1. RESTRICTIVE LUNG DISEASES THE UNUSUAL ONES Martha Richter, MSN, CRNA

2. OBJECTIVES • The student will: • Identify 3 unusual syndromes with restrictive lung effects • List specific problems that may be anticipated when caring for patients with these diagnoses

3. WEGENERS GRANULOMATOSIS • Systemic necrotizing granulomatous vasculitis • Upper &lower respiratory tract, kidneys primarily. Less common to other organ systems • Glomerulonephritis=generalized disease

4. WEGENERS GRANULOMATOSIS • May have: • Endobronchial lesions • Subglottic stenosis • Ulcerating tracheobronchitis • Tracheal/bronchial stenosis • Airway hemorrhage • Sinusitis • Rhinorrhea with nasal ulceration

5. WEGENERS GRANULOMATOSIS • 20% Have cardiac manifestations: • Pericarditis, cardiomyopathy, pancarditis, coronary arteritis • CXR=infiltrates, nodules, cavities, interstitial changes, pleural effusions • Treatment: corticosteroids L& cyclophosphamide (90% partial remission rate, 75 complete remission rate)

6. WEGENERS GRANULOMATOSIS • ANESTHETIC CONSIDERATIONS • Avoid nasotracheal intubations • Be aware of subglottic stenosis that may accompany this dx • Ophthal consult to clear eyes • Stress dose corticosteroids • Adjust drug dosages in the case of renal insufficiency

7. GOODPASTURES SYNDROME • Autoimmune; unknown pathogenesis • Predominantly in young adults • Presents with diffuse pulmonary hemorrhage & rapidly progressive glomerulonephritis • Leads to renal failure & fatal hemoptysis (rapid course)

8. GOODPASTURES SYNDROME • PFTS show restrictive pattern • Subsequent lung disease (e.g. smoking) may potentiate • Hypochromic microcytic iron deficiency anemia from chronic airway bleeds

9. GOODPASTURES SYNDROME • No elective surgery until disease is quiescent • CXR • PFTs incl diffusing capacity • ABGs • Renal status • UA/blood chem

10. GOODPASTURES SYNDROME • ANESTHETIC CONSIDERATIONS • Large ETT to accommodate pulmonary toilet • Avoid high airway pressures • Assess for prerenal factors: hypovolemia, dec cardiac output • Art line • PA catheter for proced with significant fluid shifts

11. SARCOIDOSIS • Noncontagious multisystem granulomatous disease of unknown cause • Blacks more often than caucasians • Ages 20-50 • Presentation related to distribution of granulomas • May be asymptomatic until markedly widespread

12. SARCOIDOSIS • Restrictive pattern by PFTs • Flow volume loops may detect small airway involvement before PFTs do • Hypoxic at rest • Bilateral hilar & paratracheal adenopathy • Fibrocystic & bullous changes with extensive scarring

13. SARCOIDOSIS • ANESTHETIC CONSIDERATIONS • Asymptomatic routine monitoring & care • All Cranial nerves will be affected: document any dysfunction and exercise special care with positioning • 25% have cardiac involvement, but asymptomatic

14. SARCOIDOSIS • CRANIAL NERVE ISSUES: • I (Olfactory) • II-visual disturbances • III, IV, VI-ophthalmoplegia • VII-Bells Palsy • VIII-deafness & vertigo • IX, X-depressed gag & dysphagia • X-hoarseness, vocal paralysis

15. SARCOIDOSIS • Cardiac infiltration: • May be related to infil of conduction system: arrhythmias,heart block, sudden death • Pericardial effusions • Cardiomyopathy • RX with steroid, immunosuppressors, pacemaker, AICD

16. SARCOIDOSIS • Laryngeal sarcoid: • Dysphonia, dysphagia, may have diffuse supraglottic edema • May have vocal cord granuloma • May have diffuse tracheal stenosis • Use smaller diameter ETT

17. SARCOIDOSIS • Anesthetic considerations • Need to know areas affected • Tailor the careplan to the patient’s needs • Check renal function (may be involved) • Issues of fluid balance, cardiac function, electolytes, ability to metabolize drugs

18. REMEMBER • There are many “rare” diseases that may impact many systems, including the lungs • Know where to look for information • Just because it’s rare doesn’t mean you won’t see it! • Be careful.