Rheumatologic Pleuropulmonary Dz

Question 1

Systemic Sclerosis

Answer 1

2 forms : ILD and VD

1. ILD - NSIP pattern and IS fibrosis ( more common)
2. VD - concentric arterial thinning

Question 2

What cytokines are seen w/ scleroderma lung?

Answer 2

1. IL8 and TNF ( early) - PMN attractant, latter does endothelium binding as well
2. MIF1alpha- PMN chemotaxis
3. RANTES - T cell recruitment/ activation
4. Endothelin 1 - vasoconstriction
5. TGF beta (late) - fibroplasia

Question 3

Where in the lung doe sclederma occur

Answer 3

1. bases, posterior, periphery

Question 4

what do you see in CT for scleroderma

Answer 4

ground glass then reticular infiltrates w/ late dz

Question 5

histo for scleroderma

Answer 5

1. NSIP pattern w/ lymphocytes, macs, then fibroblasts and collagen
2. temporal homogenegity - all areas are at same stage
3. trichrome stain - excess collagen is blue
4. VD - concentric thickening and fibrosis of small pulmonary arteries, can occur w/out ILD (crest syndrome: anti-centromere Ab)

Question 6

Symptoms for scleroderma

Answer 6

dyspnea (due to increased work of breathing, stiff lungs) and dry cough

Question 7

signs for scleroderma

Answer 7

dry inspiratory velcro crackles at bases - sudden opening of small airways and abnormally closes by pressure of IS inflammation, edema, and fibrosis

Question 8

Dx for scleroderma

Answer 8

skin, esophageal, renal manifestation. Anti-Scl70, restrictive pattern on PFT, decreased DLCO

Question 9

Rx for scleroderma lung

Answer 9

Cyclophosphamides, NOT Steroids

Question 10

Lupus pleuritis

Answer 10

most common lung manifestation, often asymptomatic, some have pleuritic chest pain, ANA.
Fibrinous w/ serosanguinous exudative pleural effusion (small and bilateral); few inflammatory cells in exudate

Question 11

Acute Lupus Pneumonitis

Answer 11

rare, form of ALI (DAD, can take form of diffuse hemorrhage, goes onto IS pneumonia

• dyspnea, fever, cough
• pulmonary crackles, fever
• alvelolitis w/ loose fibrin exudate, lymphocytes, and macs

Question 12

Rx for lupus pneumonitis

Answer 12

steroid and immunosuppression

Question 13

Rx for NSIP lupus

Answer 13

steroids

Question 14

Lupus Pulmonary VD

Answer 14

uncommon, PTN on echo in some pts, concentric arterial thickening
* Thromboembolic Dz - anti-phospholipid syndrome

Question 15

Lupus - shrinking lung syndrome

Answer 15

dyspnea, small lungs of Xr, decreased DLCO, restrictive PFT, self limited

Question 16

RA lung problems

Answer 16

1. pleuritis +/- pleural effusion
2. IS pneumonia - UIP or NSIP pattern - many lymphocytes and germinal centers - bronchioles and pleura
3. Follicular bronchiolitis - STEROIDS, peribronchial or centrilobular nodules and ground glass
4. Obliterative broncholitis - OBSTRUCTIVE patter, superimposed on Sjorgens
5. Organizing pneumonia - acute onset of fever, alveolar infiltrates = STEROIDS
6. RA nodule in lung

Question 17

What happens w/ IS pneumonia in RA

Answer 17

1. exertional dyspnea, dry insipiratory crackles, decreased DLCO, IF on Ct
2. temporally heterogeneous - early areas of only inflammation, late areas of only fibrosis, mix of the two present. Honeycomb lung
3. severe in periphery and lower lobes (basal)

Question 18

What does a RA nodule look like on histo

Answer 18

palisading histocytes, basophilic debris, surrounding fibrosis, lymphocytes, macs, and giant cells

Question 19

What is the problem w/ giving RA pts MTX?

Answer 19

adverse effect is pneumonitis - causes DAD/HSR – chronic IS pneumonia w/ microscopic features

1. early - patchy lymphyocytic IS inflammation
2. late - bad, fibrosis
3. 20% mortality, give steroids,
4. dyspnea, cough, fever,
5. bilateral pulmonary crackles.

Question 20

CT of pt w/ MTX pneumonitis

Answer 20

bilateral, ground glass and reticular opacification, worse posteriorly. may be linked to pulmonary fibrosis

Question 21

What other drugs can cause pulmonary toxicity

Answer 21

1. gold - DAD or chronic ISP
2. penicilliamine - alveolar hemorrhage
3. Anti TNF agents - ISP

Question 22

Polymyositis/Dermatomyositis

Answer 22

chronic, usually NSIP, ground glass/reticular, restrictive pattern, decreased DLCO

1. give steroids and immunosuppression
2. ILD and ogranizing pneumonia
3. lung dz in 70% of pts - most common cause of death just like in scleroderma lung
4. consolidation on CT scan, aspiration pneumonia may occur
5. Associated w/ MALIGNANCY

Question 23

Sjogren Syndrome

Answer 23

1. dyspnea, dry cough due to loss of submucosal gland secretion, COPD due to bronchial hyperresponsiveness
   CT - lower lobe ground glass/reticular
   NSIP cellular

Question 24

NSIP sjogren syndrome

Answer 24

1. lymphoid follicles
2. bronchiolocentric
3. more lymphyoctyes
4. macs and giant cells
5. abundant proliferating lymphocytes can become semi-autonomous – LYMPHOMA

Question 25

CT of sjogren syndrome

Answer 25

mediastinal LAD, pleural effusion, prominent nodularity of lung lesions, lung cysts

Question 26

Histo of sjogren syndrome

Answer 26

expansile lymphocytic infiltration, tracking in a lymphangitic pattern, no follicles, macs, giant cells

Question 27

these pts w/ steroid and immunosuppression get what type of pulmonary infxns?

Answer 27

acute and nodular

Question 28

Rheum lung dz presents as what

Answer 28

chronic and interstitial

Question 29

What other diseases present as chronic interstitial pneumonia

Answer 29

pneumocytis, saroidosis, and toxo