Clinical Presentation & Protocol
Patient Usually Complains Of
Patient presents for evaluation of progressive exertional dyspnea and non-productive cough. History significant for recurrent spontaneous pneumothorax or chylous effusions. Review of systems negative for systemic vasculitis symptoms but positive for fatigue. Known history of TSC or incidental findings on prior HRCT suggestive of diffuse cystic lung disease.
Clinical Examination Findings
General: Patient in no acute distress, resting saturations [X]% on room air. Respiratory: Bilateral decreased breath sounds, occasional fine end-inspiratory crackles. Chest wall: No localized tenderness. Extremities: No peripheral edema or signs of DVT. Skin: Evaluation for angiofibromas or shagreen patches (TSC stigmata).
Treatment Protocol
Initiate Sirolimus (mTOR inhibitor) therapy at [X] mg daily, targeting trough levels of 5-15 ng/mL. Monitor CBC, LFTs, and lipid profile. Pulmonary rehabilitation referral. Smoking cessation counseling. Avoid estrogen-containing contraceptives. Annual PFTs and HRCT surveillance as indicated.