ILD

Question 1

AEP

Answer 1

HP reaction to inhaled agent
Can happen with tobacco smoke
More common in young and men
Pleural effusions in up to 70%
CXR can be reticular or GGO
>25% eos in BAL

Question 2

Sjorgen

Answer 2

Associated with LIP
Women 50-70
ILD is most common pulmonary manifestation - NSIP
ILD more advanced in secondary SS

Question 3

LIP

Answer 3

Invasion of alveolar and interstitial spaces by lymphocytes, plasma cells
Benign polyclonal disorder of mature B and T cells
5% progress to lymphoma - most commonly well-differentiated, slow growing MALT
GGOs, centrilobular nodules, cysts

Question 4

Hard Metal Lung Disease

Answer 4

Giant cell interstitial pneumonitis
Hard metal - powdered tungsten carbide and cobalt
Tools for cutting stone
Cannabilistic giant cells

Question 5

SLE

Answer 5

• ILD relatively uncommon compared to others
• NSIP, UIP, OP
• Anti-Smith - highly specific for SLE
• Anti SS-A (Ro) and Anti-SS-B (La) less specific (more often seen in Sjogrens)
• 15-20% positive for p-ANCA

Question 6

Microscopic Polyangiitis

Answer 6

• c-ANCA positive

Question 7

Eosinophilic Granulomatosis with Polyangiitis

Answer 7

• Perinuclear
• directed against MPO, cathepsin G, lactoferrin
• asthma or allergic rhinitis
• started leukotriene receptor inhibitor can unmask
• eosinophilia
• 15-20% of patients with SLE with have p-ANCA
• Rheum diagnostic criteria - need 4
  
  • asthma
  • eosinophilia > 10%
  • mononeuropathy or poly
  • nonfixed opacities
  • paranasal sinus abnormalities
  • extra vascular eos on biopsy
• vasculitis of small and medium vessels
• lung and skin most common
• treatment with steroids

Question 8

Familial Pulmonary Fibrosis

Answer 8

• Autosomal dominant
• mutations
  
  • TERT and TERC
  • SFTPC
  • MUC5B
  • RTEL1
  • TOLLIP

Question 9

Birt-Hogg-Dube

Answer 9

• mutation in FLCN - encoded folloculin
• cutaneous lesions
• pulmonary cysts
• renal tumors
• 12% get pneumos
• cysts variable sized, irregular, basilar or mid lung

Question 10

IPF

Answer 10

• Panther trial - increased death and hospitalization with prednisone, azathioprine and NAC
• BAL not needed

Question 11

PLCH

Answer 11

• cysts and nodules
• centrilobular nodules
• staining with CD1a
• mutations in BRAF gene
• spontaneous pneumo in 15%
• cysts vary in size

Question 12

LAM

Answer 12

• uniformly sized cysts
• mutation in TSC2 gene
• stain with HMB45
• smooth muscle proliferation
• abnormal VEGF-D

Question 13

PAP

Answer 13

• most commonly due to acquired antibody to granulocyte-monocytes colony stimulating factor
• can also happen in infection (PJP, nocardia)
• impaired clearance of protein fluid
• first line treatment is lavage
• can treat with GM-CSF also
• median age 39
• diagnosed with PAS stain
• anti-GM-CSF abs in BAL or serum highly specific

Question 14

Cardiac Sarcoidosis

Answer 14

• if asymptomatic and normal ECG, follow up in 1 year
• often affects conduction pathway
• abnormal echo found in 25-75% of sarcoidosis patients
• diagnosed with PET or cardiac MRI with gad

Question 15

DIP

Answer 15

• pigmented macrophages in alveoli
• diffuse ground glass
• get parenchyma cysts also

Question 17

Sarcoidosis

Answer 17

• recurs after lung transplant (50%)
• often not clinically significant
• causes hypercalcemia by activating 25-hydroxyvitamin D
  
  • occurs in 6-8% of cases

Question 18

Amit-Synthetase

Answer 18

• most common ab is anti-Jo-1
• other abs
  
  • anti-PL-7
  • anti-PL-12 (associated with ILD in absence of myositis
  • OJ, EJ, KS, ZO, YRS