Case 1 69 yo M 3 year history of intermittent cough and exertional - - PDF document

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Case 1 69 yo M 3 year history of intermittent cough and exertional - - PDF document

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UCSF School of Medicine Multidisciplinary Diagnosis in Action: Challenging Case Presentations Interstitial Lung Disease: Advances in Diagnosis and Management UCSF CME November 8, 2014 Case 1 69 yo M 3 year history of intermittent cough and

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Multidisciplinary Diagnosis in Action: Challenging Case Presentations

Interstitial Lung Disease: Advances in Diagnosis and Management UCSF CME November 8, 2014

Case 1 – 69 yo M

  • 3 year history of intermittent cough and

exertional dyspnea

  • Over prior 2 years, increased functional

limitation, now SOB walking across room

  • New exertional hypoxemia
  • ROS: Negative, no reflux symptoms or

symptoms of connective tissue disease

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69 yo M

  • PMHx

– R‐sided primary spontaneous pneumothorax in his 20’s, pleurectomy – GERD

  • NKDA
  • Medications

– Pantoprazole 20 mg daily – ASA 81 mg daily – Losartan 50 mg daily

Environmental / Occupational History

  • Down comforter, used in winter
  • No birds
  • No other mold exposures
  • No occupational exposures
  • Works in media and runs a non‐profit
  • rganization
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69 Yo M

  • Social Hx

– Lifetime non‐smoker – No alcohol or illicit drug abuse

  • FHx:

– No known ILD – Mother had PMR

Physical Exam

  • BP 120/56, HR 65, T 36.9, RR 18, Sats 96% on

R/A

  • Mild bibasilar velcro crackles R>L
  • Otherwise unremarkable
  • Normal cardiac exam
  • No clubbing
  • No signs of PH
  • No signs of CTD
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Physiology

  • Oxygen saturation 97% on R/A at rest but

decreased to 85% with 3 mins walking

FVC FEV1 FEV1/FVC TLC DLCO March 4, 2014 3.19 L (67%) 2.72 L (77%) 74% 4.90 L (66%) 11.97 (35%) May 23, 2011 3.86 L (80%) 3.15 L (88%) 85% 5.24 L (71%) 15.48 (44%)

Labs

  • June 2011
  • ESR 31 mm/hr
  • Negative ‐ Scl‐70, ds‐DNA, C3, C4, D‐dimer, Jo‐

1, anti‐centromere Ab, ACE level, anti‐CCP Ab

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MDD discussion

  • Dx – unclassifiable without additional

information

  • We recommended SLB

Surgical Lung Biopsy

  • 3 surgical biopsies from the left lung

– All lower lobe (basilar anterior x 2 and superior) – Discussed during surgery

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Case 1 Diagnosis

  • Interstitial fibrosis, usual interstitial pneumonia

pattern, with increased peribronchiolar fibrosis (basilar).

  • Interstitial fibrosis, predominantly

bronchiolocentric, with small non‐necrotizing granulomas (superior).

  • Overlapping features that suggest chronic

hypersensitivity pneumonia versus usual interstitial pneumonia.

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Multidisciplinary Decision

  • Based on clinical, radiologic and surgical lung

biopsy, felt to be most consistent with hypersensitivity pneumonitis

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Multidisciplinary Management Plan

  • Remove down comforter from house
  • HP checklist
  • Professional home cleaning
  • Prednisone 50 mg daily x 4 week then taper

by 5 mg / week until on 20 mg and return with PFTs after 3 months of treatment

  • Rehab, vaccines
  • Serial PFTs

Final Thoughts from the Panel

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Case 2 ‐ 77 yo M

  • Developed dry cough that started 5 years ago
  • Denies dyspnea, although he has "slowed

down" over the last several years.

  • Denies constitutional symptoms
  • No reflux
  • Denies myalgias, arthralga, sicca, Raynaud’s

phenomena, rash, skin changes, joint stiffness

  • r muscle weakness

77 yo M

  • PMHx

– BPH

  • NKDA
  • Medications

– Finasteride 5 mg daily – Tamsulosin 0.4 mg daily

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Environmental / Occupational History

  • Retired factory (HVAC) and field worker
  • Owned parakeets and cockatiels for past 2‐3

years; kept outside away from home

  • Changes the cage q 2 weeks; feeds them on

alternating days. Does not handle the birds

  • Woodworking as a hobby (builds furniture)

77 yo M

  • Social Hx

– Minimal smoking history – No alcohol or illicit drug use

  • Family Hx

– No ILD or CTD

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Physical Exam

  • BP 128/69, HR 58, T 36.5, RR 20, Sats 99% R/A
  • Bilateral inspiratory crackles at both bases
  • No clubbing
  • Negative rheumatologic exam
  • No signs of pulmonary hypertension

Case 2 ‐ PFTs

  • Office walk test: Room air saturation at rest

97%, after 3 minutes walking 96%

Date FVC FEV1 FEV1/FVC TLC DLCO June 24, 2014 3.70 L (117%) 2.90 L (118%) 78% 5.86 L (92%) 17.16 (62%) July 12, 2013 3.81 L (119%) 3.18 L (128%) 84% 5.61 L (88%) 19.30 (70%)

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Surgical Lung Biopsy

  • Two biopsies, right upper and lower lobes.

– 3 x 1 cm – “Similar wedge that is approximately 5 cm”

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Case 2 ‐ Diagnosis

  • Interstitial fibrosis, usual interstitial

pneumonia pattern.

  • Minor component of chronic inflammation,

but associated with the regions of fibrosis.

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Initial Multidisciplinary Discussion

  • Given clinical, radiology and surgical lung

biopsy, felt the diagnosis is idiopathic pulmonary fibrosis

Multidisciplinary Management Plan

  • Remove birds
  • Pulmonary rehab
  • Serial PFTs
  • Vaccines
  • Baseline serologies
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Labs

  • ANA 1:640 (speckled)
  • RF 125
  • SSA >150
  • SSB 145
  • Anti‐CCP, Scl‐70, SM antibody, Sm/RNP

antibody, PM‐Scl antibody negative

  • Myositis panel negative

– EJ, Jo‐1, KU, MI‐2, OJ, PL‐12, PL‐7,SRP autoantibodies

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Multidisciplinary Management Plan

  • Lip biopsy to r/o Sjogren’s
  • Rheumatology consult

Final Thoughts from the Panel

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Case 3 ‐ 46 yo M

  • Developed exertional dyspnea and non‐productive

cough in spring 2013

  • Progression of dyspnea over past year
  • Cardiac work up including angiogram was negative
  • Endorses symptoms of reflux
  • No CTD symptoms except dry mouth
  • Presented to ER Feb 2014 with worsening SOB, treated

with solumedrol, antibiotics. CT and SLB arranged.

  • Discharged home on prednisone, PPI and NAC

46 yo Male

  • PMHx
  • HTN
  • T2DM
  • Dyslipidemia
  • GERD
  • Renal cyst
  • Periodic limb movement

disorder

  • B12 deficiency
  • AVN of hip with hip

replacement

  • OSA
  • Medications
  • Diltiazem 180 mg daily
  • Lovastatin 40 mg daily
  • Metformin 1000 mg BID
  • Omeprazole 20 mg daily
  • Prednisone 10 mg daily
  • NKDA
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Environmental / Occupational History

  • Down pillow
  • No birds
  • Regular humidifier use
  • Mold on central air filters
  • Worked as a manager and engineer for

construction companies on dusty work‐sites

46 yo M

  • Family Hx

– Father ‐ “black lung”

  • Died at 41

– 2 paternal uncles, 1 aunt with ILD

  • Died at 46, 46, and 51

– Grandmother – ILD

  • Died 72

– History of early graying in 30s

  • Social Hx

– Life long non‐smoker – No illicit drugs – 7 alcohol units weekly

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Physical exam

  • BP 137/88, HR 117, RR 18, T 36.4, Sats 96%

R/A

  • No clubbing
  • No crackles, wheeze
  • No signs of pulmonary hypertension
  • No signs of connective tissue disease

Physiology

Date FVC FEV1 FEV1/FVC TLC DLCO

March 3, 2014 2.43L (53%) 2.16L (58%) 89% 3.55L (54%) 14.4 (46%) May 11, 2011 3.10 L (60%) 2.63L (64%) 85% Room air saturation was 94 % at rest. After 3 minutes

  • f walking, oxygen saturation was 92 %.
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Labs

  • WBC 13, Hb 12.6, Plt 165
  • CPK normal
  • ANA, RF, ANCA negative
  • HP panel negative
  • ACE normal
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Surgical Lung Biopsy

  • Left upper lobe surgical biopsy

– 3.6 x 1.6 x 0.7 cm

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Case 3 ‐ Diagnosis

  • Cellular and fibrosing nonspecific interstitial

pneumonia with focal bronchiolocentric fibrosis and organizing pneumonia.

  • Alveolar hemorrhage, likely procedure‐

related.

  • Connective tissue disease, HP, drug reaction,
  • r familial ILD (known at time of dx).
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Multidisciplinary Discussion

  • Unclassifiable ILD
  • Differential includes
  • Chronic hypersensitivity pneumonitis (HP)
  • Fibrotic nonspecific interstitial pneumonia (NSIP)
  • With a familial predisposition
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Case 3 ‐ Management

  • Pulmonary rehab, Vaccines
  • Mycophenolate (MMF) 1000 mg twice daily
  • Wean prednisone once on full dose of MMF
  • Continue PPI
  • Weight loss
  • Remove down and humidifier
  • Serial PFTs
  • Repeat serologies (Negative)

Final Thoughts from the Panel