Rheumatologic Lung DZ Flashcards by Michael Castellarin

What are the five rheumatologic diseases that often present with pleuropulmonary symptoms

Systemic sclerosis, systemic lupus rheumatoid arthritis, dermatomyositis, Sjrogen Syndrome

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Which of the fiv efeatures the MOST lung dz symptoms

Systemic sclerosis (aka scleroderma)

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Lung dz in scleroderma takes two forms, they are:

Interstitial and Vascular

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Interstitial lung dz in scleroderma or “SCLERODERMA LUNG” most often presesnts as

non-specific interstitial pneumonia or interstitial fibrosis
Concentric arterial thickening

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About 67% of systemic sclerosis pts get

interstitial lung dz

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About 20% of systemic sclerosis pts suffer from

pulmonary hypertension

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The pathophysiological mediators in Scleroderma Lung are:

IL-8, TNF,

MIF1alpha

RANTES

Endothelin 1
TGF-Beta

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IL8 does

TNF alpha does

IL8= neutrophil attractant and activator
TNFalpha-= neutrophil attractant and promoter of neutrophil endothelial binding

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MIF1 alpha does

promoter of neutrophil chemotaxis

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RANTES

T cell recruiter and activator

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Endothelin 1

Vasoconstrictor

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TGF-beta

promoter of fibroplasia

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Scleroderma lung favors which part of the lung?

Base, posterior, periphery.

You will first see Ground glass appearance on the CT then will become more reticular

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Early histology of scleroderma lung shows

Non-specific interstitial pneumonia with lymphocytes and macrophages followed by fibroblasts and collagen

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Trichrome stain stains collagen what color?

blue

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The vascular disease component of scleroderma lung causes

pulmonary hypertension and cor pulmonaale

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What is CREST syndrome

When the the vascular component of scleroderma appears without the interstitial lung disease.
It is associated with anti-centromere antibodies and limited systemic sclerosis

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What are the symptoms of SS lung dz

Dyspnea and dry cough due to increased work load of breathing due to fibrosis

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Signs of SS Lung

Crackles due to the sudden opening of small airways abnormally closed by the pressure of interstitial inflammation

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SS Lung dz diagnosis

Associtation of symptoms , signs, and radiology of interstitial lung dz with skin, esophageal, and renal manifestations of scleroderma.

Anti-scl70 (DNA Topoisomerase) antibodies

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Treatment of systemic sclerosis

Cyclophosphamide

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Why don’t you treat SS lung dz with steroids?

Renal crisis has been reported

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Most common cause of death for scleroderma pts

Lung Disease

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Prognosis of scleroderma pts from initial survival?

12 years

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Pleuropulmonary disease of lupus generally has 5 manifestations. THey are:

- Pleuritis - Acute Lupus pneumonitis - Nonspecific interstitial pneumonia - Pulmonary Vascular dz - Shrinking lung syndrome

The most common pleuropulmonary manifestation of lupus by a long shot is:

Pleuritis (present in 20% at onset, 50% over time, and 100% at autopsy)

Lupus pleuritis is typically

Fibrinous

Is fibrinous pleuritis specific for lupus>

Nope.

Pleural effusions in lupus are typically

small and bilateral

Acute lupus pneumonitis

rare, actually a form of acute lung injury, can take form of diffuse hemorrhage

Acute lupus pneumonitis symptoms

dyspnea, fever, cough

Signs of acute lupus pneumonitis

fever, wet pulmonary crackles

CT:

Ground Glass Opacification

How do you diagnose acute lupus pneumonitis

SYmptoms, signs, CT findings, context (demographics + serology)

Treatment for acute lupus pneumonitis

steroids + immunosuppresion

Prognosis=

50% mortality

Nonspecific interstitial pneumonia is -----

RARE as shit

Pulmonary vascular disease with lupus presents as two types, they are:

Concentric arterial thickening or thromboembolic disease.

Thromboembolic disease is associated with?

Lupus anticoagulant or antiphospholipid antibodies

Shrinking Lung Syndrome. How common and what causes it?

Rare, caused by diaphragmatic weakness

SHrinking lung syndrome features?

dyspnea, small lungs on x-ray, decreased diffusing capacity, restrictive pattern abnormalities, Usually self-limited

What percentage of RA patients have pleuritic pain?

20%

Findings suggestive of RA-related disease regarding interstitial pneumonia are?

Abundant lymphocytes and germinal centers

Pleuropulmonary disease in patients with RA commonly involves what>

The bronchioles and even more commonly involves the pleura

Interstitial lung disease in RA patients is typically most severe where?

Periphery of the lung and the bases

Mucinous microcysts (honeycomb change) and Lymphoid Follicles suggest

Interstitial pneumonia in RA

Follicular bronchiolitis in RA appears as

Peribronchiolar or centrilobular nodules in bronchocentricdistribution

Follicular bronchiolitis in RA responds to

steroids

Obliterative bronchiolitis does not respond to steroids

True

Organizing pneumonia in RA

responds to steroids

What is a recognized adverse effect of methotrexate regarding the lungs?

Pneumonitis. It is due to cytotoxicity causing diffuse alveolar infiltrates or hypersensitivity causing chronic interstitial pneumonia.

What is the mortality rate in methotrexate toxicity?

20%....surprisingly high

Methotrexate toxicity responds to what>

steroid therapy

What is the presentation of methotrexate pulmonary toxicity

dyspnea, cough, fever

MEthotrexate toxicity is typically subacute in what time period

up to 4 mths

RA treatment using gold can cause

acute lung injury or chronic interstitial pneumonitis

RA treatment using penicillamine can cause

alveolar hemorrhage

Anti-tumor necrosis factor agents such as infliximab and etanercept may cause

interstitial pneumonia

NSIP pattern is similar to UIP pattern except that NSIP pattern is distinguished by temmporal homogeneity.....all the lesions are at the same phase

ok...this applies mostly to the NSIP pattern pneumonia seen in Scleroderma

Mass like lesion composed of tons of blue dots (lymphocytes) think...

Lymphoma

What is the most commonly complicated by lymphoma

Sjrogen's syndrome....KNOW

Loss of submucosal gland secretion causing an unrelentless dry cough is seen most commonly in ?

Sjrogrens syndrome

What makes Sjrogren's associated NSIP special

It is different because it has lymphoid follicles | And it is bronchiolocentric.

Sjogren's NSIP is characterized b y an abundance of proliferating lymphocytes. These lymphocytes will inevitable make some mistakes and become.......or......... causing ..........or..........

semiautonomous or autonomous causing psuedo-lymphoma or lymphoma

Malignant neoplastic lymphomas are how much more common in Sjogren's patients

44 times

Signs that a sjogren's patient has lymphoma?

Mediastinal lymphadenopathy seen on CT, pleural effusion, prominent nodularity of lung lesions, lung cysts

Rheumatology pts receiving immunosuppresives frequently get pulmonary infections. How do you distinguish these pulmonary infections from rheumatologic lung disease?

Rheumatologic lung disease is never acute. It is also very rarely nodular

Dermatomysitis/ Polymyositis is associated with ?

Malignancy (12% of all myositis pts) | Also, respiratory failure from muscle weakness