Diffuse alveolar hemorrhage (DAH), a catastrophic complication of connective tissue disorders (CTDs), manifests as rapid-onset hypoxemia, alveolar infiltrates, and progressive bleeding into the airways. While immune-mediated alveolar–endothelial injury primarily drives its pathophysiology, diagnosis is based on bronchoscopy and chest imaging. The clinical urgency lies in securing the compromised airway and stabilizing respiratory failure, a challenge increased by CTD-specific anatomical alterations such as cervical spine instability, cricoarytenoid arthritis, and subglottic stenosis. High-dose corticosteroids and immunosuppression are essential, while severe cases require extracorporeal membrane oxygenation or plasmapheresis. This comprehensive review introduces two novel approaches to address fundamental gaps in the management of CTD-induced DAH: a structured algorithm for a CTD-specific airway risk stratification tool, integrating anatomical screening and the application of lung ultrasounds (LUSs) for post-intubation CTD-induced DAH ventilation management. The need for a multidisciplinary team approach is also discussed. Despite aggressive care, mortality remains high (25–50%), underscoring the necessity for improved early recognition and intervention strategies for these high-risk patients.