Interstitial lung Dz supplement

Question 1

Treatment for interstitial lung diseases (Idiopathic)

Answer 1

• As a group, unless the inciting factor is identified, treatments are limited
• uncommon disease

Question 2

Diagnosis of ILD

Answer 2

-have usual interstitial pneumonia pattern on biopsy and also on CT so can avoid biopsy now

Question 3

Other diseases with similar biopsy to IPF

Answer 3

• scleroderma
• Rheumatoid arthritis
• look at presentation, Hx and PE for accurate Dx!

Question 4

UIP vs. IPF

Answer 4

• IPF is a specific disease of unknown cause

- UIP is a pattern found in many diseases, including IPF–they are not the same thing!!

Question 5

RB-ILD vs. NSIP

Answer 5

• RB-ILD only found in smokers!!

- NSIP found in pts with CONNECTIVE TISSUE disease

Question 6

Three ILDs only found in smokers or former smokers

Answer 6

• Desquamative interstitial pneumonia (DIP)
• Langerhan’s cell histiocytosis
• RB-ILD
• *Pulmonary alveolar proteinosis (PAP) is a very rare smoking related disease also
• if patients stop smoking, usually see improvement

Question 7

Connective Tissue Diseases (CTD) frequently involve the lung and show what kind of lung disease?

Answer 7

• Nonspecific interstitial pneumonia (NSIP)

- NSIP=biopsy specimen that shows features that don’t fit other ILD biopsies

Question 8

Rheumatoid arthritis

Answer 8

• inflammation of joint linings
• most common pulmonary feature=pleurisy, pleural effusion or both!
• Pulmonary nodule or multiple pulmonary nodules may develop as may ILD
• may develop bronchiolitis or bronchiectasis
• Pleural effusion has lowest glucose levels in fluid!!!!

Question 9

Systemic Lupus Erythematosus (SLE)

Answer 9

• systemic autoimmune disease that attacks serial surfaces like joints, pleura, and any organ system
• lung path follows activity of SLE (related to SLE)
• if quiescent SLE then think pulmonary symptoms are from different cause
• Pleural disease most common but with long standing SLE, ILD can form (UIP) and can result in pulmonary fibrosis

Question 10

Progressive systemic sclerosis (PSS)–aka scleroderma

Answer 10

• Of all CTDs, PULM FIBROSIS MOST COMMON AND SEVERE in this!!
• Biopsy shows NSIP!
• Since PSS involves arteries, it can involve pulmonary arteries that leads to pulm HTN!

Question 11

Other diseases and exposures associated with NSIP

Answer 11

1) Drugs: amiodarone, methotrexate, flecainide
2) HIV
3) Hypersensitivity pneumonitis

Question 12

Treatment for ILD

Answer 12

• For everything other than IPF, use CORTICOSTEROIDS, but limited data on effectiveness!
• when duration or dosages are high, lots of complications
• 1-2 mg/kg/day for at least 2 months
• Long term oral corticosteroids results in severe side effects

Question 13

Treatment for IPF

Answer 13

• expensive, lots of side effects and no improvement in survival or quality of life but do slow decline in PFTs somewhat
• Lung transplants not much better with about 50% 5 yr survival rate

Question 14

Smoking associated lung diseases

Answer 14

• COPD
• Lung cancer
• Respiratory Briochiolitis–radiographic diagnosis but if they have symptoms, it becomes RB-Interstitial Lung disease (RB-ILD)
• Desquamative Interstitial pneumonia or DIP
• Langerhans cell histiocytosis (formerly Langerhans cell granulomatosis, or histiocytosis X)
• IPF much more common in smokers but can happen in nonsmokers