Rhinology Systemic Disease

Question 1

What are the three primary granulomatous

diseases that can involve the nasal airway?

Answer 1

● Sarcoidosis
● Wegener granulomatosis (granulomatosis with polyangitis)
● Churg-Strauss syndrome

Question 2

What is the classic clinical triad seen in Wegener

granulomatosis?

Answer 2

● Necrotizing granulomas of the respiratory tract (lung
effected most commonly)
● Vasculitis
● Glomerulonephritis

Question 3

Describe the otolaryngologic manifestations of

Wegener disease.

Answer 3

● 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

Question 4

What are the nasal manifestations of Wegener

granulomatosis?

Answer 4

Nasal inflammation, rhinorrhea, nasal crusting and puru-

lence, CRS, septal perforation

Question 5

The antibodies used for diagnosis of Wegener

granulomatosis are directed at what protein?

Answer 5

PR3

Question 6

What blood tests are highly sensitive for Wegener
granulomatosis but if negative do not completely
exclude the diagnosis?

Answer 6

c-ANCA, PR3 (most typical for Wegener, granulomatosis
with polyangitis (GPA)
Anti-myeloperoxidase (AMO); possibly more common with
microscopic polyangiitis (MPA)

Question 7

What pathologic findings are seen on biopsies

of a patient with Wegener granulomatosis?

Answer 7

● Necrotizing, noncaseating multinucleated giant cell
granulomas
● Small and medium vessel vasculitis
● Microabscesses
Note: Biopsies are often nondiagnostic.

Question 8

What are the three systemic medications used

to treat Wegener granulomatosis?

Answer 8

● Cyclophosphamide (used in severe cases)
● Methotrexate (used in limited Wegener granulomatosis)
● Glucocorticoids

Question 9

What is the cause of sarcoidosis?

Answer 9

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.

Question 10

What is the most common nasal symptom

associated with sarcoidosis?

Answer 10

Nasal obstruction
Other symptoms include epistaxis, epiphora, nasal pain,
and dyspnea. Nasal symptoms are present in < 5%.

Question 11

Where does sarcoidosis most commonly manifest

in the nose?

Answer 11

Septum and inferior turbinates and commonly involved
with thick crusting. At later stages, yellow subcutaneous
nodules can be seen.

Question 12

What is the typical disease course of sarcoidosis?

Answer 12

● Spontaneous resolution within 2 years of disease onset

● ~ 10% → Progressive disease and pulmonary fibrosis

Question 13

What is the key histopathologic characteristic
seen on nasal biopsy in a patient with
sarcoidosis?

Answer 13

Noncaseating granulomas

Question 14

What laboratory test can be used in the diagnosis

of sarcoidosis?

Answer 14

Serum angiotensin-converting enzyme (SACE)

Question 15

What percentage of patients with active

sarcoidosis will have a positive SACE test?

Answer 15

~ 80%

Question 16

How is sarcoidosis most commonly systemically

managed?

Answer 16

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.

Question 17

What is the treatment for sinonasal sarcoidosis?

Answer 17

Nasal saline irrigations and topical nasal steroids. Some
advocate intralesional steroid injections. In acute exacer-
bations of aggressive disease, systemic corticosteroids
should be administered.

Question 18

What is the clinical triad associated with

Churg-Strauss syndrome?

Answer 18

● Asthma
● Systemic vasculitis
● Eosinophilia

Question 19

What are the three phases of Churg-Strauss

syndrome?

Answer 19

● Prodromal or allergic phase: Adult onset asthma and
allergic rhinitis
● Eosinophilic phase: Peripheral eosinophilia with variable
organ involvement
● Vasculitic phase: Systemic vasculitis

Question 20

What are the criteria for the diagnosis for

Churg-Strauss Syndrome?

Answer 20

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

Question 21

What is the management of sinonasal

Churg-Strauss disease?

Answer 21

Nasal saline irrigations and topical nasal steroids, antibiotics
as needed

Question 22

What are the two most common causes of

midline granuloma syndrome?

Answer 22

● 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).

Question 23

Describe the typical clinical presentation of T-cell

lymphoma as a midline granuloma.

Answer 23

● 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.

Question 24

What criteria are needed to make a diagnosis of

relapsing polychondritis?

Answer 24

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)

Question 25

What are the nasal symptoms commonly seen in 48 to 72% of patients with relapsing polychondritis?

Answer 25

● Acute and painful chondritis ● Sensation of fullness over the nasal bridge ● Mild epistaxis ● Long-standing disease → saddle-nose deformity

Question 26

What are the treatment options available for | relapsing polychondritis?

Answer 26

● 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.

Question 27

What are the clinical manifestations of anhidrotic | ectodermal dysplasia?

Answer 27

Abnormal development of the skin, hair, nails, teeth, and | sweat glands

Question 28

What is the method of inheritance associated with the most common form of anhidrotic ectodermal hypoplasia?

Answer 28

X-Linked recessive

Question 29

What triad is associated with anhidrotic | ectodermal hypoplasia?

Answer 29

Anhydrosis, hypotrichosis, and anodontia Patients often have hypotrichosis, hypodontia, eczema, and atrophic rhinitis.

Question 30

What percentage of patients with primary ciliary | dyskinesia will have situs inversus?

Answer 30

50%. May also have recurrent sinopulmonary infections and | infertility

Question 31

Nasal biopsy followed by electron microscopy looking at the ultrastructure of the cilia is a useful test when looking for what disorder?

Answer 31

Ciliary dyskinesia. Looking at the number of inner and outer dynein arms and evaluating ciliary orientation can assist in differentiating primary from secondary causes.

Question 32

What nasal disorder results from hypertrophy of the sebaceous glands in both the nasal skin and fibrosis?

Answer 32

Rhinophyma

Question 33

Describe two early signs of rhinophyma.

Answer 33

● Dilated (patulous) pores | ● Telangiectatic vessels on the distal nose

Question 34

Rhinophyma may manifest as the final stage of | what other skin disease?

Answer 34

Acne rosacea, although not all patients with rhinophyma | have a history of rosacea

Question 35

What malignant condition can be associated | with rhinophyma?

Answer 35

Basal cell carcinoma

Question 36

What patient populations are affected by | rhinophyma?

Answer 36

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.

Question 37

How is rhinophyma managed?

Answer 37

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.

Question 38

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?

Answer 38

● Immune disorder ● Inflammatory reaction to infection (e.g., Demodex folli- culorum, Bacillus olenorium, Helicobacter pylori) ● Other: UV radiation, vascular hyperreactivity, family history

Question 39

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?

Answer 39

Klebsiella rhinoscleromatis (Frisch bacillus)

Question 40

What are the clinical stages associated with | rhinoscleroma?

Answer 40

● 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

Question 41

Describe the histologic findings associated with | rhinoscleroma.

Answer 41

Mikulicz cells (foamy histiocytes), and Russel bodies (intra- cellular inclusions associated with excessive immunoglobu- lin synthesis)

Question 42

What is the management of choice for treating | rhinoscleroma?

Answer 42

Long-term culture-specific antibiotics and surgical debride- | ment

Question 43

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?

Answer 43

Rhinosporidiosis

Question 44

What is the agent responsible for rhinosporidiosis, | and what is the primary means of transmission?

Answer 44

● Rhinosporidium seeberi | ● Contaminated water

Question 45

What is the classic histopathologic finding(s) | associated with rhinosporidiosis?

Answer 45

Fungal sporangia with chitinous elements in the setting of pseudoepitheliomatous hyperplasia and submucosal cystic structures

Question 46

How is rhinosporidiosis managed?

Answer 46

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.