Rhinology Systemic Disease Flashcards Preview

ENT Rhinology, Allergy, and Immunology > Rhinology Systemic Disease > Flashcards

Flashcards in Rhinology Systemic Disease Deck (46)
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1
Q

What are the three primary granulomatous

diseases that can involve the nasal airway?

A

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

2
Q

What is the classic clinical triad seen in Wegener

granulomatosis?

A

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

3
Q

Describe the otolaryngologic manifestations of

Wegener disease.

A

● 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

4
Q

What are the nasal manifestations of Wegener

granulomatosis?

A

Nasal inflammation, rhinorrhea, nasal crusting and puru-

lence, CRS, septal perforation

5
Q

The antibodies used for diagnosis of Wegener

granulomatosis are directed at what protein?

A

PR3

6
Q

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

A

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

7
Q

What pathologic findings are seen on biopsies

of a patient with Wegener granulomatosis?

A
● Necrotizing, noncaseating multinucleated giant cell
granulomas
● Small and medium vessel vasculitis
● Microabscesses
Note: Biopsies are often nondiagnostic.
8
Q

What are the three systemic medications used

to treat Wegener granulomatosis?

A

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

9
Q

What is the cause of sarcoidosis?

A

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.

10
Q

What is the most common nasal symptom

associated with sarcoidosis?

A

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

11
Q

Where does sarcoidosis most commonly manifest

in the nose?

A

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

12
Q

What is the typical disease course of sarcoidosis?

A

● Spontaneous resolution within 2 years of disease onset

● ~ 10% → Progressive disease and pulmonary fibrosis

13
Q

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

A

Noncaseating granulomas

14
Q

What laboratory test can be used in the diagnosis

of sarcoidosis?

A

Serum angiotensin-converting enzyme (SACE)

15
Q

What percentage of patients with active

sarcoidosis will have a positive SACE test?

A

~ 80%

16
Q

How is sarcoidosis most commonly systemically

managed?

A

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.

17
Q

What is the treatment for sinonasal sarcoidosis?

A

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

18
Q

What is the clinical triad associated with

Churg-Strauss syndrome?

A

● Asthma
● Systemic vasculitis
● Eosinophilia

19
Q

What are the three phases of Churg-Strauss

syndrome?

A

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

20
Q

What are the criteria for the diagnosis for

Churg-Strauss Syndrome?

A

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

21
Q

What is the management of sinonasal

Churg-Strauss disease?

A

Nasal saline irrigations and topical nasal steroids, antibiotics
as needed

22
Q

What are the two most common causes of

midline granuloma syndrome?

A

● 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).

23
Q

Describe the typical clinical presentation of T-cell

lymphoma as a midline granuloma.

A

● 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.

24
Q

What criteria are needed to make a diagnosis of

relapsing polychondritis?

A

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)

25
Q

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

A

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

26
Q

What are the treatment options available for

relapsing polychondritis?

A

● 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.

27
Q

What are the clinical manifestations of anhidrotic

ectodermal dysplasia?

A

Abnormal development of the skin, hair, nails, teeth, and

sweat glands

28
Q

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

A

X-Linked recessive

29
Q

What triad is associated with anhidrotic

ectodermal hypoplasia?

A

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

30
Q

What percentage of patients with primary ciliary

dyskinesia will have situs inversus?

A

50%. May also have recurrent sinopulmonary infections and

infertility

31
Q

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

A

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

32
Q

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

A

Rhinophyma

33
Q

Describe two early signs of rhinophyma.

A

● Dilated (patulous) pores

● Telangiectatic vessels on the distal nose

34
Q

Rhinophyma may manifest as the final stage of

what other skin disease?

A

Acne rosacea, although not all patients with rhinophyma

have a history of rosacea

35
Q

What malignant condition can be associated

with rhinophyma?

A

Basal cell carcinoma

36
Q

What patient populations are affected by

rhinophyma?

A

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.

37
Q

How is rhinophyma managed?

A

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.

38
Q

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?

A

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

39
Q

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?

A

Klebsiella rhinoscleromatis (Frisch bacillus)

40
Q

What are the clinical stages associated with

rhinoscleroma?

A

● 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

41
Q

Describe the histologic findings associated with

rhinoscleroma.

A

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

42
Q

What is the management of choice for treating

rhinoscleroma?

A

Long-term culture-specific antibiotics and surgical debride-

ment

43
Q

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?

A

Rhinosporidiosis

44
Q

What is the agent responsible for rhinosporidiosis,

and what is the primary means of transmission?

A

● Rhinosporidium seeberi

● Contaminated water

45
Q

What is the classic histopathologic finding(s)

associated with rhinosporidiosis?

A

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

46
Q

How is rhinosporidiosis managed?

A

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.