Flashcards in Rheumatologic pleuropulmonary disease Deck (91)
What are the 5 rheumatologic diseases that most commonly have pleuropulmonary disease?
systemic sclerosis, systemic lupus erythematosus,
rheumatoid arthritis, dermatomyositis/polymyositis
and Sjogren syndrome
____ has more lung disease than any other autoimmune connective tissue disease.
What are the two forms of systemic sclerosis?
interstitial lung disease, vascular disease
About ____%of systemic sclerosis patients
get interstitial lung disease.
About ___% of systemic sclerosis patients
get pulmonary hypertension
What 2 things comprise the interstitial lung disease that people with systemic sclerosis get?
NSIP pattern and interstitial fibrosis
What are the systemic sclerosis mediators?
IL-8: [early] (neutrophil attractant and activator)
TNF: [early] (neutrophil attractant and promoter of neutrophil-endothelial binding)
MIF1alpha: (promoter of neutrophil chemotaxis)
RANTES: T cell recruiter and activator
Endothelin 1: vasoconstrictor
TGF-B: late, promoter of fibroplasia
What part of the lung is favored by scleroderma lung?
bases, posterior, periphery
What does CT show for scleroderma lung (early and late)
Early: ground glass, late: reticular infiltrates
____ is the most common pattern of early scleroderma lung disease.
Describe NSIP pattern.
lymphocytes and macrophage infiltrating interstitium followed by fibroblasts + collagen creating interstitial fibrosis. Lesions are homogenous.
Can scleroderma ever present with UIP pattern?
What are some differences between UIP pattern and NSIP pattern?
NSIP will be homogenous wherease UIP is not. NSIP is associated more with ground glass and UIP is more honeycomb pattern, UIP doesnt seem to respond to therapy compared to NSIP pattern and has worse prognosis and may be due to aspiration.
What is seen histologically with systemic sclerosis vascular disease?
vascular disease: concentric
thickening and fibrosis of small pulmonary arteries
What is it called when vascular disease occurs without scleroderma lung?
associated with anti-centromere antibodies and limited systemic sclerosis, CREST syndrome
What are the 2 most common symptoms of systemic sclerosis lung disease? Why do they occur
Dyspnea, dry cough
- Dyspnea is due to increased work of breathing sensed by respiratory muscle mechanoreceptors
What are signs for systemic sclerosis lung disease?
Dry inspiratory velcro crackles especially at the bases initially subtle fine thought to represent the sudden opening of small airways abnormally closed by the pressure of interstitial inflammation, edema and fibrosis around them
How do you make diagnosis of systemic sclerosis?
Association of symptoms, signs and radiology
of interstitial lung disease with skin, esophageal and renal manifestations of scleroderma and/or
anti-Scl70 (anti-DNA topoisomerase) antibodies (40%). Also decreased DLCO and abnormal PFTs
How do you treat systemic sclerosis?
Cyclophosphamide. Not steroids- could bause scleroderma renal crisis
What is the prognosis for systemic sclerosis?
Mean survival is 12 years. Death rate higher for females.
What is the most common cause of death in systemic sclerosis?
Scleroderma lung disease 60%
What pleuropulmonary diseases are seen with lupus?
pleuritis, acute lupus pneumonitis, NSIP, pulmonary vascular disease (concentric arterial thickening, pulmonary thromboembolism), shrinking lung syndrome
What is the most common pleuropulmonary manifestation of lupus?
pleuritis- present in 20% at onset, at least 50% over time and up to 100% at autopsy
What symptoms are present with pleuritis in lupus?
often asymptomatic but sometimes with pleuritic chest pain
Describe the pathology of lupus pleuritis
typically fibrinous. may be associated with serosanguinous exudative pleural effustion. May have ANA in pleural fluid. There may be few inflammatory cells in the exudate. Pleural effusion usually small and bilateral.
T or F. Pleuropulmonary disease of lupus is broader spectrum and more commonly acute than any other autoimmune connective tissue disease.
How common is acute lupus pneumonitis in lupus?
Describe the pathology of acute lupus pneumonitis.
Form of acute lung injury (DAD), can take form of diffuse hemorrhage, alveolitis with loose fibrin exudate and lymphocytes and macrophages in airspaces and a few lymphocytes in interstitium. Goes on to interstitial pneumonia and fibrosis.
What is later pathology of acute lupus pneumonitis?
goes on to interstitial pneumonia and fibrosis
Symptoms of acute lupus pneumonitis
dyspnea, fever, cough
Signs of acute lupus pneumonitis
fever, wet pulmonary crackles
CT of acute lupus pneumonitis
diffuse ground-glass opacification with pleural effusions 50% of the time
How do you treat acute lupus pneumonitis?
steroids and immunosuppresion
What is the prognosis of acute lupus pneumonitis?
How common is NSIP with lupus?
present 2% of lupus pt at onset, less than 5% overtime
How do you treat NSIP with lupus?
How common is pulmonary vascular disease with lupus?
uncommon, but 10% of lupus patients have subclinical evidence of pulmonary hypertension on echocardiography
What are the two types of pulmonary vascular disease with lupus?
concentric arterial thickening or thromboembolic disease
_____ associated with lupus anticoagulant or anti-phospholipid antibodies
Thromboembolic disease with lupus
What forms of lupus pleuropulmonary disease can cause hemoptysis?
1. pulmonary infarction due to PE
2. hemorrhagic acute lupus pneumonitis
Describe chronic pulmonary thromboembolic disease (with lupus)?
pulmonary artery webs, strands of fibrous tissue spanning the lumen, old pulmonary emboli that are organized. Recanalizing occurs.
Describe shrinking lung syndrome with lupus.
rare due to diaphragmatic weakness. Syndrome includes dyspnea, small lungs on x-ray, decreased DLCO, restrictive pattern PFT abnormalities. Usually self-limited
What types of pleuropulmonary disease are associated with RA?
1. pleuritis +/- pleural effusion
2. fibrosing interstitial lung disease
3. follicular bronchiolitis
4. obliterative bronchiolitis
5. organizing pneumonia
6. acute lung injury
7. pulmonary hemorrhage
8. rheumatoid nodules
Who is pleuropulmonary disease with RA more common in?
Smokers, 3x more common in smokers
How common is pleuropulmonary disease with RA?
14% have significant disease, 44% have subclinical disease
How common is pleuritis with fibrinous pleural effusion in RA?
20% of RA patients have pleuritic pain, less than 5% have an effusion, but 50% have pleural disease at autopsy
Describe interstitial pneumonia in pt with RA.
Has UIP or NSIP pattern in significant and likely similar proportions.
What are findings suggestive of RA-related interstitial disease?
abundant lymphocytes and germinal centers
You see a histo slide with germinal centers. What should you be thinking about?
RA-related interstitial disease
Pleuropulmonary disease in RA commonly involves what part of the lungs?
More commonly pleura but also bronchioles
How do RA pt with interstitial lung disease present on exam?
exertional dyspnea, inspiratory velco crackles, decreased DLCO, interstitial fibrosis on CT
Describe the UIP pattern of fibrosing interstitial lung disease.
Temporally heterogenous with early areas of only inflammation, late areas of only fibrosis, and everything inbetween. Most severe in periphery, lower lobes. Fibrotic lung remodeling --> honeycomb lung
What is the end stage of all fibrosing interstitial lung disease?
Histologically, what can you expect to see with later stage interstitial pneumonia in RA?
Mucinous microcysts (honeycomb change) and lymphoid follicles (germinal centers)
Describe follicular bronchiolitis.
It is more common with RA. CT shows peri-bronchial or centrilobular nodules and ground glass opacities with broncho-centric distribution.
How do you treat follicular bronchilits in RA?
describe obliterative bonchilitis
More common in RA, obstructive patter PFTs, sometimes superimposed Sjogren's, does NOT respond to steroids.
Describe organizing pneumonia.
More common in RA, present with acute onset, fever, and alveolar infiltrates, responds to Steroids
Histological appearance of rheumatic nodules
rim of palisading histiocytes, basiphilic debris and surrounding fibrosis, lymphocytes, macrophages, and multinucleated giant cells
How common is pulmonary fibrosis in the death of pt with RA?
7/8 who died of resp failure had fibrosis!
Pneumontitis is a recognized adverse effect of methotrexate due to :
cytotoxicity causing diffuse alveolar damage or hypersensitivity causing chronic interstitial pneumonia with microscopic features similar to
usual interstitial pneumonia.
What does the histology of methotrexate pulmonary tox look like early on vs later?
early it is too bad with patchy lymphocytic interstitial infiltration. Late is bad and fibrotic
How severe is methotrexate pulmonary toxicity?
Life-threatening: up to 20% mortality
How should you treat methotrexate pulmonary toxicity?
How does methotrexate pulmonary toxicity present?
most often subacute, in first 4 months. Presents with dyspnea, cough, fever, have bilateral pulmonary crackles
How does methotrexate pulmonary toxicity appear on CT?
can be bilateral, partly ground glass, partly reticular or can be coarser, denser, more reticular and worst posterior.
What are pulmonary toxicities for RA therapies besides MTX?
Gold: acute lung injury or chronic interstitial pneumonia.
Penicillamine: alveolar hemorrhage
Anti-tumor necrosis factor agents can cause interstitial pneumonia
Ways that polymyositis/dermatomyositis-associated lung disease is like other connective tissue diseases?
Usually chronic, Usually NSIP, Usually have dyspnea, Usually have ground-glass or reticular infiltrates, Usually have restrictive pattern PFT abnormalities and decreased DLCO, Can present with acute lung injury like lupus or rheumatoid lung disease, Treatment = steroids +/- immunosuppression
How is polymyositis/dermatomyositis-associated lung disease treated?
steroids and immunosuppresion
How common is polymyositis/dermatomyositis-associated lung disease?
Occurs in 70% of pt and is most common cause of death (like scleroderma)
How common is ILD and organizing pneumonia in polymyositis/dermatomyositis-associated lung disease?
50% of the 70% of pt who have lung disease
How does polymyositis/dermatomyositis-associated lung disease appear on CT in 50% of cases?
T or F. Respiratory failure from muscle weakness is COD in 25% of pt with polymyositis/dermatomyositis-associated lung disease.
Muscle weakness can lead to what complications in polymyositis/dermatomyositis-associated lung disease?
Death, aspiration pneumonia
What is anti-synthetase syndrome and how common is it?
myositis+ arthritis + lung disease (20%)
T or F myositis is associated with MALIGNANCY.
How is Sjogren syndrome-associated lung disease
like other connective tissue diseases?
1. Most common symptom is dyspnea
(10% of Sjogren’s patients have it)
2. Most common CT scan finding is lower lobe
ground-glass (or reticular) opacities
3. Most common histopathology is
nonspecific interstitial pneumonia (NSIP)
4. Not much else
(a lot more differences than similarities)
How common is Sjogren syndrome-associated lung disease?
11% of pt
What part of the lung is involved in Sjogren syndrome-associated lung disease?
airways, especially submucosal glands
T of F xerotrachea and xerobronchea are associated with Sjogren syndrome-associated lung disease.
How does someone with Sjogren syndrome-associated lung disease get a dry relentless cough?
loss of submucosal gland secretion
When does Sjogren syndrome-associated lung disease resemble COPD?
What does histological Sjogren syndrome-associated lung disease look like?
it has germinal centers and is bronchiolocentric, has more lymphocytes, has macrophages and multinucleated cells
What is the relationship of lymphoma and Sjogren syndrome-associated lung disease?
The proliferating lymphocytes in Sjogren syndrome-associated lung disease can become semi or fully autonomous causing pseudolymphoma or lymphoma and can commonly be called lymphoproliferative disorders. 44x more common in these pt.
______ are all suggestive of lymphoma in Sjogren syndrome-associated lung disease.
CT: Mediastinal lymphadenopathy, pleural effusion, prominent nodularity of the lung lesions and lung cysts
Histo: expansile or tumefactive pattern of lymphocytic infiltration, tracking in a lymphangitic pattern, absence of follicles, macrophages and multinucleated giant cells
T or F Rheumatologic disease is commonly acute.
FALSE- it is not. Infection could be acute
T or F Rheumatologic disease is commonly nodular.
FALSE- it is not! Think fungal infection
What are intersitial infections that cause chronic pneumonias that could be confused with rheumatologic lung disease?
pneumocystic, sarcoidosis, t. gondii
What are nodular chronic pneumonias that could be confused with rheumatologic lung disease?
TB, histo, aspergillosis, cryptococcosis, coccidio, blasto
T or F Subacute or chronic dyspnea in a rheum pt is more likley rheum than infection.