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%)