Sinonasal Manifestations of Systemic Diseases

• Other H&N Findings: laryngeal ulcerations, subglottic stenosis, CHL from serous otitis media, SNHL, lacrimal obstruction, uveitis, keratitis, gingivitis, gingival hyperplasia

• Systemic Findings: pulmonary (cough, hemoptysis, pulmonary infiltrates), cardiac (endocarditis, myocarditis, myocardial infarction), neurological (mononeuritis multiplex, cranial nerve palsy), focal glomerulonephritis, constitutional symptoms, cutaneous eruptions (palpable purpura, ulcers)

• Diagnosis: serologic findings in conjunction with tissue biopsy from site of active disease provides definitive diagnosis

1. C-ANCA: directed against PR3 and is most specific (87%)

2. Biopsy: lung (highest yield), nasal mucosa or renal

3. Other Tests: CBC, ESR, SPEP, BUN, creatinine, UA, autoimmune panel, smooth muscle and neutrophil cytoplasm antibodies; chest and sinus imaging

• Histology: vasculitis of small and medium vessels with intramural, eccentric, necrotizing granulomas (multinucleated giant cells); coalescence of microabscesses

• Treatment:

1. Medical: induction therapy with systemic corticosteroids, followed by methotrexate (mild disease), cyclophosphamide (severe disease), or rituximab; saline irrigation and lubricants for nasal crusting

2. Surgical: ESS not recommended in active disease unless to treat complications of rhinosinusitis; reconstructive surgery for nasal deformities

Sarcoidosis

• Pathophysiology: multisystem, immune-mediated disease of unknown etiology that manifests as noncaseating granulomas with accumulation of T cells and IL-2 in involved organs, especially lymphatic system, lungs, liver, spleen and bones; may lead to fibrosis of tissue

• Epidemiology: incidence peaks in individuals aged 20 to 40 years, more common among African American women and Scandinavian individuals

• Sinonasal Sx: nasal congestion, nasal obstruction, rhinorrhea, epistaxis, facial/nasal pain, anosmia

• Sinonasal Findings: infrequent (occur in 1% to 5% of cases, but is a poor prognostic indicator); purple, friable nasal mucosa (on septum and inferior turbinate), mucosal hypertrophy, nasal polyps, yellow submucosal nodules (intramucosal granulomas), septal perforation, oronasal fistulae

• Other H&N Findings: cervical lymphadenopathy (25% to 50%, most common H&N presentation), uveoparotid fever (Heerfordt’s disease—uveitis, fever and parotid enlargement), supraglottic/epiglottic submucosal mass, orbital mass, bilateral granulomatous uveitis

• Systemic Findings: incidental hilar lymphadenopathy, cutaneous lesions (erythema nodosum, lupus pernio, rashes)

• Diagnosis: biopsy often required but can be supported by elevated ACE and serum/urinary calcium levels, CXR findings, and negative stains for fungus and acid-fast bacilli

• Histopathology: no specific features; multiple noncaseating granulomas consisting of tightly packed epitheloid cells surrounded by lymphocytes, fibroblasts, and multinucleated giant cells

• Treatment:

1. Medical: most patients experience spontaneous remission within 2 years without treatment; asymptomatic patients do not require treatment; treat mild symptomatic disease with NSAIDs, severe disease with systemic corticosteroids and immunomodulators (ie, methotrexate, antimalarials, TNF-alpha inhibitors)

2. Surgical: ESS can improve quality of life if granulomatous or polypoid lesions present

Churg-Strauss Syndrome

(Eosinophilic Granulomatosis with Polyangiitis)

• Pathophysiology: allergic, granulomatous, small-vessel vasculitis of unknown etiology; characterized by triad of bronchial asthma, eosinophilia, and systemic vasculitis

• Stages

1. Prodromal/Allergic Stage: allergic rhinitis, asthma, nasal polyposis, rhinosinusitis

2. Eosinophilic Infiltrative Stage: eosinophilic pneumonia or gastroenteritis

3. Vasculitic/Systemic Stage: systemic vasculitis with granulomatous inflammation

• Epidemiology: mean age of diagnosis is 50 years

• Risk Factors: genetic (HLA-DRB4 positivity)

• Sinonasal Sx: allergic rhinitis (48%), progressive worsening of nasal obstruction and rhinorrhea

• Sinonasal Findings: nasal polyposis, crusting

• Systemic Findings: lung lesions, myocardial infarction, constitutional symptoms

• Diagnosis: at least four of six criteria; asthma, eosinophilia >10%, neuropathy, nonfixed pulmonary infiltrates, paranasal sinus abnormalities, extravascular eosinophils on biopsy

• Histopathology: necrotizing vasculitis with extravascular necrotizing granulomas; intense eosinophilia of vessels and perivascular tissue

• Treatment:

1. Medical: corticosteroids, cytotoxic agents (cyclophosphamide) for life-threatening cases; medical therapy for sinonasal inflammation

2. Surgical: sinus surgery as needed

AUTOIMMUNE/INFLAMMATORY DISEASES

Sjögren’s Syndrome

• Pathophysiology: systemic autoimmune lymphocytic infiltration of exocrine glands causing hypofunction and destruction; postulated to involve initial, inciting event (ie, viral infection) resulting in aberrant immune response in genetically susceptible individuals

• Epidemiology: more common in middle-aged women (fourth to fifth decade)

• Sinonasal Sx: nasal obstruction and dryness (most common), epistaxis, hyposmia, and recurrent rhinosinusitis

• Sinonasal Findings: dry mucosa, crusting, septal ulceration

• Other H&N Findings: keratoconjunctiva sicca (filamentary keratitis), xerostomia (dental caries, dry mucosa), intermittent bilateral parotid swelling, intraoral fungal overgrowth

• Systemic Findings: renal tubular acidosis, Raynaud’s phenomenon, polyneuropathy

• Diagnosis: at least two of the three objective features

1. Positive serum anti-SSA/Ro and/or anti-SSB/La or (positive rheumatoid factor and antinuclear antibody titer ≥1:320)

2. Minor salivary gland biopsy exhibiting focal lymphocytic sialadenitis with a focus score ≥1/4 mm²

3. Keratoconjunctivitis sicca with ocular staining score ≥3 (assuming no treatment for glaucoma and history of eye surgery in past 5 years)

• Treatment: symptom-driven; artificial lubricants and moisturizers, pilocarpine; medical therapy for sinonasal inflammation; corticosteroids and cyclophosphamide for major organ involvement

• Prognosis: associated with non-Hodgkin’s lymphoma

Relapsing Polychondritis

• Pathophysiology: autoimmune disease of unknown etiology; characterized by recurrent inflammation of cartilage (ie, hyaline) and tissues containing glycosaminoglycans, with resultant replacement by granulation and fibrosis

• Epidemiology: often presents in fourth to fifth decade

• Sinonasal Sx: rhinorrhea, epistaxis, rhinosinusitis

• Sinonasal Findings: crusting, nasal chondritis, cartilaginous destruction (septal perforation, saddle nose deformity)

• Other H&N Findings: auricular chondritis (lobule-sparing), ocular inflammation, collapse of laryngotracheal cartilage framework, and airway compromise

• Systemic Findings: aortic/mitral valve disease, nonerosive polychondritis, skin lesions (aphthous ulcers, purpura, nodules)

• Diagnosis: at least three of McAdam’s criteria with histological evidence of chondritis in two or more locations

1. Bilateral auricular chondritis

2. Nonerosive seronegative inflammatory polyarthritis

3. Nasal chondritis

4. Ocular inflammation

5. Respiratory tract chondritis

6. Audiovestibular damage

• Histopathology: cartilage loses its basophilia, loss of chondrocytes, mixed inflammatory infiltrate in perichondrum; cartilage eventually replaced by fibrous connective tissue

• Treatment: treat acute phase and flare-ups with systemic corticosteroids; immunosuppressives (ie, cyclophosphamide, azathioprine, methotrexate) can control symptoms and possibly delay disease progression

NEOPLASTIC

Nasal T Cell Lymphoma

(Angiocentric T Cell Lymphoma, Polymorphic Reticulosis, Lymphomatoid Granulomatosis, Lethal Midline Granuloma)

• Pathophysiology: form of extranodal non-Hodgkin’s lymphoma; originates from natural killer or T cells; almost always associated with EBV (>95%)

• Epidemiology: rare; more common among Asians, commonly presents in sixth decade

• Risk Factors: EBV infection, autoimmune disease, immunodeficiency, chemical or drug exposure (ex: phenytoin), prior radiation or chemotherapy

• Sinonasal Sx: nasal obstruction (most common initial symptom) followed by purulent rhinorrhea, epistaxis, nasal swelling

• Sinonasal Findings: pale and friable mucosa, crusting, unilateral mucosal ulceration with extension to the palate, maxillary sinus, and/or upper lip, oronasal fistulae, septal perforation, friable necrotic mass

• Other H&N Findings: orbital involvement

• Systemic Findings: constitutional symptoms (fever, weight loss, malaise), cutaneous lesions (maculopapular rash, ulcerative cutaneous lesions); may also involve pulmonary, renal, GI or CNS regions

• Diagnosis: CT, MRI, biopsy

• Histopathology: atypical cells with irregular nuclei, granular chromatin, small nucleoli, and pale-to-clear cytoplasm; inflammatory cell infiltrate may be present

• Treatment: radiation for early-staged (Stage I/II) disease is an option but may result in high rates of recurrence; chemoradiation yields 5-year survival of 20% to 80%

INFECTIOUS DISEASES

Rhinoscleroma

• Pathophysiology:

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