Nichols RA lung and some Vasculitis Flashcards
Describe the features of Polyarthritis Nodosa
How would you characterize it and does it involve granulomas and nephropathies.
Necrotizing inflammation of the medium sized arteries
It is a segmental, Transmural, Nodular Arteritis with fibrinoid necrosis
Does not involve granulomas or nephropathis
What will the labratory findings be in PAN ?
Anemia
Thrombocytopenia
Increased ESR and CRP
Hematuria without RBC casts
Elevated Creatine
Describe these findings and what disease does this lead you toward ?
PAN with Fibrinoid Necrosis and Neutrophillic infiltrate. This is in the acute phase.
FIBRINOID NECROSIS WITH IMMUNE DEPOSITIS AND MICROANEURISM—-> PAN EVERY FUCKING TIME
This Trichrome stain, What are the findings ?
This is a transmural vasculitis with Fibrinoid Necrosis which is highlighted by the trichrome stain. This would be Polyarteritis Nodosa
How do you diagnose PAN ?
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
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.
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
What is the most prominant feature of AC mediated vasculitis ?
Cutaneous involvement of small blood vessels.
Ex: Palpable purpura, pustules, vessicles, uticaria, and small ulcerations.
What vasculitis is associated with a long standing Hep C infection ?
Cryoglobulinemic vasculitis—> mixed cryoglobulinemia because it is often associated with IgM and IgG
What type of vasculitis is associated with medication administration ?
Hypersensitivity vasculitis.
What is hypersensitivity vasculitis ?
What is the mechanism of Hypersensitivity vasculitis ?
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
What is this and what vasculitis is it associated with ?
Livedo Reticularis and it is associated with Polyarteritis Nodosa
What causes helonch Schonlein Purpura / Allergic Granulamatosis with polyangiitis?
What is classic of this disease ?
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.
What is this a pathological manifestation of ?
Leukocytoclastic vasculitis. —> notice the invasion of the small capillaries and venules with neurophils that will be activated and release their debris.
What do you see in this pathologic slide
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
What are the manifestations of Scleroderma in the Lung?
NSIP and sometimes UIP
Concentric hypertrophy of the pulmonary arteries.
What is the early pathophysiology of Scleroderma lung ?
IL-8 -> Neutrophil attractant
TNF–>Neut att and Endo Activ
MIF1-Alpha–> Neutrophil Chemotax
RANTS–> T Cell Act
Endothelin-1 Vasoconstrictor
What is the late phase of scleroderma lung ?
TGF-Beta that promotes fibroplasia
Describe in general the process of scleroderma lung ?
Ground Glass infiltrates then later reticular infiltrates
Early or late scleroderma lung ?
Late—> Reticular Infiltrates
What is this ?
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
What is this ?
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
What is this ?
Concentric thickening of the pulmonary artery with the features of non specific interstital pneumonia.
SCLERODERMA
Pyspnea, Dry Cough, VELCRO CRACKELS
Restrictive patterns of DLCO
Anti-SCL 70
Scleroderma
Should you give scleroderma patients steroids ?
NO It will precipitate renal failure.
What is the most common pleuropulomnary disease in lupus ?
Pleuritis- This can be the only symptom
What is this ?
Fibrinous Pleuritis in Lupus
What are the 4 Lung Complications of Lupus ?
Pleuritis
Acute Lupus Pneumonitis
Nonspecific Interstital Pneumonia (NSIP)
Pulmonary Vascular Disease
What is this ?
Fibrinous Pneumonitis, This is associated with Lupus and it is because the fibroblasts have fibrosed this lung
What is on the top and what is on the bottom ?
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
Dyspnea, Fever, Cough, WET PULMONARY CRACKELS and GROUND GLASS OPACITIES
Acute Lupus Pneumonitis
If you suspect pulmonary vascular disease with Lupus how could you differentiate between …
Concentric Arterial thickening and
**Thromboembolic Disease **
Thrombotic disease will be associated with Lupus Anticoagulant
ANTI PHOSPHOLIPID ANTIBODY SYNDROME
What is this ?
Thromboembolic disease with concentric thickening of pulmonary arteries in Lupus
What are these ?
Pulmonary artery webs, fibrous tissue spanning the lumen with old pulmonary organized emboli
What are the most common findings in RA Lung
Germinal centers and Tons of B and T cell lymphocytes
Most comon RA lung manifestations ?
Bronchioles on the top and the pleura on the bottom
Why would RA patients have dyspnea, Velcrow Crackles, and low DLCO ?
NSIP
What is this ?
What disease can this be a complication of ?
What are M and L ?
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
What is this ?
What will it look like on CT ?
Will this respond to steroids ?
This is follicular bronchiolitis
It will look like ground glass opacity in the bronchiolar distribution with nodules
Yes responds to steroids
Does organizing pneumonia respond to steroids ?
It presents with acute onset of fever and alveolar infiltrates
Yes
Does obstructive obonchiolitis respond to steroids ?
NO IT DOES NOT
It presents with obstructive changes on PFT’s
What two diseases is this ?
Interstital Pneumonia and ALI
e is for edema
hm is for hylane membranes
What can methotrexate cause ?
Pneumonitis with chronic interstital pneumonia with a UIP pattern
Dyspnea cough and Fever bilateral crackels being treated for RA….. with this ?
What could save this persons life ?
Pneumonitis characterized by lymphocytic interstital infiltration due to MTX
Steroids will save this persons life
What drug can cause this with chronic toxicity ?
This is late stage fibrosis associated with MTX
What are the more common toxicities associated with MTX ?
Neutropenia and Hepatic toxicity
But pneumonitis has a higher chance of being fatal
Do you see fibrosis in RA patients without MTX ?
Yes in 20% and it can be aggravated by MTX therapy
How is the NSIP seen in sogren syndrome unique ?
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
How can you tell if a Sogren patient is getting a lymphoma ?
It will appear on a CT scan or lung biopsy
What is unique about this NSIP ?
It has abundant Lymphocytes —–> Sogren Syndrome
LYMPHOMA x44 risk
What is unique about this NSIP ?
It has multinucleated giant cells and macrophages
Sogren Syndrome
A Rheumatoid patient on immunosupressants begins to cough… How could you differentiate rheumatic lung disease from a lung infection ?
Rheumatologic Lung Disese is rarly **Acute **
And it is rarely **Nodular **
What are the 6 important things about Autoimmune Interstital Pneunomia ?
- It is Chronic
- Dyspnea (+/- ) cough
- Velcro Crackles
- Basal, Posterior, Peripheral Ground Glass or reticular infiltrates
- Restrictive Pattern of PFT’s
- Decreased DLCO
What is the most common type of Acute Lung disease in a RA patient ?
More Likely Infectous than Rheumatologic
Subacute or chronic dyspnea in a RA patient is most likely what ?
More likely Rheumatoligic than infectous
Nodular Lung Disease in a rheum patient is most likely what ?
INFECTOUS than neoplastic or rheumatoligic
What is the cause of death with myositis ?
ILD + Organizing Pneumonia
What will tell you on a biopsy if a patient with Sogren might have a lymphoma ?
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
Patient has wet pulmonary crackels, fever, dyspnea, cough, rash
Pulmonary effusion and ground glass opacity
What is the pathophysiology of the pulmonary findings ?
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
Small Lungs, Decreased DLCO, Rash, Restrictive PFT’s
Shrunken Lung Syndrome with Lupus
What is the pathophysiology of this ?
Webs in the pulmonary artery are evidance of organized thrombus—> Chronic Organizing thromboembolic disease
