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Rheumatology and MC System > Nichols RA lung and some Vasculitis > Flashcards

Nichols RA lung and some Vasculitis Flashcards

1
Q

Describe the features of Polyarthritis Nodosa

How would you characterize it and does it involve granulomas and nephropathies.

A

Necrotizing inflammation of the medium sized arteries

It is a segmental, Transmural, Nodular Arteritis with fibrinoid necrosis

Does not involve granulomas or nephropathis

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2
Q

What will the labratory findings be in PAN ?

A

Anemia

Thrombocytopenia

Increased ESR and CRP

Hematuria without RBC casts

Elevated Creatine

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3
Q

Describe these findings and what disease does this lead you toward ?

A

PAN with Fibrinoid Necrosis and Neutrophillic infiltrate. This is in the acute phase.

FIBRINOID NECROSIS WITH IMMUNE DEPOSITIS AND MICROANEURISM—-> PAN EVERY FUCKING TIME

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4
Q

This Trichrome stain, What are the findings ?

A

This is a transmural vasculitis with Fibrinoid Necrosis which is highlighted by the trichrome stain. This would be Polyarteritis Nodosa

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5
Q

How do you diagnose PAN ?

A

78 % of PAN cases present with peripheral neuropathy—> sensory dysthesias are the predominating clinical feature.

The patient will present with foot drop.

NERVE BIOPSY FOR DIAGNOSIS

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6
Q

40 year old in the ER with MI + Foot Drop + Renal Failure in surgery because of severe abdominal pain

Has hematuria but no RBC casts.

A

PAN

Neuropathy with wrist and foot drop with sensory symptoms

GI with acute abdomen and necrotizing cholecystitis and pancreatitis

Cardiac manifestation as MI CHF and pericarditis

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7
Q

What is the most prominant feature of AC mediated vasculitis ?

A

Cutaneous involvement of small blood vessels.

Ex: Palpable purpura, pustules, vessicles, uticaria, and small ulcerations.

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8
Q

What vasculitis is associated with a long standing Hep C infection ?

A

Cryoglobulinemic vasculitis—> mixed cryoglobulinemia because it is often associated with IgM and IgG

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9
Q

What type of vasculitis is associated with medication administration ?

A

Hypersensitivity vasculitis.

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10
Q

What is hypersensitivity vasculitis ?

What is the mechanism of Hypersensitivity vasculitis ?

A

An immune complex mediated small vessel vasculitis of the skin that spares internal organs and usually follows drug exposure.

It is caused by IC deposition in capillaries and post capillary venules and arterioles.

Similiar to a serum sickness reaction to an administered drug

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11
Q

What is this and what vasculitis is it associated with ?

A

Livedo Reticularis and it is associated with Polyarteritis Nodosa

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12
Q

What causes helonch Schonlein Purpura / Allergic Granulamatosis with polyangiitis?

What is classic of this disease ?

A

IgA deposition within the vessels

Tetrad of purpura, arthritis, and abdominal pain and glomerulonephritis. Purpura are usually in the lower extremity and DO NOT BLANCH

Elevated IgA and IgA1 in the serum.

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13
Q

What is this a pathological manifestation of ?

A

Leukocytoclastic vasculitis. —> notice the invasion of the small capillaries and venules with neurophils that will be activated and release their debris.

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14
Q

What do you see in this pathologic slide

A

This is a later stage of the leukocytoclastic exocytosis. The neurtophils have extravasated out of the blood vessel into the interstitum and have been activated to release their toxic debris.

Heleonch Schonlein Purpura

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15
Q

What are the manifestations of Scleroderma in the Lung?

A

NSIP and sometimes UIP

Concentric hypertrophy of the pulmonary arteries.

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16
Q

What is the early pathophysiology of Scleroderma lung ?

A

IL-8 -> Neutrophil attractant

TNF–>Neut att and Endo Activ

MIF1-Alpha–> Neutrophil Chemotax

RANTS–> T Cell Act

Endothelin-1 Vasoconstrictor

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17
Q

What is the late phase of scleroderma lung ?

A

TGF-Beta that promotes fibroplasia

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18
Q

Describe in general the process of scleroderma lung ?

A

Ground Glass infiltrates then later reticular infiltrates

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19
Q

Early or late scleroderma lung ?

A

Late—> Reticular Infiltrates

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20
Q

What is this ?

A

Scleroderma Lung Nonspecific Interstital Pneumonia pattern.

Lymphocytes and macrophages in the interstitum followed by fibroblasts and collagen

NOTICE ALL AREAS ARE IN THE SAME STAGE

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21
Q

What is this ?

A

Interstital fibrosis from Fibrosing lung disease NSIP Pattern.

Is this isolated to the lung or are there other features of scleroderma ?

Distinguish it from Pneumonia because the treatments are opposite

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22
Q

What is this ?

A

Concentric thickening of the pulmonary artery with the features of non specific interstital pneumonia.

SCLERODERMA

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23
Q

Pyspnea, Dry Cough, VELCRO CRACKELS

Restrictive patterns of DLCO

Anti-SCL 70

A

Scleroderma

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24
Q

Should you give scleroderma patients steroids ?

A

NO It will precipitate renal failure.

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25
Q

What is the most common pleuropulomnary disease in lupus ?

A

Pleuritis- This can be the only symptom

26
Q

What is this ?

A

Fibrinous Pleuritis in Lupus

27
Q

What are the 4 Lung Complications of Lupus ?

A

Pleuritis

Acute Lupus Pneumonitis

Nonspecific Interstital Pneumonia (NSIP)

Pulmonary Vascular Disease

28
Q

What is this ?

A

Fibrinous Pneumonitis, This is associated with Lupus and it is because the fibroblasts have fibrosed this lung

29
Q

What is on the top and what is on the bottom ?

A

Acute Lupus Pneumonitis.

On top you have alveolitis with loose fibrin exudate / Lymphocytes and neutrophils in the alveolus

On bottom you ahve hemorrhage into the alveoli. Lupus can present with both

30
Q

Dyspnea, Fever, Cough, WET PULMONARY CRACKELS and GROUND GLASS OPACITIES

A

Acute Lupus Pneumonitis

31
Q

If you suspect pulmonary vascular disease with Lupus how could you differentiate between …

Concentric Arterial thickening and

**Thromboembolic Disease **

A

Thrombotic disease will be associated with Lupus Anticoagulant

ANTI PHOSPHOLIPID ANTIBODY SYNDROME

32
Q

What is this ?

A

Thromboembolic disease with concentric thickening of pulmonary arteries in Lupus

33
Q

What are these ?

A

Pulmonary artery webs, fibrous tissue spanning the lumen with old pulmonary organized emboli

34
Q

What are the most common findings in RA Lung

A

Germinal centers and Tons of B and T cell lymphocytes

35
Q

Most comon RA lung manifestations ?

A

Bronchioles on the top and the pleura on the bottom

36
Q

Why would RA patients have dyspnea, Velcrow Crackles, and low DLCO ?

A

NSIP

37
Q

What is this ?

What disease can this be a complication of ?

What are M and L ?

A

This is a UIP pattern of lung fibrosis. This can be seen in RA.

M are mucinous microcysts. They are the early stage of honey comb lung

L are lymphoid granules

38
Q

What is this ?

What will it look like on CT ?

Will this respond to steroids ?

A

This is follicular bronchiolitis

It will look like ground glass opacity in the bronchiolar distribution with nodules

Yes responds to steroids

39
Q

Does organizing pneumonia respond to steroids ?

A

It presents with acute onset of fever and alveolar infiltrates

Yes

40
Q

Does obstructive obonchiolitis respond to steroids ?

A

NO IT DOES NOT

It presents with obstructive changes on PFT’s

41
Q

What two diseases is this ?

A

Interstital Pneumonia and ALI

e is for edema

hm is for hylane membranes

42
Q

What can methotrexate cause ?

A

Pneumonitis with chronic interstital pneumonia with a UIP pattern

43
Q

Dyspnea cough and Fever bilateral crackels being treated for RA….. with this ?

What could save this persons life ?

A

Pneumonitis characterized by lymphocytic interstital infiltration due to MTX

Steroids will save this persons life

44
Q

What drug can cause this with chronic toxicity ?

A

This is late stage fibrosis associated with MTX

45
Q

What are the more common toxicities associated with MTX ?

A

Neutropenia and Hepatic toxicity

But pneumonitis has a higher chance of being fatal

46
Q

Do you see fibrosis in RA patients without MTX ?

A

Yes in 20% and it can be aggravated by MTX therapy

47
Q

How is the NSIP seen in sogren syndrome unique ?

A

It is Lymphocytic or Lymphoid LIP

Has tons of proliferating lymphocytes. The rapid divisions can lead to LYMPHOMA

Lymphoma is 44 times more common in Sojgren

48
Q

How can you tell if a Sogren patient is getting a lymphoma ?

A

It will appear on a CT scan or lung biopsy

49
Q

What is unique about this NSIP ?

A

It has abundant Lymphocytes —–> Sogren Syndrome

LYMPHOMA x44 risk

50
Q

What is unique about this NSIP ?

A

It has multinucleated giant cells and macrophages

Sogren Syndrome

51
Q

A Rheumatoid patient on immunosupressants begins to cough… How could you differentiate rheumatic lung disease from a lung infection ?

A

Rheumatologic Lung Disese is rarly **Acute **

And it is rarely **Nodular **

52
Q

What are the 6 important things about Autoimmune Interstital Pneunomia ?

A
  1. It is Chronic
  2. Dyspnea (+/- ) cough
  3. Velcro Crackles
  4. Basal, Posterior, Peripheral Ground Glass or reticular infiltrates
  5. Restrictive Pattern of PFT’s
  6. Decreased DLCO
53
Q

What is the most common type of Acute Lung disease in a RA patient ?

A

More Likely Infectous than Rheumatologic

54
Q

Subacute or chronic dyspnea in a RA patient is most likely what ?

A

More likely Rheumatoligic than infectous

55
Q

Nodular Lung Disease in a rheum patient is most likely what ?

A

INFECTOUS than neoplastic or rheumatoligic

56
Q

What is the cause of death with myositis ?

A

ILD + Organizing Pneumonia

57
Q

What will tell you on a biopsy if a patient with Sogren might have a lymphoma ?

A

Lack of nodularity, Lack of follicles and lack of multinuclear giant cells.

Think—> you have a LYMPHOMA, so you will get Lymphocytic proliferations with Sogren syndrome

58
Q

Patient has wet pulmonary crackels, fever, dyspnea, cough, rash

Pulmonary effusion and ground glass opacity

What is the pathophysiology of the pulmonary findings ?

A

Acute Lupus Pneumonitis —> There will be a loose fibrinous infiltrate that can be hemorrhagic and can organize

__> Hemorrhagic disease will also present with anti-phospholipid antibody disease

59
Q

Small Lungs, Decreased DLCO, Rash, Restrictive PFT’s

A

Shrunken Lung Syndrome with Lupus

60
Q

What is the pathophysiology of this ?

A

Webs in the pulmonary artery are evidance of organized thrombus—> Chronic Organizing thromboembolic disease

61
Q
A

Rheumatology and MC System (4 decks)

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