How should Hughes‑Stovin syndrome be managed, including acute massive hemoptysis, immunosuppressive therapy, anticoagulation considerations, and follow‑up?

Hughes-Stovin Syndrome Management

Treat Hughes-Stovin syndrome with high-dose glucocorticoids and cyclophosphamide as first-line therapy, avoid anticoagulation when pulmonary artery aneurysms are present, and manage life-threatening hemoptysis with embolization rather than surgery.

Immediate Assessment and Risk Stratification

• Assess for hemoptysis severity immediately, as massive hemoptysis (>240 mL/24h or any amount causing respiratory compromise) carries mortality risk up to 59-100% if untreated and is the leading cause of death in Hughes-Stovin syndrome 1, 2, 3.
• Obtain CT pulmonary angiography (CTPA) urgently to identify pulmonary artery aneurysms, their size, stability, and risk of rupture; CTPA has 77-80% diagnostic accuracy and is superior to other imaging modalities 4, 5, 3.
• Screen for deep vein thrombosis in all patients, as the syndrome is defined by the combination of pulmonary artery aneurysms and venous thrombosis 6, 7, 2.
• Evaluate for Behçet's disease features including oral/genital ulcers, uveitis, and skin lesions, as Hughes-Stovin syndrome is considered a variant of Behçet's disease 8, 6, 2.

Primary Immunosuppressive Therapy

Initiate high-dose glucocorticoids and cyclophosphamide immediately upon diagnosis, as this combination is the recommended first-line treatment for pulmonary artery aneurysms in Behçet's disease and Hughes-Stovin syndrome 8.

• Administer intravenous methylprednisolone pulses (typically three successive doses of 500-1000 mg) followed by oral prednisolone 1 mg/kg/day 8.
• Start cyclophosphamide as monthly intravenous pulses (typically 500-1000 mg/m² adjusted for renal function) or daily oral dosing, continuing for at least 6-12 months 8, 6, 7.
• Consider monoclonal anti-TNF antibodies (infliximab) for refractory cases that do not respond adequately to glucocorticoids and cyclophosphamide 8, 7.

Critical Anticoagulation Considerations

Avoid anticoagulation in the presence of pulmonary artery aneurysms due to catastrophic bleeding risk, as this is the most important pitfall in Hughes-Stovin syndrome management 8, 2.

• Rule out pulmonary artery aneurysms before considering anticoagulation for venous thrombosis; if aneurysms are present, anticoagulants are generally contraindicated 8.
• Use immunosuppression rather than anticoagulation to treat the underlying vasculitis causing both thrombosis and aneurysms 8, 6, 2.
• Consider anticoagulation only in exceptional circumstances (e.g., intracardiac thrombi, massive pulmonary embolism without aneurysms) with extreme caution and only after multidisciplinary discussion 2.
• Insert an inferior vena cava filter if recurrent pulmonary embolism occurs despite adequate immunosuppression and anticoagulation remains contraindicated 8, 6.

Management of Acute Massive Hemoptysis

Proceed directly to bronchial artery embolization (BAE) without delay in hemodynamically unstable patients, as delaying intervention significantly increases mortality 1, 5.

• Intubate immediately with a single-lumen cuffed endotracheal tube to allow bronchoscopic suctioning and clot removal if respiratory compromise is present 1, 5.
• Avoid BiPAP entirely in massive hemoptysis, as positive pressure ventilation worsens bleeding 1.
• Perform BAE as first-line therapy, which achieves immediate hemostasis in 73-99% of cases; over 90% of massive hemoptysis originates from bronchial arteries 1, 4, 5.
• Reserve surgical intervention (lobectomy/pneumonectomy) only for life-threatening situations where embolization has failed or is not feasible, as surgical mortality in Hughes-Stovin syndrome is high (16% in massive hemoptysis series) 8, 1, 2.
• Prefer transcatheter arterial embolization over open surgery for patients at high risk of major bleeding from pulmonary aneurysms 8.

Medical Management During Active Hemoptysis

• Stop all NSAIDs immediately, as they impair platelet function and worsen bleeding 1, 4, 5.
• Discontinue anticoagulants during active bleeding episodes to reduce hemorrhage risk 5.
• Administer antibiotics for hemoptysis ≥5 mL, as bleeding may represent pulmonary infection or exacerbation 1, 4, 5.
• Stop all airway clearance therapies immediately in massive hemoptysis to allow clot formation 1.
• Avoid aerosolized hypertonic saline, as it can exacerbate bleeding 1.

Bronchoscopic Interventions for Hemoptysis Control

• Use bronchoscopy for airway clearance and tamponade rather than diagnosis in unstable patients 4.
• Apply topical hemostatic tamponade with oxidized regenerated cellulose mesh, which arrests hemoptysis in 98% of cases 1, 5.
• Consider thermal ablation techniques (argon plasma coagulation, Nd:YAG laser, electrocautery) for visible central airway lesions 1, 4.
• Instill iced saline solution to constrict blood vessels and control bleeding 1, 5.
• Use bronchial blockade balloons for tamponade of bleeding bronchus 1, 5.

Follow-Up and Long-Term Management

• Monitor for recurrent hemoptysis closely, as recurrence occurs in 10-55% of cases after initial BAE, with higher rates in pulmonary aneurysms 1, 4, 5.
• Perform repeat CTPA at 3-6 month intervals to assess aneurysm stability and response to immunosuppression 3.
• Continue immunosuppression for at least 12-24 months, with gradual glucocorticoid taper guided by clinical response and imaging findings 7, 9.
• Reassess for Behçet's disease manifestations regularly, including ophthalmologic examination for uveitis 8.
• Consider repeat BAE for recurrent hemoptysis, as recent studies show no increased morbidity or mortality with repeat interventions 1, 4.

Common Pitfalls to Avoid

• Never delay BAE for CT imaging or bronchoscopy in unstable patients with massive hemoptysis, as this significantly increases mortality 1, 5.
• Never anticoagulate patients with documented pulmonary artery aneurysms without ruling out bleeding risk, as fatal hemorrhage is the leading cause of death 8, 2, 3.
• Never perform surgery as first-line treatment for pulmonary aneurysms; medical management with immunosuppression must be optimized first 8, 2.
• Never use cyclosporine-A in Hughes-Stovin syndrome, as it should be avoided in nervous system involvement and is not recommended for arterial disease in Behçet's disease 8.