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Section 6 Systemic Disorders/Surrounding Structures Involving PNS306
Others
■ Recurrent acute sinusitis, chronic rhinosinusitis, polyposis, and even
nasal perforations can be seen but less commonly in other vasculitis,
such as Churg-Strauss syndrome, systemic lupus erythematosus, and
polyarteritis nodosa. Nasal mucosal atrophy may be seen in Sjögren’s
syndrome.
IMMUNODEFICIENCY
Primary Immunodeciency
■ Inherited defects aecting cell-mediated/humoral immunity, phagocyte
function, or mucociliary clearance can result in recurrent and persistent
infections in the head and neck.
■ ese include rhinosinusitis, otitis media, oral cavity infections, and
adenopathy.
■ If a patient develops two or more episodes of severe sinusitis within a
year, investigations for immunodeciencies should be initiated.
■ erapy besides long-term antibiotics includes immunoglobulins and
bone marrow transplantation, depending on the disorder.
Structural Defects
Dysfunctions aecting mucociliary clearance mechanism result in recurrent
sinusitis and polyposis, in addition to frequent pulmonary infections.
Cystic Fibrosis
■ Autosomal recessive inheritance.
■ Sinonasal involvement seen in about 20% of patients.
■ e various features of involvement include nasal polyposis and chronic
rhinosinusitis. e mucopurulent secretions can lead to bulging of the
lateral wall of the maxillary sinus simulating a mucocele, referred to as
maxillary pseudomucoceles.
Primary Ciliary Dyskinesia
■ Autosomal recessive disorder.
■ About 50% of patients have Kartagener’s syndrome comprising of
bronchiectasis, situs inversus, and chronic sinusitis.
■ Rhinitis, polyposis, maxillary, and ethmoid sinusitis are seen, while
frontal sinuses are often hypoplastic (Figs. 22.5A to C).
Acquired Immune Disorders
Acquired Immunodeciency Syndrome
■ Nasopharyngeal mucosal inammation and hypertrophy is seen.
■ Sinusitis: It is usually recurrent, may be acute and chronic.
■ Rhinitis: It may exacerbate allergic rhinitis.
307Chapter 22 Systemic Disorders Aecting Sinonasal Cavity
A
C
B
Figs. 22.5A to C: Primary ciliary dyskinesia.
(A and B) Coronal computed tomography
(CT)-paranasal sinus (PNS) showing maxillary
and ethmoid sinusitis (*); and (C) Lung
window CT showing extensive bronchiectasis,
bronchial wall thickening with centrilobular
nodules, and ground-glass opacities (*).
Bone marrow Transplant Recipients
■ Increased predisposition to infections with Gram-negative bacteria and
fungi.
■ Sinusitis especially invasive fungal sinusitis is a real risk.
Solid Organ Transplant Recipient
■ Similar increased risk of invasive fungal infections, especially
aspergillosis.
DISORDERS WITH SINOPULMONARY INVOLVEMENT
Several disorders of varying etiology tend to involve the respiratory system,
especially the airways and sinonasal apparatus (Flowchart 22.2).
Some of these have been discussed earlier in this chapter and elsewhere.
Allergic Bronchopulmonary Aspergillosis
■ Allergic bronchopulmonary aspergillosis is a hypersensitivity response
to Aspergillus fumigatus with bronchial and pulmonary manifestations,
seen in asthmatics.
■ It may be associated with allergic Aspergillus sinusitis with exacerbations
parallel to the pulmonary symptoms.
■ Imaging reveals chronic rhinosinusitis which is more often unilateral
with central hyperdensities and sinus expansion (Figs. 22.6A and B).
■ Seen in young asthmatics, patients give history of passage of brownish
plugs/casts with nasal discharge.
Section 6 Systemic Disorders/Surrounding Structures Involving PNS308
Flowchart 22.2: Classication of disorders with sinopulmonary involvement.
(GPA: Granulomatosis with polyangiitis; EGPA: Eosinophilic Granulomatosis with Polyangiitis
also in the table also replace CSS with EGPA).
BA
Figs. 22.6A and B: Allergic aspergillus sinusitis. (A) Coronal computed tomography (CT)
image showing hyperdense contents distending the maxillary and ethmoid sinuses (*). Note that
the edematous mucosa is hypodense; and (B) Lung window of same patient showing patchy
area of consolidation in right lower lobe with central bronchiectasis (arrows).
Asthma
■ It is logical that diseases of the upper and lower airway may coexist in
the same patient. Hence, a large proportion (up to 80%) of patients with
asthma has allergic rhinitis, while nasal polyposis can occur in 7%.
■ Imaging reveals nonspecic ndings of sinus opacication, air-uid
levels, mucosal thickening, or polyposis.
Samter’s Triad
Aspirin-exacerbated respiratory disease (AERD), Widal’s triad/aspirin
sensitive asthma syndrome comprising of asthma and nasal polyposis related
to aspirin/other nonsteroidal anti-inammatory drugs (NSAIDs) intake.
Inammatory Bowel Disease
Chronic mucosal inammation may be present in up to 50% patients
of ulcerative colitis, and rarely in Crohn’s disease. Occasionally septal
perforation may also be seen.
Amyloidosis
■ Nasal or maxillary sinus mucosal thickening maybe seen in amyloidosis.
■ It is mostly unilateral.
GRANULOMATOUS DISORDERS
■ Granulomatous diseases are often multisystem disorders that result in
formation of granulomas (macrophages or mononuclear inammatory
cells with or without giant cells surrounded by lymphocytes).
■ In the head and neck, these essentially include the multisystem diseases
that we have discussed earlier in the chapter.
■ ese disorders are of varying etiology, mainly autoimmune (GPA,
Churg-Strauss syndrome, and Behcet disease), infectious (TB, fungal,
leprosy, and rhinoscleroma), idiopathic (sarcoidosis), or hereditary
(chronic granulomatous disease).
■ As discussed earlier, in the head and neck region, malignancy is an
important dierential of these disorders (Figs. 22.7A and B).
309Chapter 22 Systemic Disorders Aecting Sinonasal Cavity
BA
Figs. 22.7A and B: NK/T-cell lymphoma. (A) Axial CT soft tissue window; and (B) Coronal bone
window CT showing destruction of the nasal septum and turbinates with heterogeneous soft
tissue thickening. (CT: Computed tomography; NK: Natural killer).
Immunoglobulin-4-related Disease
■ Relatively recently described entity, rst reported in 2001.
■ Multisystem disease, sinonasal involvement rare. Sinonasal involvement
is part of multisystem disease isolated (more frequent).
■ Nonspecic symptoms, epistaxis is common presenting symptom.
■ Imaging: No specic feature.
• Site—Maxillary sinus is most common, more often unilateral, and can
be bilateral
• Focal soft tissue mass with bony destruction. Perineural/marrow
inltration also seen
• Computed tomography—Isoattenuating mass with bone expansion
and remodeling. Bone sclerosis/erosion.
■ Magnetic resonance imaging—Isointense to gray matter with homogenous
enhancement (Figs. 22.8A and B).
Section 6 Systemic Disorders/Surrounding Structures Involving PNS310
BA
Figs. 22.8A and B: IgG4-related disease. (A) Coronal fat suppressed T2-weighted (T2W)
images showing heterogeneously hypointense soft tissue lling bilateral maxillary, frontal, and
ethmoid sinuses; and (B) Extension to extraconal compartment of both orbits and nasal cavities
(arrow).
■ Diagnostic criteria based on international consensus statement require
presence of two out of three characteristic histopathological features
which include a dense lymphoplasmacytic inltrate (with more than 50
IgG4 plasma inltration), brosis (characteristic storiform pattern), and
obliterative phlebitis. Eosinophils are also seen within the inltrate.
■ Supportive evidence is elevation of serum IgG4 levels. It is, however, an
insensitive marker and can be negative. Tissue biopsy is essential.
■ Treated with steroids/surgery. In nonresponders rituximab has also
been advocated.
Rhinoscleroma
■ Chronic granulomatous disease of infectious etiology.
■ Organism: Klebsiella rhinoscleromatis (Gram-negative bacteria).
■ Clinical features: Nasal crusting, expansion of nasal cavity, and foetid
odor.
■ Endemic areas: Tropical and temperate regions including parts of India.
■ Sites: Nasal cavity is involved in almost all patients with subsequent
spread to the rest of upper airway, pharynx, nasopharynx, oropharynx,
larynx, and less commonly trachea and even bronchi involved. Middle
and inferior turbinate are involved rst, then nasal septum. May extend
through septum to maxillary sinus (40% cases). Sphenoid and ethmoid
sinus involvement is uncommon.
■ Histopathology of scleroma: Granulation tissue with plasma cells, Russell
bodies (degenerated plasma cells), and Mikulicz cells (foamy histiocytes
containing bacteria).
■ Treatment: Antibiotics (broad spectrum), surgery, and laser for laryngeal
lesions.
Box 22.1: Suspicion of granulomatous disorders on imaging.
When to suspect granulomatous disorders on imaging:
• Chronic sinonasal inammation (mucosal thickening and/or polyposis)
• Osseous and cartilage erosion, perforation of nasal septum ± palate perforation
• Nasal cavity is involved before the paranasal sinuses
• Maxillary/ethmoid sinus involvement
• Frontal sinuses usually spared
• Orbital extension
• Less commonly intracranial extension.
■ Imaging ndings:
• Depend on the stage of the disease, there is expansion of the nasal
cavity, scalloping of sinus wall (chronic disease unlike malignancy)
• Catarrhal stage: Nonspecic mucosal thickening or soft tissue nodules
• Granulomatous (hypertrophic) stage: Masses, bilateral asymmetrical
in majority, and unilateral in one-third (Box 22.1).
• Computed tomography: Scleromas are well-dened and homogenous
in appearance.
• Magnetic resonance imaging:
▶ T1-weighted image: It may be hyperintense (increased protein in
Mikulicz cells and Russell bodies)
▶ T2-weighted image: Mildly hyperintense due to cellular com po-
nent, with hypointense foci due to brotic component.
▶ Contrast-enhanced magnetic resonance imaging: Homogenous
enhancement.
▶ Diusion-weighted imaging: Restricted diusion (high cellularity
and fatty areas).
■ Sclerotic (brotic stage):
• Atrophy/destruction of turbinates
• Nasal septum destruction
• Vestibular, laryngeal stenosis.
Rhinosporidiosis
■ Chronic granulomatous disease of infectious etiology.
■ Organism: Rhinosporidium seeberi, fungus of class Mesomycetozoea.
■ Endemic areas: Southern India, Sri Lanka, and Pakistan.
■ Adult males commonly aected.
■ Predisposing factor: Bathing in stagnant water ponds. Enters through
skin and mucosal abrasions.
■ Sites of involvement: Nasal cavity, nasopharynx, oropharynx, and
nasolacrimal ducts (in decreasing order)
• Other systems reported: Trachea, bones, central nervous system, and
orbits.
311Chapter 22 Systemic Disorders Aecting Sinonasal Cavity
Section 6 Systemic Disorders/Surrounding Structures Involving PNS312
Fig. 22.9: Rhinosporidiosis. Clinical image showing vascular polyp of the nasal cavity (*).
■ Imaging for atypical/recurrent cases and for extent of disease.
■ On clinical examination: Vascular polyp with white dots classical in
endemic areas (Fig. 22.9).
■ Computed tomography: Nasal cavity with common sites including
inferior turbinate, inferior meatus and oor
• Well-dened polypoidal mass with moderate to intense enhancement
• Foci or air and calcication within
• Multiple dilated supplying vessels
• Compared to nasal cavity and nasopharyngeal lesions, those in
oropharynx or trachea show less enhancement
• Nasolacrimal duct may be involved with spread to lacrimal sac
• Adjoining bones involved with thinning/rarefaction or erosion.
Marked erosion of inferior turbinate, septal erosion, and thinning of
medial wall of the maxillary sinus
• Magnetic resonance imaging: Heterogeneous appearance with ow
voids. Intense enhancement on contrast-enhanced MRI (CEMRI).
Imaging pattern may resemble the cribriform pattern of enhancement
of inverted papilloma. However, site and spread is dierent.
■ Diagnosis: Fine-needle aspiration cytology (FNAC) with examination
under potassium hydroxide (KOH)/Papanicolaou smear. However,
histopathology often required.
■ Treatment: Surgical removal with high recurrence rates. Dapsone is also
used.
ILLUSTRATIVE CASE
Demographics
A 40-year-old housewife.
Clinical Picture
■ Chronic cough
■ Nasal discharge
■ Skin ulcers
■ Microscopic hematuria
■ Received oral prednisolone.
Baseline Imaging (Figs. 22.10A to C)
Axial CT paranasal sinus (Fig. 22.10A) and coronal reconstruction
(Fig. 22.10B):
■ Opacication of the left maxillary and ethmoid sinuses
■ Rarefaction of the left maxillary wall
■ Nasal septum intact
■ Polypoidal soft tissue the right maxillary sinus.
Coronal reconstruction CT thorax (Fig. 22.10C):
■ Cavitating lesions in right middle lobe and a nodule in left lower lobe.
313Chapter 22 Systemic Disorders Aecting Sinonasal Cavity
6 Months Follow-up Imaging (Figs. 22.10D to F)
Axial CT paranasal sinus (Fig. 22.10D) and coronal reconstruction
(Fig. 22.10E):
■ Characteristic lateral progression of the destructive process to involve
the septum and the maxillary walls.
■ Note the nal appearance is indistinguishable from primary atrophic
rhinitis.
■ Coronal CT lung window (Fig. 22.10F) showing increase in the size as
well as the cavitation of both right and left side lesions.
Diagnosis
Granulomatosis with polyangiitis.
Section 6 Systemic Disorders/Surrounding Structures Involving PNS314
A
B
D
E
C F
Figs. 22.10A to F
BIBLIOGRAPHY
1. Abdel Razek AA. Imaging of scleroma in the head and neck. Br J Radiol.
2012;85(1020):1551-5.
2. Alobid I, Guilemany JM, Mullol J. Nasal manifestations of systemic illnesses. Curr
Allergy Asthma Rep. 2004;4(3):208-16.
3. Deshpande V. IgG4 related disease of the head and neck. Head Neck Pathol.
2015;9(1):24-31.
4. Nwawka OK, Nadgir R, Fujita A, et al. Granulomatous disease in the head and
neck: developing a dierential diagnosis. Radiographics. 2014;34(5):1240-56.
5. Pakalniskis MG, Berg AD, Policeni BA, et al. e Many Faces of Granulomatosis
with Polyangiitis: A Review of the Head and Neck Imaging Manifestations. AJR
Am J Roentgenol. 2015;205(6):W619-29.
315Chapter 22 Systemic Disorders Aecting Sinonasal Cavity
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