A 42-year-old woman with a 10-year history of chronic rhinosinusitis and recent nasal polyp surgery presents with recurrent epistaxis from the anterior nasal septum. Examination reveals a granular, friable mucosa with petechial haemorrhages. Coagulation profile is normal. Chest X-ray shows bilateral hilar lymphadenopathy. What is the most likely diagnosis?

## Sarcoidosis — Nasal and Systemic Manifestations **Key Point:** Sarcoidosis is a multisystem granulomatous disease of unknown etiology. Upper respiratory tract involvement occurs in ~20% of cases and can manifest as chronic rhinosinusitis, nasal polyps, and epistaxis from granular, friable nasal mucosa. **Bilateral hilar lymphadenopathy** is the hallmark radiological finding, present in up to 90% of pulmonary sarcoidosis cases. ### Clinical Features Supporting Sarcoidosis | Feature | Finding in This Case | Sarcoidosis Relevance | |---------|----------------------|-----------------------| | **Chronic rhinosinusitis + nasal polyps** | Yes (10-year history, recent polypectomy) | Nasal sarcoidosis mimics CRS; polyps are a recognized manifestation | | **Granular, friable mucosa with petechiae** | Yes | Non-caseating granulomas infiltrate nasal mucosa | | **Bilateral hilar lymphadenopathy** | Yes | Classic radiological hallmark (Stage I/II sarcoidosis) | | **Normal coagulation profile** | Yes | Rules out coagulopathy; consistent with granulomatous disease | | **Recurrent epistaxis** | Yes | Friable granulomatous mucosa bleeds easily | ### Why NOT GPA (Granulomatosis with Polyangiitis)? - GPA causes **necrotizing** vasculitis with saddle-nose deformity, septal perforation, and "strawberry gingivitis" — not typically nasal polyps - Pulmonary involvement in GPA manifests as **nodules, cavities, or infiltrates** — NOT bilateral hilar lymphadenopathy - Bilateral hilar lymphadenopathy is **not** a recognized feature of GPA; it is the hallmark of sarcoidosis ### Why NOT HHT (Hereditary Haemorrhagic Telangiectasia)? - HHT presents with **telangiectasias** on lips, oral mucosa, and fingertips; family history is typical - Does NOT cause hilar lymphadenopathy or nasal polyps - Coagulation profile is normal in HHT, but the mucosal appearance is telangiectatic, not granular/friable ### Pathophysiology of Nasal Sarcoidosis ``` CD4+ T-cell activation → Macrophage recruitment → Non-caseating granuloma formation → Nasal mucosal infiltration → Granular, friable mucosa → Epistaxis → Mediastinal lymph node involvement → Bilateral hilar lymphadenopathy ``` ### Diagnostic Approach **High-Yield:** The diagnostic workup for suspected sarcoidosis includes: 1. **Chest X-ray / HRCT chest** — bilateral hilar lymphadenopathy (Stage I) or parenchymal infiltrates (Stage II) 2. **Serum ACE (Angiotensin-Converting Enzyme)** — elevated in ~60% of active sarcoidosis 3. **Nasal/sinus biopsy** — non-caseating epithelioid granulomas without central necrosis 4. **Serum calcium, 24-hour urine calcium** — hypercalcemia/hypercalciuria in ~10–20% 5. **Ophthalmology referral** — uveitis in ~25% **Clinical Pearl:** A patient with "refractory chronic rhinosinusitis," nasal polyps, and bilateral hilar lymphadenopathy should be investigated for sarcoidosis before attributing symptoms to allergic or infectious etiology. The combination of upper airway granulomatous disease + bilateral hilar lymphadenopathy is pathognomonic for sarcoidosis until proven otherwise. **Mnemonic: SARCOID** — **S**kin (erythema nodosum, lupus pernio), **A**CE elevated, **R**espiratory (hilar LAD), **C**alcium elevated, **O**cular (uveitis), **I**nfiltrates (non-caseating granulomas), **D**iagnosis by biopsy ### Treatment - **Systemic corticosteroids** (prednisolone) — first-line for symptomatic disease - **Methotrexate / Hydroxychloroquine** — steroid-sparing agents - **Topical intranasal steroids** — for localized nasal disease [cite: Harrison's Principles of Internal Medicine 21e, Ch 360 (Sarcoidosis); Robbins & Cotran Pathologic Basis of Disease 10e, Ch 13]