Flashcards in Rheumatological Lung Disease Prep Ppt (Nichols) Deck (53)
What are the 2 forms of systemic sclerosis?
interstitial lung dz and vascular dz
Describe the sclerodermal lung
1. interstitial dz starting as nonspecific intersitial pneumonia PATTERN --> interstitial fibrosis
2. concentric arterial thickening (fibrosis)
What are the mediators of sclerodermal lung pathology?
IL-8, TNF, MIF1-alpha, RANTES, endothelin-1, TGF-beta
T cell recuiter and activator assc with sclerodermal lung
vasoconstrictor and assc with sclerodermal lung
what does MIF1-alpha do?
promotes neutrophil chemotaxis
What part of lung is most affected by sclerodema and how do the affect areas appear on CT
favors bases (posterior and peripherally)
ground glass then reticuar infiltrate
Cells that mediate damage in scleroderma lung
lymphocytes and macrophages --> fibroblasts
Apparently scleroderma lung looks very similar to UIP, so how is it distinguished?
in scleroderma all areas of the lung are in the same phase (i.e. temporal homogeneity)
concentric arterial thickening in sclerodermal lung can cause...
PAH and cor pulmonale
What is CREST syndrome?
limited systemic sclerosis WITHOUT interstitial lung dz
*is anti-centromere Ab +
Signs and symptoms of systemic sclerosis lung dz
symp: dyspnea and dry cough
signs: velcro crackles (esp at bases)
How is systemic clerodermal lung dz diagnosed?
anti-Scl70 (DNA topo) Ab +
dyspnea, dry cough, velcro crackles
sing, esophagus, and renal amifestations of SS
What organs (other than lungs) are involved in systemic sclerosis?
skin, esophagus, kidneys
How does SS lung dz affect PFTs and DLCO?
dec compliance = dec volumes
What is the treatment for sclerodermal lung dz? How is this different that non-specific intersitial pneumonia?
cyclophosphamide and NO serterids
(remember, NSIP responded to steriods)
**steroids may cause renal problems
prognosis of SS? What usually kills them?
12 yrs after Dx
60% die from scleroderma lung dz
Lupus sucks, so there are 5 ~lung dz that can be going on at once in these poor people, what are they?
2. acute lupus pneumonitis
3. NSIP (NOT pattern, actual dz)
4. pulm vascular dz (concentric arterial thickening and thromboemboli)
5. shrinking lung syndrome
Most common pulm manifestation of lupus?
Lupus pleuritis is typically ____ and may or may not be assc with ____
serosanguinous exudative pleural effusions (usually small and bilateral)
What can be found in the plural fluid of pts with lupus pleuritis
lymphocytes and anti-ANA Abs
3 characteristics of acute lupus pneumonitis
2. form of ARDS/ALI (diffuse alveolar damage)
3. +/- diffuse hemorrhage
Acute lupus pneumonitis symptoms and signs:
How does this appear on CT?
symp: dyspnea, cough, fever
signs: fever, wet pulm crackles
CT: diffuse ground glass
How is NSIP with lupus different than NSIP in SS?
in lupus you treat with steriods bc it is the actualy dz not just the pattern
What are the 2 types of pulm vascualr dz seen in lupus pts?
1. concentric arterial thickening
2. pulm thromboembolism (assc with lupus anticoag/anti-phospholipid Abs)
Pulmonary artery webs,strands of fibrous tissue, spanning the lumen
old (organized) pulmonary emboli
what causes shrinking lung syndrome in lupus?
What is the criteria for shrinking lung syndrome
2. small liungs on CXR
3. dec DLCO
4. restrictive PFTs (low vol)
lupus lung dz that is usually self-limited
shrinking lung syndrome
pleuropulm dz in pts with RA is more common in ...
men and smokers
What are the types of pleuropulm dz in RA
1. pleuritis +/- fibrinous pleural effusion
2. fibrosing interstitial lung disease (UIP or NSIP)
3. follicular bronchiolitis
4. obliterative bronchiolitis
5. organizing pneumonia
6. acute lung injury
7. pulmonary hemorrhage
8. rheumatoid nodules
What microscopic findings are assc with RA related dz?
abundant lymphocytes and germinal centers
Signs and symptoms of interstitial lung dz in RA
How is ILD in RA different than SS and lupus?
RA is UIP pattern and is temporally heterogenous (SS and lupus is NSIP and SS is temp homo)
-areas of inflam only (early)
-areas of fibrosis (late)
What part of the lung does ILD in RA affect?
UIP is more SEVER in periph and lower lobes
microcysts containing mucus =
honeycomb lung in RA
*lower lobe and periph inc severity
RA + obstructive pattern PPTs (inc lung vol)
RA + acute onset of fever and alveolar infiltrates
rim of palisading histiocytes, basophilic debris and surrounding fibrosis, lymphocytes, macrophages and multinucleated giant cells surroundign large area of necrosis
What is used to treat RA that has an adverse effect of causing pneumonitis
pathophys of MTX mediated pneumonitis
cytotoxicity causes DAD or hypersensitivity
--> chronic interstitial pneumonia similar to UIP
How does MTX pulm toxicity present?
-subacute (w/in 4 weeks of starting therapy)
-dyspnea, cough, fever
-bilateral pulm crackles
How do you treat MTX pulm toxicity?
There are other therapies for RA that also cause pulm toxicity. What are these drugs and what pulm toxicity do they cause?
1. gold: ALI or chronic interstitial pneumonia
2. Penicillamine: alveolar hemorrhage
3. anti-TNF-alpha: interstitial pneumonia
lung disease that is ~ specific to it
1. ILD + organizing pneumonia
2. consolidation on CT
3. resp failure from muscle weakness
4. aspiration pneumonia from muscle weakness
5. part of anti-synthetase syndrome (myositis + arthritis + lung dz)
myositis is assc with
histopathology seen with sjogren's
1. lymphoid follicles (germinal centers)
2. is bronchiolocentric
3. MORE LYMPHOCYTES
4. macrophages and multinucleated giant cells
specific features of lung dz in sjogren's pts
1. involves airways
2. get xerotrachea and xerobronchea
3. loss of submucosal gland secretion --> relentless dry cough
4. bronchial hyper-responsivemess ~COPD
clues that a sjogren;s pt has lymphoma
1. CT scan (nodularity and cysts)
2. lung biopsy (no follicles, macrophages, or multinucleated giant cells)
3. mediastinal LAD
4. pleural effusion
How is pulm infection in rheumatology pts different than rheumatological lung dz (general concepts)
rheum lung dz is rarely acute and rarely nodular
Acute lung dz in rheum pt is more likely ...