Rheumatologic Pleuropulmonary Dz Flashcards
Systemic Sclerosis
2 forms : ILD and VD
- ILD - NSIP pattern and IS fibrosis ( more common)
- VD - concentric arterial thinning
What cytokines are seen w/ scleroderma lung?
- IL8 and TNF ( early) - PMN attractant, latter does endothelium binding as well
- MIF1alpha- PMN chemotaxis
- RANTES - T cell recruitment/ activation
- Endothelin 1 - vasoconstriction
- TGF beta (late) - fibroplasia
Where in the lung doe sclederma occur
- bases, posterior, periphery
what do you see in CT for scleroderma
ground glass then reticular infiltrates w/ late dz
histo for scleroderma
- NSIP pattern w/ lymphocytes, macs, then fibroblasts and collagen
- temporal homogenegity - all areas are at same stage
- trichrome stain - excess collagen is blue
- VD - concentric thickening and fibrosis of small pulmonary arteries, can occur w/out ILD (crest syndrome: anti-centromere Ab)
Symptoms for scleroderma
dyspnea (due to increased work of breathing, stiff lungs) and dry cough
signs for scleroderma
dry inspiratory velcro crackles at bases - sudden opening of small airways and abnormally closes by pressure of IS inflammation, edema, and fibrosis
Dx for scleroderma
skin, esophageal, renal manifestation. Anti-Scl70, restrictive pattern on PFT, decreased DLCO
Rx for scleroderma lung
Cyclophosphamides, NOT Steroids
Lupus pleuritis
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
Acute Lupus Pneumonitis
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
Rx for lupus pneumonitis
steroid and immunosuppression
Rx for NSIP lupus
steroids
Lupus Pulmonary VD
uncommon, PTN on echo in some pts, concentric arterial thickening
* Thromboembolic Dz - anti-phospholipid syndrome
Lupus - shrinking lung syndrome
dyspnea, small lungs of Xr, decreased DLCO, restrictive PFT, self limited
RA lung problems
- pleuritis +/- pleural effusion
- IS pneumonia - UIP or NSIP pattern - many lymphocytes and germinal centers - bronchioles and pleura
- Follicular bronchiolitis - STEROIDS, peribronchial or centrilobular nodules and ground glass
- Obliterative broncholitis - OBSTRUCTIVE patter, superimposed on Sjorgens
- Organizing pneumonia - acute onset of fever, alveolar infiltrates = STEROIDS
- RA nodule in lung
What happens w/ IS pneumonia in RA
- exertional dyspnea, dry insipiratory crackles, decreased DLCO, IF on Ct
- temporally heterogeneous - early areas of only inflammation, late areas of only fibrosis, mix of the two present. Honeycomb lung
- severe in periphery and lower lobes (basal)
What does a RA nodule look like on histo
palisading histocytes, basophilic debris, surrounding fibrosis, lymphocytes, macs, and giant cells
What is the problem w/ giving RA pts MTX?
adverse effect is pneumonitis - causes DAD/HSR – chronic IS pneumonia w/ microscopic features
- early - patchy lymphyocytic IS inflammation
- late - bad, fibrosis
- 20% mortality, give steroids,
- dyspnea, cough, fever,
- bilateral pulmonary crackles.
CT of pt w/ MTX pneumonitis
bilateral, ground glass and reticular opacification, worse posteriorly. may be linked to pulmonary fibrosis
What other drugs can cause pulmonary toxicity
- gold - DAD or chronic ISP
- penicilliamine - alveolar hemorrhage
- Anti TNF agents - ISP
Polymyositis/Dermatomyositis
chronic, usually NSIP, ground glass/reticular, restrictive pattern, decreased DLCO
- give steroids and immunosuppression
- ILD and ogranizing pneumonia
- lung dz in 70% of pts - most common cause of death just like in scleroderma lung
- consolidation on CT scan, aspiration pneumonia may occur
- Associated w/ MALIGNANCY
Sjogren Syndrome
- dyspnea, dry cough due to loss of submucosal gland secretion, COPD due to bronchial hyperresponsiveness
CT - lower lobe ground glass/reticular
NSIP cellular
NSIP sjogren syndrome
- lymphoid follicles
- bronchiolocentric
- more lymphyoctyes
- macs and giant cells
- abundant proliferating lymphocytes can become semi-autonomous – LYMPHOMA
CT of sjogren syndrome
mediastinal LAD, pleural effusion, prominent nodularity of lung lesions, lung cysts
Histo of sjogren syndrome
expansile lymphocytic infiltration, tracking in a lymphangitic pattern, no follicles, macs, giant cells
these pts w/ steroid and immunosuppression get what type of pulmonary infxns?
acute and nodular
Rheum lung dz presents as what
chronic and interstitial
What other diseases present as chronic interstitial pneumonia
pneumocytis, saroidosis, and toxo
