HIV Manifestations in ENT - K.J. Lee's Essential Otolaryngology

1. Infectious/inflammatory conditions from immunodeficiency
2. Complicated infections from immunodeficiency
3. Malignancy related to immunodeficiency
4. AIDS-related illness

I. Infectious/Inflammatory Conditions Associated With HIV

Salivary Gland

• Parotid gland is most commonly affected (due to intraparotid lymph nodes)
• Salivary gland lesions are especially prevalent in children - up to 18% of primary complaints yielding an HIV diagnosis
• ~75% of parotid lesions are Benign Lymphoepithelial Cysts (BLECs); ~6% are neoplasms

Benign Lymphoepithelial Cysts (BLECs)

• Most common parotid lesion in HIV+ patients
• Etiology: viral inflammation
• Often bilateral (up to 80% bilateral on imaging), though may present unilaterally
• Typically asymptomatic; compression of parotid ducts can cause pain/sialadenitis
• Cervical lymphadenopathy in up to 90% of cases
• Can mimic Warthin tumor (both cystic and bilateral)

Diffuse Infiltrative Lymphocytosis Syndrome (DILS)

• Clinically similar to Sjogren syndrome in HIV+ patients
• Incidence ~1% of HIV patients
• Presentation: diffuse parotid enlargement + sicca symptoms (xerostomia and xerophthalmia)
• Associated with interstitial lymphocytic pneumonitis and peripheral neuropathy
• HLA-DR5 is a risk factor for developing DILS

Oral Cavity

Oral Candidiasis

• Diagnosed via KOH preparation or PAS stain
• CD4 >200 cells/mm³ → topical antifungals (nystatin mouthwash)
• CD4 <200 cells/mm³ → systemic antifungals (fluconazole)
• Risk of fluconazole-resistant candidiasis with repeated courses

Oral Histoplasmosis

• Caused by Histoplasma capsulatum - an AIDS-defining illness
• Endemic to Ohio River Valley region
• Presentation: painful erythematous mucosa → granulomatous lesions ± pseudomembrane; cervical lymphadenopathy
• Diagnosis: biopsy + fungal culture
• Treatment: amphotericin B or itraconazole based on severity/systemic involvement
• Presence in a previously healthy individual should prompt HIV testing

Oral Hairy Leukoplakia (OHL)

• White, corrugated, hyperkeratotic lesion on the lateral tongue
• Caused by Epstein-Barr virus (EBV)
• Lesions are asymptomatic
• Strongly associated with HIV - should prompt HIV workup if found in previously healthy individual
• No specific treatment needed (no malignant potential)
• Differential: lichen planus, idiopathic leukoplakia, carcinoma in situ - requires observation

Gingival and Periodontal Disease

• Pathogenesis: altered immune response + bacterial/fungal overgrowth
• Necrotizing or ulcerative gingivitis is highly suggestive of HIV infection

1. Linear gingival erythema - fiery red band of marginal gingiva out of proportion to dental plaque; persists despite plaque removal; prone to hemorrhage
2. Necrotizing ulcerative gingivitis (NUG) - necrosis of the gingival papillae; presents with odynophagia, hemorrhage, halitosis, and severe oral pain
3. Necrotizing ulcerative periodontitis (NUP) - extension to alveolar bone; rapid loss of bone and soft tissue; may progress to necrotizing stomatitis with exposure of underlying bone

• Debridement and irrigation with chlorhexidine or povidone-iodine
• Systemic antibiotics (metronidazole)
• ART optimization

II. Complicated Infections as a Result of HIV

Cryptococcal Meningitis

• Caused by Cryptococcus neoformans
• An AIDS-defining illness - CD4 count typically <100 cells/mm³
• Presentation: headache, fever, neck stiffness, photophobia, meningismus; can progress to altered mental status and coma
• Diagnosis: lumbar puncture - India ink stain demonstrates encapsulated yeast; latex agglutination for cryptococcal antigen
• Complications: elevated intracranial pressure (managed with serial LPs)
• Treatment:
  • Induction: amphotericin B + flucytosine for 2 weeks
  • Consolidation: fluconazole for 8 weeks
  • Maintenance: fluconazole for 1 year

Cerebral Toxoplasmosis

• Caused by Toxoplasma gondii
• An AIDS-defining illness - CD4 typically <100 cells/mm³
• Most common CNS mass lesion in HIV patients
• Presentation: headache, focal neurologic deficits, seizures
• Imaging: ring-enhancing lesions on MRI
• Diagnosis: empiric treatment, with biopsy only if no response
• Treatment: pyrimethamine + sulfadiazine + leucovorin (folinic acid)
• Prophylaxis: trimethoprim-sulfamethoxazole when CD4 <100 cells/mm³

III. HIV-Associated Malignancies

Kaposi Sarcoma (KS)

• Most common malignancy in HIV+ patients
• Caused by Human Herpesvirus 8 (HHV-8), also called Kaposi sarcoma-associated herpesvirus (KSHV)
• An AIDS-defining illness
• Head and neck involvement: oral cavity (palate most common), pharynx, larynx, skin of face/neck
• Clinical features: violaceous (purple/brown), non-tender, non-pruritic macules/patches → plaques → nodules
• Staging: TIS classification (Tumor, Immune system, Systemic illness)

• Localized disease: local therapies (intralesional chemotherapy, cryotherapy, radiation)
• Disseminated disease: systemic chemotherapy (liposomal doxorubicin or paclitaxel) + ART
• ART alone can cause KS regression

Non-Hodgkin Lymphoma (NHL)

• Second most common malignancy in HIV
• Predominantly B-cell lymphomas (diffuse large B-cell lymphoma most common type)
• EBV is implicated in many cases
• Tends to present at a later stage with worse performance status, B symptoms, and higher Ann Arbor stage vs. HIV-negative patients
• Note: lymphoma (especially Burkitt) does not correlate with low CD4 count

Burkitt Lymphoma (specifically)

• Fastest growing human tumor - doubling time of 24-48 hours
• WHO classification: endemic / sporadic / immunodeficiency-related
• HIV-related: EBV isolated in ~40% of cases
• Differs from endemic form - rarely presents as jaw lesion; more commonly presents as a head/neck or abdominal mass

Cervical Lymphadenopathy

• A common presenting complaint in HIV
• Persistent generalized lymphadenopathy (PGL): defined as enlarged nodes (>1 cm) in ≥2 extra-inguinal sites persisting >3 months with no other explanation
• Common in early HIV infection, associated with vigorous immune response
• Differential in HIV+ patient includes: reactive, mycobacterial (TB, MAC), fungal, KS, lymphoma, metastatic disease

IV. AIDS-Related Illness / Other ENT Manifestations

• Hearing loss - sensorineural hearing loss (SNHL) in HIV+ patients; workup as per normal SNHL algorithm
• Sinusitis - more frequent and severe; commonly caused by typical organisms, but also opportunistic organisms (fungal, Pseudomonas)
• Cervical lymphadenopathy - as noted above
• Facial lipoatrophy - related to nucleoside analogue reverse transcriptase inhibitors (NRTIs); loss of fat in the buccal and temporal regions creating a gaunt appearance; managed with injectable fillers or fat transfer