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NON-ALLERGIC RHINITIS
Pregnancy
Rhinitis of pregnancy is relatively common and probably caused by high levels of oestrogen.
It may occur in women without any past history of rhinitis, allergic or otherwise. Symptoms
of AR may also be exacerbated during pregnancy, probably by the same cause. Although
topical nasal steroids with low bioavailability are relatively safe, hypertonic saline douches or
occasional use of topical decongestants may be preferable. e condition is self-limiting and
resolves rapidly following childbirth.
KEY POINTS
• AR is an IgE-mediated disease.
• In western society it is common (>20%).
• It may have signicant negative effects on sleep, work and study.
• History and patient symptoms are crucial to making a diagnosis.
• Patients may have other systemic diseases such as asthma.
• Treatment strategies are mainly allergen avoidance, topical or systemic antihistamines
and topical corticosteroids.
• Immunotherapy and humanised monoclonal antibodies have an increasing role in
disease modication.
Further Reading
1. Bousquet J, Khaltaev N, Cruz AA, et al. Allergic Rhinitis and its Impact on Asthma
(ARIA) 2008 update (in collaboration with the World Health Organization, GA(2)
LEN and AllerGen). Allergy 2008; 63: S8–160.
32. NON-ALLERGIC RHINITIS
Introduction
Rhinitis causes widespread morbidity, treatment costs, reduced productivity and lost school
days. It is dened as a symptomatic inammation of the inner lining of the nose, leading to
nasal obstruction, rhinorrhea (anteriorly or posteriorly), sneezing or nasal/ocular itch. Two
nasal symptoms should be present for at least 1 hour daily for a minimum of 12 weeks per
year to dene chronic rhinitis.
Non-infectious rhinitis can be divided into allergic rhinitis (AR; see Chapter 31) and non-
allergic rhinitis (NAR). Unlike AR there are no specic diagnostic tests for NAR, and the
diagnosis is made on the basis of rhinitis symptoms in the absence of identiable allergy (by
allergy testing), structural abnormality, immune deciency, sinus disease or other cause.
Subgroups of NAR are as follows: drug-induced rhinitis, rhinitis of the elderly, hormonal
rhinitis including pregnancy-induced rhinitis, nonallergic occupational rhinitis, gustatory
rhinitis and idiopathic rhinitis.
1
Types of Non-Allergic Rhinitis
Idiopathic Rhinitis
Idiopathic rhinitis is present in up to 50% of patients with NAR and is characterised by nasal
blockage, rhinorrhoea and sneezing, although sneezing, conjunctival symptoms and pruritis
is lower than that in AR. e aetiology is unknown in most cases and the disease is thought
to be triggered mainly by irritants and changes in atmospheric conditions.
168 Rhinology and Facial Plastic Surgery
NON-ALLERGIC RHINITIS
Rhinitis of the Elderly
Among patients with chronic symptoms, the percentage with a non-allergic aetiology
increases progressively with age and reaches >60% beyond the age of 50 years. Neurogenic
dysregulation is considered the cause of the symptoms. e dominant symptom is bilateral
watery rhinorrhoea without mucosal and/or anatomic abnormality.
Occupational Rhinitis
Occupational rhinitis is dened as rhinitis caused by exposure to airborne agents present in
the work place. ese agents elicit predominantly sneezing, nasal discharge and/or blockage
and may act via both immunologic (IgE-mediated) and non-immunologic mechanisms. e
non-immunologic triggers are oen irritant or toxic small-molecular-weight compounds
such as aldehydes, isocyanates, aircra fuel and jet stream exhaust, solvents and so forth, or
they may be physical (long-term exposure to cold air).
Hormonal Rhinitis
Hormonal rhinitis is typically associated with pregnancy, although puberty is also known
to induce the symptoms of rhinitis. e cumulative incidence of pregnancy rhinitis is 22%,
and in women who smoke there is a relative risk enhancement of 69%. Oestrogens cause
nasal obstruction and/or nasal hypersecretion. Beta-oestradiol and progesterone have been
shown to increase the expression of histamine H1 receptors on human nasal epithelial cells
and mucosal microvascular endothelial cells, and to induce eosinophil migration and/or
degranulation, in marked contrast to testosterone, which decreases eosinophil activation
and viability.
Drug-Induced Rhinitis
Common medications, including non-steroidal anti-inammatory drugs (NSAIDs), beta
blockers, angiotensin-converting enzyme (ACE) inhibitors, methyldopa, oral contraceptives, psychotropic agents and nasal topical decongestants (e.g. xylometazoline) may induce
symptoms of rhinitis. ey may be predictable, as would be the case for known side eects
of particular drugs, or unpredictable, based on individual hypersensitivity to certain drugs.
Intolerance to aspirin and/or NSAIDs predominantly produces rhinorrhoea, which may be
either isolated or part of a complex involving hypertrophic rhinosinusitis, nasal polyps and
asthma. In contrast, intolerance to ACE inhibitors, methyldopa or oral contraceptives, which
is less common than aspirin intolerance, leads predominantly to nasal blockage. Persistent
overuse of topical nasal vasoconstrictors also causes nasal hyper-reactivity and hypertrophy
of the nasal mucosa, a condition known as ‘rhinitis medicamentosa’.
Other Forms
Other forms include gustatory rhinitis, which presents as watery discharge aer ingestion of
hot or spicy food.
Mixed Rhinitis
In reality, a signicant portion of chronic rhinitis patients have more than one known/
unknown aetiologic factor. erefore, the precise diagnostic process is paramount.
Diagnosis as a Stepwise Approach
Diagnosis of NAR and its subgroups is mainly based on a comprehensive clinical history and
rhinologic examination, followed by the stepwise exclusion of possible dierential diagnoses
(Figure 32.1).
Rhinology and Facial Plastic Surgery 169
NON-ALLERGIC RHINITIS
Chronic Rhinitis
Discoloured discharge & crusting
Infectious rhinitis
Mucosa: oedematous, pale and boggy
Klimek L, Cingi C, et al. Non-allergic rhinitis: position paper of the European Academy of Allergy and
Clinical Immunology. Allergy. 2017;72(11):1657–1665).
Mucosa: can appear normal with increased clear watery
secretions but also may be erythematous or atrophic
Positive SPT
Specic IgE in serum
Non-allergic rhinitisAllergic rhinitis
No sensitisation
No signs of infection
Senile rhinitis
Gustatory rhinitis
Occupational rhinitis
Hormonal-induced
rhinitis
Drug-induced rhinitis
Idiopathic rhinitis
If the case history is suggestive of clinically relevant non-infectious rhinitis then
Check possible stimuli, severity and duration of disease
•
Check drug use (systemic and topical), exposure at workplace, hormonal status (preg-
•
nancy, hypothyroidism, acromegaly) and involvement of other organs (asthma, hormonal status)
Exclude other nasal disease (nasal endoscopy)
•
Exclude allergy by using skin prick test (SPT) or serum IgE-antibodies for the most
•
frequent inhalant allergens
Exclude chronic rhinosinusitis (nasendoscopy ± computed tomography [CT] scan of
•
the paranasal sinuses)
Up until now, allergen provocation testing, microbiological analysis, nasal cytology and
nasal hyperreactivity is not recommend due to low clinical value.
Local Allergic Rhinitis (LAR)
Local AR (LAR) or entopy is a localised nasal allergic response with negative SPT and undetectable specic IgE to inhalant allergens. e diagnosis is made by the detection of nasalspecic IgE and/or a positive nasal allergen provocation test. As in AR, patients with LAR
respond well to nasal corticosteroids. Due to unavailability of diagnostic testing for LAR,
patients are still frequently classied as having NAR.
Treatment for Non-Allergic Perennial Rhinitis
In case of drug-induced, food-induced or occupational rhinitis, avoidance is employed as
rst-line therapy. Aspirin-intolerant individuals may benet from aspirin desensitisation
(Figure 32.2).
Several treatments (pharmaceutical and surgical) have been employed for idiopathic NAR.
Intra-nasal anticholinergics (ipratropium bromide) may be useful in patients with nasal
hypersecretion as the predominant symptom, especially those suering with senile rhinitis. Topical nasal glucocorticoids and antihistamines (e.g. azelastine) form the mainstay of
treatment. Two randomised control trials (RCTs) found azelastine nasal spray to be more
170 Rhinology and Facial Plastic Surgery
Nonallergic senile rhinitis
TreatmentNAR Phenotypes
(>65 y)
NON-ALLERGIC RHINITIS
Pathophysiology
Mucosal/glandular atrophy and/or MO
lpratropium bromide
Gustatory rhinitis
Nonallergic occupational
rhinitis
Hormonal-induced rhinitis
Nonallergic drug-induced
rhinitis
Idiopathic rhinitis
Neurogenic inflammation
Neurogenic inflammation
Neuronal imbalance
Neuronal imbalance
Neurogenic inflammation and/or MO
MO = mechanism unknown
Avoidance
Nasal capsaicin
Avoidance
Nasal corticosteroids
Nasal chromones?
Avoidance
Nasal corticosteroids
Nasal capsaicin
Figure 32.2 Overview of non-allergic rhinitis (NAR) management. (From Hellings PW, Klimek L,
Cingi C, et al. Non-allergic rhinitis: position paper of the European Academy of Allergy and Clinical
Imm u nolog y. Al l ergy. 2017;72(11):16 57-16 65. d oi:10 .1111/al l.13200.)
eective than placebo for control of rhinorrhoea, post-nasal drip, sneezing and nasal congestion. e precise mode of action remains to be elucidated. e ecacy of intra-nasal steroids
in patients with vasomotor rhinitis has been inconsistent.
Furthermore, whilst topical nasal steroids are more frequently used for treatment of more
severe symptoms, they are mostly useful in patients in whom an inammatory pathogenesis
is a prominent feature of their disease (occupational rhinitis and drug-induced rhinitis). In
patients with idiopathic rhinitis who do not respond to treatment with nasal steroids, treatment with non-conventional therapies, particularly intra-nasal capsaicin, may be benecial.
In cases where nasal obstruction is resistant to medical treatment and/or the inferior turbinate is hypertrophic, turbinate reduction has been shown to be useful. Endoscopic transnasal vidian neurectomies (excision, diathermy and cryotherapy) produced results with
varying degrees and duration of success.
KEY POINTS
• Approximately half of rhinitis patients may suffer from NAR.
• NAR is diagnosed on the basis of rhinitis symptoms in the absence of identiable
allergy, structural abnormality, immune deciency, sinus disease or other cause.
• A thorough clinical history is the best diagnostic tool available and should focus on
drugs, occupational exposure and hormonal status to subdivide the condition into
subgroups.
• Surgery is an option in patients with persistent nasal obstruction not improved by
medication.
• LAR entails a negative SPT and undetectable specic IgE to inhalant allergens, but
positive nasal-specic IgE and/or nasal allergen provocation test. Patients with respond
well to nasal corticosteroids.
Further Reading
1. Hellings PW, Klimek L, Cingi C, et al. Non-allergic rhinitis: position paper of the
European Academy of Allergy and Clinical Immunology. Allergy. 2017; 72(11):1657–
1665. doi:10.1111/all.13200.
Rhinology and Facial Plastic Surgery 171
RHINOSINUSITIS
33. RHINOSINUSITIS
Rhinosinusitis Denition
International guidelines have provided current denitions of rhinosinusitis. EPOS2020
maintains the criteria laid out in Table 33.1.1 Making a diagnosis therefore relies on the history and clinical examination with radiological conrmation if required. Rhinosinusitis can
then be further dened as acute or chronic based on duration of symptoms: acute <12 weeks
duration; chronic ≥12 weeks. Within acute rhinosinusitis (ARS), further distinctions can be
made on a timeline basis such that a common cold (acute viral rhinosinusitis) will last for
less than 10 days and acute post-viral rhinosinusitis will last between 10 days and 12 weeks
in duration.
For a diagnosis of recurrent acute rhinosinusitis (RARS), there must be symptom-free episodes between the clinical events. RARS is dened as four episodes per year with symptomfree intervals. Some patients may experience acute exacerbations of chronic rhinosinusitis
(AECRS) with worsening of symptoms for short periods, which may be triggered by viral
upper respiratory tract infections (URTIs).
Epidemiology and Socioeconomic Impact
ARS is a very common condition with reported prevalence rates varying from 6–15% with
a prevalence of recurrent ARS estimated at 0.035%. It is estimated that 5.5% of the population consult their primary care physician for an acute respiratory tract infection each year.
is high volume of patients then receives a variable response from medical practitioners in
terms of treatment. Only 0.5–2.0% of all cases of ARS are thought to be bacterial with over
prescribing of antibiotics based on symptoms and limited examination.
Chronic rhinosinusitis (CRS) represents a signicant disease burden worldwide, aecting at
least 11% of the population with a higher prevalence in the United Kingdom than ischaemic
heart disease (3.7%), diabetes (4%), chronic obstructive pulmonary disease (1.5%), heart failure (<1%) and stroke (<1%) and equivalent to that of peripheral vascular disease, arthritis
and back pain, several of which have been shown to have a lesser impact on patients’ quality
of life than CRS. Patients with CRS also have a 5–17% prevalence of asthma as shown by a
recent European study; when broken down into phenotypes the prevalence of asthma is 21%
Table 33.1 EPOS2020 clinical denition of rhinosinusitis
Diagnostic criteria for rhinosinusitis Symptoms should be correlated by either
Primary symptoms (requires at least one
to be present, but if both present is
sufcient to make diagnosis on basis of
symptoms)
Additional symptoms (may also be present
and at least one is needed if only one of the
primary symptoms is present)
Duration >10 days, <3 months = acute
Endoscopy (any of these) Nasal polyps
CT scan ndings (as well as or instead of
endoscopic ndings)
172 Rhinology and Facial Plastic Surgery
1
endoscopic and/or radiological ndings
Nasal blockage/obstruction/congestion
Nasal discharge (anterior/posterior)
Facial pain/pressure
Olfactory dysfunction (Hyposmia/anosmia)
>3 months = chronic
Mucopurulent discharge (middle meatus)
Oedema/mucosal obstruction in middle meatus
Mucosal changes within the ostiomeatal
complex and/or sinuses
RHINOSINUSITIS
in CRS without nasal polyps (CRSsNPs), 47% in CRS with nasal polyps (CRSwNPs) and 73%
in allergic fungal rhinosinusitis (AFRS).
A recent UK study has shown that CRS aects the social spectrum equally and that poor
socioeconomic status does not appear to be a risk factor for CRS, but there remains a need for
larger scale studies that may also examine the natural history of the disease. Two European
studies have reported direct costs of CRS. In CRSwNP, it was estimated that yearly costs were
€1501. In a mixed group of CRS, individual costs for primary and secondary care amounted
to £2974 compared with £555 for controls and £304 versus £51 for out-of-pocket expenses. In
England and Wales, CRS is thought to account for at least 120,000 outpatient visits per year
and leads to over 30,000 polypectomies or sinus operations being performed. With data suggesting 1 in 5 are undergoing revision surgery within 5 years, the specic cost of this revision
surgery is estimated at £15 million per year. Surgical treatment for CRS certainly appears to
inuence drug costs, and there is increasing evidence that earlier surgical intervention may
reduce the prevalence of late-onset asthma.
Acute Rhinosinusitis
e viruses most commonly implicated in ARS include rhinoviruses (50%), inuenza and
parainuenza viruses, adenovirus, respiratory syncytial virus and enterovirus. If bacteria do
become implicated the organisms, those most commonly seen are Streptococcus pneumoniae
(27%), Haemophilus inuenzae (44%) and Moraxella catarrhalis (14%) with other organisms
sometimes seen including S. pyogenes and Staphylococcus aureus. e proportion of these
organisms has changed in recent years with vaccination schemes. Diagnosis of ARS is typically the domain of primary care and therefore endoscopy and computed tomography (CT)
are rarely available in this setting. Key determinants of whether a case of ARS appears more
likely to be bacterial are listed in Table 33.2. Most cases will be self-limiting with complications of ARS exceedingly rare; there is an estimated 2–4 cases per million of the population
per year. is was perhaps thought to be due to the high rate of antibiotic use, but there is
evidence to suggest that in cases where complications have occurred, this has not been prevented by prior use of oral antibiotics.
Chronic Rhinosinusitis
To date, CRS has been largely subcategorised into cases with polyps (CRSwNPs) or without
polyps (CRSsNPs) based on pathophysiology.
CRSwNPs is characterised by an intense oedematous stroma in the sinonasal epithelium,
with albumin deposition, pseudocyst formation and subepithelial/perivascular inammatory cell inltration. It appears to be associated with a typical T-helper 2 cell (TH2) skewed
eosinophilic inammation, with high interleukin (IL)-5 and eosinophil cationic protein (ECP)
concentrations in the polyps.
In comparison, CRSsNPs is characterised by brosis, basement membrane thickening, goblet cell hyperplasia, subepithelial oedema, and mononuclear cell inltration. It exhibits a
T-helper 1 cell (TH1) milieu, with increased levels of interferon gamma (IFN-γ) in inamed
sinus mucosa and low ECP/myeloperoxidase ratios. Also, Asian studies have identied a predominantly TH1 cell and TH17 pattern within polyp tissue.
is basic division into the two subgroups does, however, represent an oversimplication of
a heterogeneous disease. e various aetiological mechanisms that have been proposed for
Table 33.2 Additional symptoms or signs for acute bacterial rhinosinusitis
At least three of the following should be present:
• Discoloured discharge (unilateral predominance)
• Severe local pain (unilateral predominance)
• Fever (>38°C)
• ‘Double sickening’ – deterioration after initial milder phase of illness
Rhinology and Facial Plastic Surgery 173
RHINOSINUSITIS
Fungal antigens
way disease
Osteitic bone
Staphylococcal enterotoxins
Unified air
Biofilm
+++
Eosinophilic mucus
Figure 33.1 Aetiological mechanisms proposed for CRS1.
• Asthma
• Aspirin sensitivity
Inflammatory polyp
CRS are summarised in Figure 33.1. EPOS 20201 has sought to introduce a new classication
for CRS separating out primary and secondary cases and anatomical cases (Figure 33.2).
is classication helps to guide the clinician to the appropriate management. Anatomical
causes, be they primary or secondary, ultimately need a surgical solution, whereas primary
diuse cases lend themselves to appropriate medical treatment prior to consideration of surgical management. In a similar vein, diuse secondary cases require appropriate management of the underlying systemic disorder which may oen be medical. With the advent of
biologics this classication may also help to focus these new therapeutic agents on the correct
patient cohort.
Anatomical variation may predispose a patient to isolated inammation in only one of the
sinuses as a post-obstructive phenomenon. CT scanning may identify anatomic variants
such as an infra-orbital ethmoid cell, concha bullosa and narrow nasal cavity secondary to
deviated nasal septum. Microbiology sampling of mucopus should be performed in conjunction with surgical drainage and aeration as medical therapy typically fails but these
patients tend not to need any sustained post-operative medical treatment. In approximately
10% of these cases the cause will be odontogenic, and in unilateral cases an oral examination and a careful review of the maxillary sinus oor should be undertaken to look for
signs of an oro-antral stula. Involvement of oral maxillofacial surgery colleagues will be
benecial at an early stage where odontogenic aetiology is suspected to enable optimisation
of management. In CRSwNP, ostiomeatal complex (OMC) obstruction may be a ‘barometer’
of overall disease burden, in that increasing Lund-Mackay scoring is associated with OMC
involvement overall.
e new classication also helps to remind the clinician that ‘nasal polyposis’ is not a diagnosis; it is merely a description of a pathological endpoint of sinonasal disease, and localised
examples of nasal polyps include antrochoanal polyps and reactive polyps as may be seen
around other inammatory foci such as a fungal ball or inverted papilloma. A unilateral
nasal polyp should always be viewed with suspicion, especially in older patients and in anyone exposed to wood dust. CT scanning and biopsy of a unilateral polyp are needed to decide
on the medical and surgical treatment strategy to pursue. Caution should be advised with
174 Rhinology and Facial Plastic Surgery
rhinosinusitis
Anatomic distribution Endotype dominance Clinicals/Phenotypes
Primary CRS
Secondary CRS
Th2
Localized?
Focal? Local?
Non-Th2
Th2
Diuse
Non-Th2
Anatomic distribution Endotype dominance Clinicals/Phenotypes
Anatomically
discrete
Diuse Inammatory
Local Pathology
Mechanical
Immunity
AFS
OMC
Isolated frontal
Isolated sphenoid
CCAD
eCRS/CRSwNP
AFS
Non-eCRS
Corticosteroid –ve
Older >60 yrs
Odontogenic
FB/Fungal Ball
Tumour
PCD
CF
GPA
Eosinophilic
angitis
Selective IgA
deciency
CVID
Figure 33.2 Primary and secondary CRS – EPOS2020 classication1. CRS = chronic rhinosinusitis,
AFS = allergic fungal sinusitis, OMC = ostiomeatal complex, CCAD = central compartment atopic
disease, eCRS = eosinophilic chronic rhinosinusitis, CRSwNP = chronic rhinosinusitis without nasal
polyps, FB = fungal ball, PCD = primary ciliary dyskinesia, CF = cystic brosis, GPA = granulomatosis
with polyangiitis, IgA = immunoglobulin A, CVID = common variable immune deciency
performing a biopsy of any polyp that arises superiorly within the nose, or from the back of
the nose in a juvenile male patient. Magnetic resonance imaging (MRI) scanning may be
appropriate in select cases where extra-sinus involvement is identied or suspected.
Given the insidious nature of CRS, some cases may appear as CRSsNP at rst presentation
but eventually develop nasal polyps. A number of recognised mechanisms appear to have a
role in polyp formation, including bacterial (super-antigen response), fungal sensitisation
and atopy. ese may be associated with biolm formation or frank eosinophilia, both exacerbating and propagating the inammatory process.
A proportion of patients with CRSwNP also fall into a unique subset, characterised by
coexistent asthma and aspirin sensitivity known as Samter’s triad or aspirin/non-steroidal
anti-inammatory exacerbated respiratory disease (NERD). NERD is believed to account
for approximately 15% of CRSwNPs, classically manifest by severe nasal and/or respiratory
symptoms following ingestion of salicylates.
When severe bilateral nasal polyposis is the possibility, NERD should be considered as well
as AFRS, another smaller subgroup, believed to represent 10% of those with CRSwNP. CT
scanning may elucidate a pansinus opacication typical of AFRS/NERD (double density
signs), with thick granular (eosinophilic) mucin usually found at initial presentation, during
Rhinology and Facial Plastic Surgery 175
RHINOSINUSITIS
Table 33.3 Systemic conditions causing rhinosinusitis or possible differential diagnosis
Category Diagnosis Key features
Congenital Cystic brosis Abnormal sweat test
CFTR gene mutation on
chromosome 7
Primary ciliary dyskinesia Abnormal mucociliary clearance and
cilial ultrastructure
Primary immunodeciencies (CVID, SCID,
hypo/dysgammaglobulinaemias
Infectious/
inammatory
Neoplastic Haematological malignancies Abnormal FBC/bone marrow
Iatrogenic Atrophic rhinitis Excessive crusting following radical
Metabolic Malnutrition Low BMI
Note: CFTR = cystic brosis transmembrane conductance regulator, CVID = common variable
immune deciency, SCID = severe combined Immunodeciency, HIV = human immunodeciency
virus, ELISA = enzyme-linked immunoassay, ACE = angiotensin converting enzyme, CXR=chest X-ray,
cANCA = cytoplasmic antineutrophil cytoplasmic antibody, pANCA = perinuclear antineutrophil
cytoplasmic antibodies, FBC = full blood cell count, BMI = body mass index
HIV Positive ELISA for HIV
Sarcoidosis Non-caseating granulomata
Tuberculosis Acid-fast bacilli
Granulomatosis with polyangiitis
(Wegener’s syndrome)
Eosinophilic granulomatosis with
polyangiitis (Churg-Strauss Syndrome)
Sinonasal malignancies Biopsy positive
Chemotherapy/immunosuppression Relevant dug history
Low/absent antibodies
Elevated ACE
CXR signs (hilar lymphadenopathy)
Mantoux test positive
cANCA positive
Leukocytoclastic vasculitis on biopsy
pANCA positive
Radiological changes
nasal surgery
surgery or sometimes in the post-operative period. e role and signicance of osteitis in
CRS is currently unclear, although it may explain why some cases are resistant to standard
oral treatment regimens. In CRSwNPs, osteitis may act as a marker of severity and has been
shown to occur signicantly more oen in revision cases.
Systemic Conditions Causing Rhinosinusitis
Ciliary Dyskinesia
Although newborn screening for cystic brosis (CF) will pick up most cases, any doubt in a
child presenting with diuse nasal polyposis in conjunction with total opacication of the
sinuses as well as hypoplastic sinuses (typically frontal and sphenoid) should be followed
up with a sweat test. Nasal endoscopy demonstrates polyp formation in up to 45% of adults
with CF, although most will show radiological evidence of disease. Polyps from children
with CF have a signicantly lower eosinophil count than patients with ‘typical’ adult-onset
CRSwNPs. However, there have also been studies to demonstrate signicant overlap in
eosinophil concentrations between groups.
Primary ciliary dyskinesia is oen associated with situs inversus and known in this circumstance as Kartagener’s syndrome. Patients oen present in adolescence and may have a prior
history of otitis media with eusion; they may also suer with bronchiectasis. Ciliary dyskinesias will show delayed mucociliary clearance times and require investigation at specialist centres, of which there are three in the United Kingdom. Absent mucociliary clearance
176 Rhinology and Facial Plastic Surgery
NASAL POLYPOSIS
exhibited in primary ciliary dyskinesia results in recurrent bacterial infections and CRSwNP
in approximately 40% of patients.
Other systemic conditions such eosinophilic granulomatosis with polyangiitis, granulomatosis with polyangiitis and sarcoidosis are described in detail in Chapter 44.
Other Differential Diagnoses to Consider
Clear rhinorrhoea, especially unilaterally, should always prompt investigation for a cerebrospinal uid (CSF) leak. Tumours will typically present with unilateral symptoms such
as blockage or bleeding, but inverted papillomas may sometimes be found amongst bilateral
CRSwNPs; hence, this is why it is important to send material for histology during any surgery to remove polyps, especially the rst time, if there is any index of suspicion.
Rhinosinusitis may also be a presenting or secondary feature of patients with immunodeciencies (e.g. HIV) or who are immunocompromised by systemic treatment for other disorders (e.g.
receiving immunosuppressive drugs for organ transplantation). In those cases, patients require
endoscopic retrieval of mucopus where possible, as unusual organisms may be found.
Immunodeciency should always be considered a possibility in patients who appear not
to respond to standard medical and surgical care for CRS and in cases of recurrent ARS.
Common variable immune deciency (CVID), selective IgA deciency, IgG subclass deciency, and specic antibody deciency are all possible immunodeciencies that can be
detected in cases of CRS. Certainly there is a role for screening for immunodeciency in CRS
patients refractory to standard treatment.
In all secondary cases, involvement of relevant physicians is likely to be needed for their
global management.
KEY POINTS
Rhinosinusitis is a common disease with CRS affecting an estimated 10% of the
population worldwide
• Most ARS is not bacterial and requires symptomatic relief only.
• CRS is a heterogeneous disease with differing phenotypes and endotypes; the latter
have yet to be clearly determined.
• CRS presents a signicant burden on health care resources, but guidelines exist for its
medical and surgical management.
• Where other clues are present, consideration must be given to other systemic causes
for sinonasal disease.
Further Reading
1. Fokkens WJ, Lund VJ, Hopkins C, et al. European Position Paper on Rhinosinusitis
and Nasal Polyps 2020. Rhinology. 2020;58(Suppl S29):1–464. Published 2020 Feb 20.
doi:10.4193/Rhin20.600.
34. NASAL POLYPOSIS
Nasal polyps (NPs) are oedematous protrusions of sinonasal mucosa on a background of
chronic inammation. ey are a pathological endpoint of sinonasal disease, phenotypically
described as chronic rhinosinusitis with nasal polyposis (CRSwNP). Based on the up-to-date
EPOS2020 classication described in the Chapter 33, CRSwNP falls mainly into the category
Rhinology and Facial Plastic Surgery 177
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