Rhinology Systemic Disease Flashcards
What are the three primary granulomatous
diseases that can involve the nasal airway?
● Sarcoidosis
● Wegener granulomatosis (granulomatosis with polyangitis)
● Churg-Strauss syndrome
What is the classic clinical triad seen in Wegener
granulomatosis?
● Necrotizing granulomas of the respiratory tract (lung
effected most commonly)
● Vasculitis
● Glomerulonephritis
Describe the otolaryngologic manifestations of
Wegener disease.
● Sinonasal: Obstruction, rhinorrhea, crusting, sinusitis,
epistaxis, septal perforation, saddle-nose deformity
● Otologic: Conductive hearing loss with serous otitis
media; less commonly, sensorineural hearing loss and
vertigo
● Subglottic: Stridor, dyspnea, subglottic stenosis, crusting
What are the nasal manifestations of Wegener
granulomatosis?
Nasal inflammation, rhinorrhea, nasal crusting and puru-
lence, CRS, septal perforation
The antibodies used for diagnosis of Wegener
granulomatosis are directed at what protein?
PR3
What blood tests are highly sensitive for Wegener
granulomatosis but if negative do not completely
exclude the diagnosis?
c-ANCA, PR3 (most typical for Wegener, granulomatosis
with polyangitis (GPA)
Anti-myeloperoxidase (AMO); possibly more common with
microscopic polyangiitis (MPA)
What pathologic findings are seen on biopsies
of a patient with Wegener granulomatosis?
● Necrotizing, noncaseating multinucleated giant cell granulomas ● Small and medium vessel vasculitis ● Microabscesses Note: Biopsies are often nondiagnostic.
What are the three systemic medications used
to treat Wegener granulomatosis?
● Cyclophosphamide (used in severe cases)
● Methotrexate (used in limited Wegener granulomatosis)
● Glucocorticoids
What is the cause of sarcoidosis?
Unknown. There is the potential for a genetic susceptibility
that is triggered by exposure to an antigen (bacteria, virus,
dust, etc). African Americans are 10 to 20 times more likely
to be affected than are whites.
What is the most common nasal symptom
associated with sarcoidosis?
Nasal obstruction
Other symptoms include epistaxis, epiphora, nasal pain,
and dyspnea. Nasal symptoms are present in < 5%.
Where does sarcoidosis most commonly manifest
in the nose?
Septum and inferior turbinates and commonly involved
with thick crusting. At later stages, yellow subcutaneous
nodules can be seen.
What is the typical disease course of sarcoidosis?
● Spontaneous resolution within 2 years of disease onset
● ~ 10% → Progressive disease and pulmonary fibrosis
What is the key histopathologic characteristic
seen on nasal biopsy in a patient with
sarcoidosis?
Noncaseating granulomas
What laboratory test can be used in the diagnosis
of sarcoidosis?
Serum angiotensin-converting enzyme (SACE)
What percentage of patients with active
sarcoidosis will have a positive SACE test?
~ 80%
How is sarcoidosis most commonly systemically
managed?
Medically with one or more of the following:
● Corticosteroids
● Antirejection medications
● Antimalarial medications
● Tumor necrosis factor (TNF)-α inhibitors
Rarely organ transplant can be considered.
What is the treatment for sinonasal sarcoidosis?
Nasal saline irrigations and topical nasal steroids. Some
advocate intralesional steroid injections. In acute exacer-
bations of aggressive disease, systemic corticosteroids
should be administered.
What is the clinical triad associated with
Churg-Strauss syndrome?
● Asthma
● Systemic vasculitis
● Eosinophilia
What are the three phases of Churg-Strauss
syndrome?
● Prodromal or allergic phase: Adult onset asthma and
allergic rhinitis
● Eosinophilic phase: Peripheral eosinophilia with variable
organ involvement
● Vasculitic phase: Systemic vasculitis
What are the criteria for the diagnosis for
Churg-Strauss Syndrome?
Four of the following must be present in a patient with
known vasculitis:
● Asthma
● More than 10% eosinophils in peripheral blood
● Neuropathy
● Pulmonary opacities on chest X-ray
● Sinonasal disease
● Biopsy of blood vessel showing eosinophil accumulation
What is the management of sinonasal
Churg-Strauss disease?
Nasal saline irrigations and topical nasal steroids, antibiotics
as needed
What are the two most common causes of
midline granuloma syndrome?
● Wegener granulomatosis
● T-cell lymphoma
Improved diagnostic techniques have largely proven that
these two are the only true diagnostic entities. Other older
terms, such as malignant midline granuloma, lethal midline
granuloma, and others, generally should not be used).
Describe the typical clinical presentation of T-cell
lymphoma as a midline granuloma.
● Most common in Asian men
● Associated with Epstein-Barr virus
● Initial symptoms include nasal obstruction, drainage,
pain, epistaxis, fatigue, weight loss, and potentially night
sweats. May demonstrate external midface destructuring
with advanced disease.
What criteria are needed to make a diagnosis of
relapsing polychondritis?
Michet et al criteria:
● Chondritis of two of three sites (auricular, nasal, or
laryngotracheal) or
● Chondritis of one of the above-listed sites, with two other
features (ocular inflammation, seronegative inflamma-
tory arthritis or vestibular dysfunction, hearing loss)
What are the nasal symptoms commonly seen in
48 to 72% of patients with relapsing
polychondritis?
● Acute and painful chondritis
● Sensation of fullness over the nasal bridge
● Mild epistaxis
● Long-standing disease → saddle-nose deformity
What are the treatment options available for
relapsing polychondritis?
● Immune-suppressing medications: Corticosteroids, dap-
sone, azathioprine, cyclophosphamide, cyclosporine,
penicillamine
● Plasma exchange
● Airway surgery: Tracheostomy, tracheal stent placement,
etc.
● Reconstructive surgery: Rhinoplasty for saddle nose can
be considered if disease is well controlled.
What are the clinical manifestations of anhidrotic
ectodermal dysplasia?
Abnormal development of the skin, hair, nails, teeth, and
sweat glands
What is the method of inheritance associated
with the most common form of anhidrotic
ectodermal hypoplasia?
X-Linked recessive
What triad is associated with anhidrotic
ectodermal hypoplasia?
Anhydrosis, hypotrichosis, and anodontia
Patients often have hypotrichosis, hypodontia, eczema, and
atrophic rhinitis.
What percentage of patients with primary ciliary
dyskinesia will have situs inversus?
50%. May also have recurrent sinopulmonary infections and
infertility
Nasal biopsy followed by electron microscopy
looking at the ultrastructure of the cilia is a useful
test when looking for what disorder?
Ciliary dyskinesia. Looking at the number of inner and outer
dynein arms and evaluating ciliary orientation can assist in
differentiating primary from secondary causes.
What nasal disorder results from hypertrophy of
the sebaceous glands in both the nasal skin and
fibrosis?
Rhinophyma
Describe two early signs of rhinophyma.
● Dilated (patulous) pores
● Telangiectatic vessels on the distal nose
Rhinophyma may manifest as the final stage of
what other skin disease?
Acne rosacea, although not all patients with rhinophyma
have a history of rosacea
What malignant condition can be associated
with rhinophyma?
Basal cell carcinoma
What patient populations are affected by
rhinophyma?
Although acne rosacea is more common in women (3:1)
compared with men, rhinophyma almost always affects
men (30:1). The disease typically afflicts white males in
their 50s to 70s.
How is rhinophyma managed?
Inflammation can be managed conservatively, similar to
rosacea. For significant hypertrophy, deformity, and nasal
obstruction, surgical recontouring can be performed using
most commonly a carbon dioxide laser with or without
dermabrasion.
Rosacea is a chronic skin disorder that can appear
with erythematotelangiectatic, papulopustular,
phymatous, or ocular characteristics and is thought
to be related to what underlying cause?
● Immune disorder
● Inflammatory reaction to infection (e.g., Demodex folli-
culorum, Bacillus olenorium, Helicobacter pylori)
● Other: UV radiation, vascular hyperreactivity, family
history
What is the infectious cause of rhinoscleroma, an
infectious granulomatous disease that is endemic
to Africa, southeast Asia, and Central America and
affects the nasal cavity and occasionally the larynx,
paranasal sinuses, and nasopharynx?
Klebsiella rhinoscleromatis (Frisch bacillus)
What are the clinical stages associated with
rhinoscleroma?
● Catarrhal (atrophic): Purulent rhinorrhea, rhinitis, honey
nasal crusting
● Granulomatous (hypertrophic): Painless granulomatous
nodules in the upper respiratory tract
● Sclerotic: Healing with extensive scar tissue formation
and nasal stenosis
Describe the histologic findings associated with
rhinoscleroma.
Mikulicz cells (foamy histiocytes), and Russel bodies (intra-
cellular inclusions associated with excessive immunoglobu-
lin synthesis)
What is the management of choice for treating
rhinoscleroma?
Long-term culture-specific antibiotics and surgical debride-
ment
What granulomatous infection is endemic to
Africa, Pakistan, Sri Lanka, and India and manifests
with strawberry-red, friable, polypoid lesions of
the nose and eye causing nasal obstruction and
epistaxis?
Rhinosporidiosis
What is the agent responsible for rhinosporidiosis,
and what is the primary means of transmission?
● Rhinosporidium seeberi
● Contaminated water
What is the classic histopathologic finding(s)
associated with rhinosporidiosis?
Fungal sporangia with chitinous elements in the setting of
pseudoepitheliomatous hyperplasia and submucosal cystic
structures
How is rhinosporidiosis managed?
Surgical excision with close follow-up for recurrent lesions
Note: Long-term treatment with dapsone has been effec-
tive. Other treatments that can be tried are local steroid
injection, and antifungal agents.
