PULMONOLOGY CASE PRESENTATION

Dr. GJ Visagie

Patient

43 yo FemaleKnown with

Obesity Class 3 Recently lost 45 Kg Obstructive sleep apnea Pulmonary hypertension and Cor Pulmonale Polycystic ovarian Syndrome Significant smoking history Benign intracranial hypertension

Patient (Continued)

Admitted to neurology with headache While in ward she experienced a episode

of chest pain. Caused by pulmonary hypertension In unit it was noticed that the patient had

a low oxygen saturation Pulmonology was consulted!!!

Initial Thoughts

Pulmonary embolism COPD with respiratory failure Obesity hypoventilation syndrome Infective lung process

Physical examination

Overweight middle aged female Hypertrichosis of face Central cyanosis Plethoric Comfortable with no distress CVS: Loud P2 and heaving Right ventricle Resp: Fine late inspiratory crackles Abd: Central obesity

Further investigationsCXR

Flow volume curve

Flow Volume (Cont)

Blood investigations

Arterial blood gas pH 7,37 pO2 52,6 pCO2 37,6 SaO2 87%

Full Blood count HB 17,8 WCC 6,08 Plt 190

Thyroid function

Lipid profile

Raised GGT

HRCT

Additional Blood investigations

ANCA Negative ANF Negative RF Negative S-Ace Negative HIV Negative

Summary of patient

Patient with Interstitial lung disease Type 1 respiratory failure Long smoking history Obstructive sleep apnea Obesity stage 3

Smoking related interstitial lung disease

Introduction

Introduction

Diseases associated with smoking

Desquamative interstitial Pneumonia Respiratory bronchiolitis-associated

interstitial lung disease Pulmonary Langerhans cell histiocytosis

Desquamative interstitial pneumonia (DIP)

90% of patients with this diagnosis have smoked

Male predominance Clinical and radiologically non specific

findings Dyspnea and cough Inspiratory crackles 50-60% Clubbing 25%-50%

DIP histology

Increased number of pigmented macrophages in alveolar spaces

Thickened alveolar septae Overall architecture maintained with very

little honeycombing

DIP Lung functions

Restrictive pattern Decreased diffusion capacity Less pronounced than with idiopathic

pulmonary fibrosis

Radiological appearance of DIP

• Ground Glass appearance

• Correlate with macrophage accumulation in alveoli and alveolar ducts

• Chest X-ray may be normal in up to 22% of biopsy proven cases

Treatment and Prognosis

DIP There has been a +/- 30% mortality rate

reported in these patients ? Smoking cessation Some improvement on Steroid therapy but

data is unclear Measurement of improvement ? Sustained improvement Correlate to smoking cessation

Role of other immuno-therapy

Respiratory bonchiolitis

Respiratory bronchiolitis, First described as an incidental post mortem finding in smokers by Niewoehner

Extremely common in Smokers Usually without symptoms of

accompanying lung disease May account for 20% of subclinical

radiographic changes in smokers Small portion of these patients may have

accompanying interstitial lung disease!

Respiratory bonchiolitis-associated interstitial lung disease (RB-ILD)

Characterised by Pigmented macrophages Mild interstitial changes in interstitium

surrounding respiratory bronchioles and alveoli Virtually all cases are smoking related Histologically less extensive and diffuse

compared to DIP

RB-ILDClinical picture

Symptoms usually start in the 4th-5th decade

Slightly more males than females involved

Cough and dyspnea

50% have crackles and very few have clubbing

RB-ILDLung Functions

Normal or mixed obstructive restrictive pattern

DLCO2 impaires Total lung volumes may be

Normal Increased Decreased

RB-ILDRadiologyCXR• Reticular or reticulo

nodular opacities in +/- 60%

• Ground glass

HRCT• Areas of ground

glass attenuation• Fine nodules• Associated

emphysematous changes

Treatment and Prognosis

Good Prognosis compared to DIP No deaths have been attributed to RB-ILD No Progression to Lung fibrosis

Treatment Stop smoking ? Role of corticosteroids

Pulmonary Langerhans’ cell histiocytosis (PLCH)

Langerhans Cell histiocytosis Non malignant disorder Abnormal proliferation of dendritic cells May affect multiple organs or involve only single

organ system Spectrum of severity (Children usually more

severely affected: Letterer Siwe disease, Hand schuller christian syndrome)

Lung involvement Usually in isolation Associated with Smoking

PLCHHistology

Nodular sclerosing lesions containing Langerhans’ cells

Bronchiolocentric distribution

Progression Fibrosis and cystic changes

PLCHClinical features

Adults in 3rd and 4th decade Sex distribution is uniform > 90% of patients are smokers or ex-

smokers Dyspnea (35-87%) Cough (50-70%) Previous Pneumothorax (10%) Crackles and clubbing very rare Wheezes may be present Cystic bone lesions (Skull, pelvis,ribs)

10%

PLCHLung functions

Obstructive and restrictive changes may be seen

Difficult to distinguish effects of cigarette smoking

Abnormal diffusion capacity Abnormal exercise tolerance

Radiology of PLCHCXR• Nodular or

reticular nodular

• Middle and upper lung zones

• Cystic changes increased lung volumes

Radiology of PLCHHRCT• Upper lung

involvement• Sparing of lung

bases• Complex cysts• Nodules and

cysts

PLCHDiagnosis

History and CT findings may be diagnostic

Surgical biopsy Broncho alveolar lavage

> 5% of CD1a positive cells

Treatment and Prognosis

Natural history is uncertain Treatment

? Smoking cessation ? Corticosteroids Other Cytotoxic drugs

Ryu JH, Colby TV, Hartman TE, Vassallo R, Smoking related interstitial lung diseases: A concise review, Eur Resp J 2001; 17:122-132

Lung diseases related to smoking

Lung Cancer Chronic Obstructive airways disease Interstitial lung diseases

Conclusion

References

1. Ryu JH, Myers JL, Capizzi SA et al, Desquamative Interstitial Pneumonia and Respiratory Bronchiolitis-Associated Interstitial Lung Disease, Chest 2005;127;178-184

2. Ryu JH, Colby TV, Hartman TE, Vassallo R, Smoking related interstitial lung diseases: A concise review, Eur Resp J 2001; 17:122-132

3. Hidalgo A, Franquet T, Gimenez A et al, Smoking-related interstitial lung diseases: radiologic-pathologic correlation, Eur Radiol 2006; 16: 2463–2470

4. Atilli AK, Kazarooni EA, Gross BH et al, Smoking-related Interstitial Lung Disease: Radiologic- Clinical-Pathologic Correlation, RadioGraphics 2008; 28: 1383–1398

5. Caminati A, Harari S, Smoking-related Interstitial Pneumonias and Pulmonary Langerhans Cell Histiocytosis, Proc Am Thorac 2006; 3: 299–306

6. Selman M, The Spectrum of Smoking-Related Interstitial Lung Disorders: The Never-Ending Story of Smoke and Disease, Chest 2003; 124: 1185-1187