenoids
RA: cricorytenoid joint involvement with ankylosis
Chronic form
A rare autoimmune disease that affects the skin and mucous membranes
Pemphigus
Loosening of the epidermal-dermal junction leads to the characteristic formation of blebs
Pemphigus
TRUE or FALSE
Pemphigus may be isolated to the larynx, but this is unusual
TRUE
Most common laryngeal presentation in pemphigus
Hoarseness
Fiberoptic exam: Pemphigus
Tan, fibrinous base with halo of erythema
Most common site of pemphigus in the head and neck
Oral cavity mucosa
TRUE or FALSE
In pemphigus, scarring can be prevalent and can lead to supraglottic narrowing
TRUE
Histologic finding in pemphigus
Intraepithelial acantholysis
Direct immunofluorescence in pemphigus
Intracellular pattern
Indirect immunofluorescence in pemphigus
Can measure the amount of circulating antibodies and correlates with disease severity
Treatment of pemphigus
Oral corticosteroids
Maintenance medications for pemphigus
- Azathioprine
- Cyclophosphamide
- Cyclosporine
Etiologic agent of whooping cough
Bordetella pertussis
Treatment of whooping cough
Erythromycin
TRUE or FALSE
Laryngeal TB is the result of hematogenous or lymphatic spread
TRUE
Most common location of laryngeal TB
- Posterior glottis
- Aryepiglottic folds
- Ventricular
- Vocal
TRUE of FALSE
The lesions of laryngeal TB may be nodular, exophytic or ulcerative, sometimes mistaken for SCCA
TRUE
Diagnosis of laryngeal TB
- Sputum culture
- Biopsy
- Chest X-ray
Etiologic agent of Histoplasmosis
Dimorphic fungus Histoplasma capsulatum
TRUE or FALSE
Histoplasmosis manifest as either an acute or chronic pulmonary infection or an acute or chronic disseminated infection with systemic symptoms
TRUE
TRUE or FALSE
Acute disseminated histoplasmosis is almost always fatal
TRUE
TRUE or FALSE
Chronic disseminated histoplasmosis manifest as focally destructive lesions
TRUE
Histologic findings in histoplasmosis
Presence of poorly defined granulomas with macrophages and multinecleated giant cells
Stains can show intracellular Histoplasma capsulatum
- Periodic acid-Schiff
2. Grocott-Gomori methenmaine-silver nitrate
Treatment of histoplasmosis
IV amphotericin B
Etiologic agent of Blatomycosis
Dimorphic fungus Blastomyces dermatitidis
The primary portal of entry of Blastomyces dermatitidis
Lungs
Most commonly affected system in Blastomycosis
Skin
Most common subsite of larynx affected in Blastomycosis
True vocal cords with extension to the ventricular folds
Histologic finding in Blastomycosis
Acute and chronic inflammation, microabscess, and giant cell formation
Hallmark finding in Blastomycosis
Pseudoepitheliomatous hyperplasia
Treatment for uncomplicated Blastomycosis
- Oral ketoconazole
2. Itraconazole
Treatment of Blastomycosis with central nervous system involvement
Amphotericin B
Etiologic agent of Cryptococcosis
Cryptococcus neoformans (yeast-like fungus with a thick polysaccharide capsule)
Most commonly affected system in Cryptococcosis
Central nervous system
Histologic finding in Cryptococcosis
Budding yeast
Treatment of Cryptococcosis
- Amphotericin B
2. Fluconazole
Etiologic agent of San Joaquin Valley fever
Coccidioides immitis
Symptoms of laryngeal involvement of San Joaquin Valley fever
Hoarseness, pain, and odynophagia to airway involvement with stridor
Treatment of Coccidioidomycosis
Amphotericin B
A chronic suppurative infection characterized by the presence of purulent drainage from the chronic discharge of sinus tract that contain sulfur granules
Actinomycosis
Etiologic agent of Actinomycosis
Actinomyces israelii (anaerobic or microaerophilic, non-acid-fast, gram-positive organism)
These organism are endogenous to the oral cavity and can be found in carious teeth, plaque, and tonsillar crypts
Actinomyces israelii
Treatment of actinomycosis
Prolonged antibiotic therapy
- Penicillin (mainstay)
- Clindamycin
Lesion of candidiasis
White sessile plaques on an erythematous base, ulceration may be noted
Therapy of candidiasis
Eliminating the causative agent
Antifungal: Fluconazole
Amyloidosis is classified on the basis of three parameters
- Fibrillar protein making up the deposits
- Precursor protein from which the amyloid protein was derived
- Clinical description of the associated disease process
The most common location of amyloid deposit in larynx
True and false vocal cords and ventricles
Classic findings of amyloidosis under polarizing light microscopy
Bright green birefringence after staining with Congo red
RA affects larynx in 2 forms
- Active form
2. Chronic form
Involvement of the larynx is with tenderness and erythema
Laryngoscopy: red, swollen arytenoids
Active form of RA
Causes cricoarytenoid joint involvement with ankylosis
Chronic form of RA
