Can saddle‑nose deformity occur in ANCA‑associated vasculitis such as granulomatosis with polyangiitis?

Saddle Nose Deformity in ANCA-Associated Vasculitis

Yes, saddle-nose deformity is a well-recognized and common complication of ANCA-associated vasculitis, particularly granulomatosis with polyangiitis (GPA), occurring in up to 25% of patients as progressive cartilage destruction develops. 1, 2

Mechanism and Pathophysiology

The saddle-nose deformity in GPA results from necrotizing vasculitis and granulomatous inflammation that progressively destroys the nasal cartilaginous structures, particularly the cartilaginous dorsum and nasal septum. 3, 1 This is distinct from the vascular injury pattern seen in microscopic polyangiitis, which primarily affects capillaries without granuloma formation and does not typically cause nasal cartilage destruction. 4

Clinical Presentation and Associated Features

Upper respiratory tract involvement occurs in over 90% of GPA patients, making sinonasal manifestations a cardinal feature of the disease. 1 The progression typically follows this pattern:

• Early manifestations: Persistent nasal bleeding, crusting, nasal obstruction, and epistaxis 1
• Progressive disease: Nasal septal perforation develops as cartilage destruction advances 1, 2
• Advanced disease: Saddle-nose deformity emerges in up to 25% of patients with worsening cartilage destruction 2
• Associated findings: Chronic rhinosinusitis, friable erythematous mucosa with granulation tissue on nasal endoscopy, and loss of mucociliary function 3, 2

Diagnostic Considerations

The combination of clinical features, positive ANCA serology (PR3-ANCA present in 80-90% of GPA cases), and biopsy evidence of necrotizing vasculitis with granulomatous inflammation establishes the diagnosis. 3, 5, 1 However, a critical diagnostic pitfall exists: nasal biopsies frequently lack sufficient diagnostic features specific to GPA, necessitating tissue sampling from other affected organs such as lung or kidney to obtain definitive histologic confirmation. 3, 1

In disease limited to the sinonasal tract, ANCA may be negative, complicating diagnosis—the likelihood of positive serum ANCA increases with more widespread and severe clinical involvement. 1

Differential Diagnosis

Saddle-nose deformity can also occur in other conditions, which must be excluded:

• Infectious causes: Leprosy and syphilis 6
• Other inflammatory conditions: Relapsing polychondritis 6
• Inflammatory bowel disease: Crohn disease has been associated with nasal cartilage collapse 6
• Trauma: Direct nasal injury 6
• Idiopathic: Some cases arise without evident precipitating cause 6

Reconstruction Considerations

Surgical reconstruction of saddle-nose deformity in GPA patients is safe and effective when performed during minimal or no local disease activity, though an increased rate of revision surgery (approximately 16% requiring revision) should be anticipated compared to non-GPA patients. 7, 8

Key surgical principles based on systematic review evidence:

• Optimal graft technique: Single L-shaped strut grafts with autologous tissue demonstrate superior outcomes compared to individually placed grafts 9
• Preferred graft materials: Split-calvarial bone appears to have slightly lower complication rates than costal cartilage, though both are acceptable autologous options 9, 8
• Increased risk factors: Graft failure risk increases with higher numbers of overall grafts and use of non-autologous tissue 9
• Overall success rate: 84.1% success rate for rhinoplasty (primary and secondary combined) with 20% complication rate 9
• Timing: Surgery should be reserved for patients with minimal or no local disease activity and unresponsive to maximal medical therapy 2, 7

Medical Management of Sinonasal Disease

Before considering reconstruction, aggressive medical management should be optimized:

• Culture-directed antibiotics for bacterial colonization (Staphylococcus aureus colonization occurs in 72% of GPA patients) 5
• Topical antibiotic and saline irrigations 2
• Occasional debridement of adherent crusts to reduce frequency of sinonasal exacerbations and improve obstructive symptoms 2
• Systemic immunosuppressive therapy for active disease 2