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Practical Application: Case Reviews

Practical Application: Case Reviews. 51-Year-Old Man with IPF and Worsening Dyspnea: History . Obese white male with IPF (thoracoscopic lung biopsy) Remote smoking history Minimal GERD symptoms No occupational exposure No family history of IPF No CTD symptoms.

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Practical Application: Case Reviews

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  1. Practical Application:Case Reviews

  2. 51-Year-Old Man with IPF and Worsening Dyspnea: History Obese white male with IPF (thoracoscopic lung biopsy) Remote smoking history Minimal GERD symptoms No occupational exposure No family history of IPF No CTD symptoms

  3. Tests at Presentation (2005) • PFTs • FVC: 69% predicted • TLC: 64% predicted • DLCO: 61% predicted • FEV1/FVC ratio 82% • RA ABG: pH 7.42; pCO2 37 mmHg; pO2 88 mmHg • 6MWT: desaturation to 91% on room air • 6MWD: 1250 feet • Echocardiogram: normal RV and LV function but tricuspid regurgitation inadequate to determine RVSP

  4. HRCT at Presentation

  5. Clinical Course • Late 2006 • Initial Treatment: pred/AZA, NAC • Increased dyspnea • FVC 60% predicted • DLCO 49% predicted • Desaturation to 78% on 6MWT • Therapy with mycophenolate initiated • 2007–2008 • PFTs stabilized • No significant change in dyspnea • ECHO 1/08: RVSP 44 mmHg • Bosentan started without improvement, discontinued 4/08

  6. Clinical Course June 2008 • Worsening dyspnea (now at rest) over 2 weeks • No fever, chills, or night sweats • Admitted to a local hospital • O2 saturation 90–91% on 100% O2 • BIPAP • Used to maintain oxygenation • Initially on room air, later with O2 supplementation • Patient remained afebrile over 48h after admission • Blood and sputum cultures negative • Treatment: high dose steroids, diuretics, and antibiotics • Bronchoscopy not performed due to severity of illness • Transferred to regional center on day 9 of hospitalization

  7. HRCT – On Admission

  8. Clinical Course • Nitric oxide given on day 2 after transfer with minimal improvement in oxygenation • Transplant re-evaluation • Evaluation completed in 48h • Double lung transplant 24h later • Extubated on POD 2 • On room air POD 5 • Back to work in 6 months on room air

  9. Usual Interstitial Pneumonia nl f

  10. Explant Lung Pathology

  11. Potential Patterns of Acute Exacerbation in IPF Diffuse Alveolar Damage Fibrinous and Organizing Interstitial Organization Intra-alveolar Organization

  12. Pathology Summary Acute exacerbation in IPF appears to be part of the natural history of the disease The acute disease usually has features of DAD or organizing pneumonia; OP tends to be steroid responsive “Acute on chronic” lung disease manifestations are not restricted to IPF

  13. Clinical Features of AE of IPF • May occur at any time during course of IPF • New or worsening dyspnea within 30 days (typically 2-4 weeks) • Low-grade fever, worsening cough, flu-like symptoms • Presentation with severe hypoxemia and respiratory failure Akira M, et al. Am J Respir. 1997:168-179. Ambrosini V, et al. Eur Respir J. 2003;22:821-826.

  14. Take Home Messages: AE of IPF • Definition: clinical worsening (typically over 2-4 weeks) not associated with an identifiable cause • Deterioration characterized by • Fever • Worsening cough, dyspnea, and gas exchange • New bilateral GGO ± consolidation on HRCT • In the absence of a specific diagnosis, pulse steroids and broad spectrum antibiotics should be considered; immunosuppression may be subsequently considered • Patients requiring mechanical ventilation for hypoxemic respiratory failure have high mortality • Transplantation is an option

  15. 66-Year-Old Man with UIP: History • In spring of 2001, increased dyspnea on exertion, worse with stairs and minimal dry cough • Dyspnea not associated with chest pain, lightheadedness, dizziness, palpitations, or diaphoresis • Medical History: • Left adrenal mass • Obstructive sleep apnea • Insomnia • Allergic rhinitis • Coronary artery disease • Hypertension • Diabetes • Tracheostomy at age 7 for strep throat • RCA stent placement in 1999 • No known allergies

  16. History Family History: Significant for CAD Patient's mother died at age of 79 of CHF 1 sibling and 3 children and are healthy, no respiratory disease Social History Patient owns a wholesale bakery Very active gardener Lately unable to maintain his tomato garden due to his lung disease Patient denies any smoking history Occasional alcohol Married for 42 years with 3 adult children No pets or birds Born and raised in California, lived in the SF Bay Area since 1994 Review of systems Symptoms consistent with GERD

  17. Clinical Course: Diagnosis and Treatment • Patient was evaluated at regional pulmonary center February 2004 • Thoracoscopic lung biopsy May 2004 • Pathology consistent with the usual interstitial pneumonia (UIP) • Clinical: mild-to-moderate disease, and the patient was referred for pulmonary rehabilitation • Initially treated with prednisone and azathioprine, but he did not tolerate prednisone, ie, weight gain, irritability • Symptoms progressed despite therapy • Mycophenolate mofetil initiated April 2005, increased to 1.5 g bid; prednisone was tapered to zero • Patient has done quite well on mycophenolate mofetil • Stabilization of his PFTs • Increased energy • PFTs and HRCT suggest only mild progression of disease • Mr. U had an initial pre-transplant evaluation but disease was too mild to become viable LT candidate

  18. Current Medications Mycophenolate mofetil 1500 mg bid Sulfamethoxazole and trimethoprim 5 days per week Furosemide 20 mg daily Cetirizine 10 mg daily Isosorbide 30 mg daily Atorvastatin 20 mg daily Diltiazem 180 mg daily Valsartan 80 mg daily Zolpidem 10 mg nightly Rabeprazole 20 mg bid Aspirin 325 mg daily Ibuprofen 200 mg daily Acetaminophen 650 mg daily N-Acetylcysteine 600 mg tid

  19. Clinical Course: Sleep Mr. U. was diagnosed with OSA by a sleep study at a community hospital Patient likes to sleep on his stomach and has not been able to find an acceptable CPAP mask Weight loss and nocturnal oxygen saturation study recommended

  20. Clinical Exam General Appearance: mildly obese HEENT: Normocephalic and atraumatic Extraocular movements intact Pupils equal, round, and reactive Sclerae anicteric Oropharynx clear Nose was within normal limits Neck Thick and short, collar size 17 Supple, no thyromegaly Nodes: No cervical or axillary lymphadenopathy Back: No costovertebral angle tenderness Lungs Bilateral air entry Few bibasilar rales

  21. Clinical Exam (cont) Cardiac Regular rhythm Regular heart rate = 100 bpm Blood pressure = 120/66 No jugular venous distention No murmurs or gallops are appreciated Breasts: No masses or nipple discharge Abdomen Soft, obese, and nontender Normal bowel sounds present No palpable organomegaly Extremities No cyanosis at rest 1+ clubbing No edema Distal pulses were equal and intact Neurologic and Psychiatric Patient was alert and oriented Cranial nerves II through XII intact Motor examination was nonfocal

  22. HRCT 8/04 5/09 • Subpleural reticulation with a basal and posterior predominance • Minimal progression of abnormalities

  23. Change in HRCT Stable enlargement of pretracheal, precarinal, and subcarinal lymph nodes Lymphadenopathy No evidence of axillary or hilar Stable mediastinal Coronary artery calcification is noted No pleural or pericardial effusions Re-evaluation of lung parenchyma reveals area of subpleural reticulation and ground-glass opacity with architectural distortion and traction bronchiectasis Small hiatal hernia detected; no evidence of suspicious lytic or sclerotic bony lesions Minimal worsening of fibrosis on 5/09 compared with 8/04

  24. Clinical Course PFT Interpretation Moderate restrictive ventilatory defect Severe reduction in DLCO in July 2004 Improved in 2005 Progressed in 2009 ECHO, 3/07 LVH with normal RV function Estimated RVSP-35 mm Hg, mild PH Laboratory Data Comprehensive metabolic panel, CBC, PT, and INR unremarkable Only abnormality noted was rheumatoid factor, positive (22) PFT Changes

  25. Case Summary Mr. U is a 66-year-old gentleman with ILD Lung biopsy is consistent with UIP, but HRCT and clinical course are not typical of IPF Ground glass opacities but no honeycombing on HRCT Initial clinical improvement and then stabilization on mycophenolate mofetil Non-specific interstitial pneumonia (NSIP) can be more responsive to immunosuppressive therapy and has a better prognosis than UIP/IPF Management Plan • Continue medications • Mycophenolate mofetil for immunosuppression • Sulfamethoxazole and trimethoprim DS TIW for PCP prophylaxis • Rabeprazole for reflux • Repeat sleep study • Follow-up visit with transplant team in 6 months • Right heart catheterization to evaluate for pulmonary hypertension

  26. Take Home Messages All that is UIP is not IPF UIP is a pathological diagnosis, not a clinical one Need to look at entire body of data, ie, HRCT, clinical presentation and response to therapy, and progression of disease IPF itself is heterogeneous so Mr. U could still have an atypical variant of IPF Although patients with UIP on biopsy and atypical HRCTs are known to have a better prognosis than patients with a concordant diagnosis, more research and studies are needed to determine the prevalence and significance of this entity

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