356 - The Vasculitis Syndrome Flashcards by Salman Majid

ANCA is a family of autoantibodies, auto immunogenic that act against proteins in the ___ of ___ and ___. They tend to be high in ___ or ___, and low in ___.

granules
neutrophils
monocytes
Wegner's
microscopic polyangiitis
Churg Strauss

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c-ANCA=___, common in ___

cytoplasmic Anti-neutrophil cytoplasmic antibody

Wegner’s

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p-ANCA=___, is more common in ___. Nevertheless only Ab for ___ can be found in ___, ___, ___.

perinuclear Anti-neutrophil cytoplasmic antibody
IBD
MPO
microscopic polyangiitis
Churg Strauss
Wegner's

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Clinical symptoms that suggest vasculitis include: (5). Remember we must first overrule other disease with similar presentation (mostly infectious)

palpable urticaria
pulmonary infiltration/microscopic hematuria
chronic sinusitis
mononeuritis multiplex
GN with systemic disease

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What is the classic triad of Wegner’s (granulomatosis with polyangiitis- GPA)? What gets damaged?

granulomatosis vasculitis of the URT/LRT + GN

necrotizing of small veins together with granuloma formation inside/outside the blood vessel

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In GPA we can find multifocal _-lateral infiltrations in the lungs. URT gets damages especially in the ___ and ___.

bi
nasopharynx
sinuses

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Renal involvement in GPA in its early stages is defined by ___ and ___inflammation of the __ which can evolve into ___.

focal
segmental
glomeruli
RPGN (rapidly progressive glomerulonephritis)

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In GPA patients we can find an increase in ___ blood cells which secret an increased amount of ___ and ___ with an increase of ___. Additionally, there will be high levels of ___ secreted from monocytes.

mononuclear 
IFN- gamma
TNF- alpha
T CD4
IL 12

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Which systems can be involved in GPA? (6)

respiratory (90%)
renal (72%)  
ocular (52%)
cutaneous (46%)
nervous (23%)
cardiac (8%)

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What are the clinical symptoms of the respiratory system in GPA (4)?

asymptomatic infiltration
cough
hemoptysis
dyspnea+ chest pain

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What are the clinical symptoms of the ocular system in GPA (3)?

conjunctivitis
episcleritis
sclerites

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What are the clinical symptoms of the cutaneous system in GPA (5)?

papule
vesicles
palpable rash
ulcers
nodules

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What are the clinical symptoms of the cardiac system in GPA (3)?

pericarditis
coronary arteries vasculitis
cardiomyopathy

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What are the clinical symptoms of the nervous system in GPA (3)?

cranial neuritis
mononeuritis multiplex
brain vasculitis/granuloma

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Non specific GPA symptoms such as (4) may also appear when the disease is active.

fever
arthralgia
lethargy
weight loss/anorexia

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In GPA patients there is an increase in ___ ___ events. Therefor we must pay attention to __ and ___

venous
thromboembolic
PE
DVT

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90% of patients with active GPA have anti-PR3 ___, and in a non active disease- __%. A small percentage will also have __ instead of anti PR3, and 20% will not have __ at all.

ANCA
60%
MPO
ANCA

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Lab results of GPA patients may include an increase in __, ___, ___and ___, ____ (especially IgA)

ESR
anemia
leukocytosis
thrombocytosis
hypergammaglobulinemia

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Diagnosing GPA is through ___ showing ___ in a patient with the suspected clinical presentation. The best location to sample is the ___.

biopsy
necrotizing granulomatous vasculitis
lung

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Name 5 different disease in the DDx of GPA:

anti GBM (Goodpasture’s syndrome)
URT/lung malignancies
lymphoma
infectious disease (histoplasmosis/leishmania)

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GPA treatment includes two phases:

1- Induction to remission

2- Maintenance

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In severe cases of GPA, __+__ is given to achieve remission. In fulminant disease we will also add ___. Other alternative could be ___ or ___

cyclophosphamide
glucocorticoids
plasmapheresis
rituximab
methotrexate

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If cyclophosphamide was used for induction use one of the following drugs for maintenance: (4). This treatment should be given for at least ___ years from remission

methotrexate
azathioprine 
mycophenolate mofetil
rituximab 
2

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Microscopic polyangiitis is a __ and ___vessel disease of ___ vasculitis type. ___ Ab are common.

small
medium
necrotizing vasculitis
ANCA

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What is the main difference between GPA and microscopic polyangiitis?

the lack of granulomas in the latter

When the vasculitis is more focused in the ___ or ___ it is more likely to GPA

URT | lung nodules

The treatment of ___ and ___ is similar and includes: (3)

``` GPA microscopic polyangiitis cyclophosphamide prednisone rituximab ```

Churg Strauss involves the __ and __ blood vessels.

small | medium

In Churg Strauss it is common to see: (5)

asthma eosinophilia extravascular granuloma vasculitis

Churg Strauss patients usually present with non specific clinical symptoms of: (4). In the early stages ___ and ___ are common.

``` fever anorexia abdominal pain loss of weight rhinitis allergic sinusitis ```

The second most common clinical presentation of Churg Strauss is:

mononeuritis multiplex

The typical lab results of patients with Churg Strauss include:

> 1000 eosinophils increased ESR fibrinogen anti MPO ANCA

In order to diagnose a Churg Strauss patient, we must see ___, ____ in peripheral blood, and typical ____ clinical manifestation.

asthma eosinophils vasculitis

If untreated Churg Strauss patients have a poor prognosis with a __ year survival of __%. The main cause for mortality is ___ involvement. For this reason, ___ should be performed when diagnosed.

5 25 cardiac echocardiography

In most Churg Strauss patients ___ will be sufficient treatment.

glucocorticoid

polyarteritis nodosa (PAN) is a ___ and ___ necrotizing vasculitis with systemic and ___ involvement

small medium renal

IN PAN the vascular lesions tend to be ___ and involve the ___ and ___ of the arteries and nearby veins.

segmental bifurcations branching

If we see involvement of __ in polyarteritis nodosa than it is most likely infect ___

venules | microscopic polyangiitis

The renal pathology in PAN is of ___ without ___. In patients with substantial HTN the kidney may have ___ features

arteritis glomerulonephritis glomerulosclerosis

In patients with viral infections (___ or ___) similar vasculitis to PAN can appear. Another disease that may be associated to PAN is ___

HCV HBV hairy cell leukemia

PAN will have non specific clinical presentation (5), and complains regarding the affected organs.

``` fever weight loss abdominal pain lethargy/myalgia headache ```

In order to diagnose PAN we must perform ___ from a relevant site (__, __, __). If it is not possible, we can use ___ to show aneurisms in small/medium blood vessels.

``` biopsy testis skin patches muscles arteriography ```

There is no diagnostic ___ test in PAN. usually we will see ____with ___ dominance. Other results may include ___, ___, ___.

``` serologic leukocytosis neutrophils anemia (of chronic disease) ESR hypergammaglobulinemia ```

If left untreated, PAN has a __ prognosis. Death may occur mainly due to ___ or ___complication. Treatment should include ___ and ___ to improve survival.

``` bad GI cardiovascular prednisone cyclophosphamide ```

In less sever cases of PAN we can treat patients with __. In HBV patients with PAN treat with ___ + __ + __. Remember that treating HTN will also improve survival

GC antiviral GC plasmapheresis

Temporal arteritis= ___

Giant cell arteritis & polymyalgia rheumatica

Temporal arteritis is an inflammation of the __ and __ arteries, usually involving the branches of the ___, especially the __ but also the __ in its branches.

``` medium large carotid temporal aorta ```

Temporal arteritis is more common in __ __ __ under the age of __. A correlation was found to HLA DR4 and HLA-DRB1

young white women 50

In __% of patients with temporal arteritis we will also find ___ syndrome which characterized in pain and stiffness of the muscles (4)

``` 50 polymyalgia rheumatica neck back shoulders knees ```

The pathogenesis of temporal arteritis involves the attachment of __ cells to the walls of the arteries encouraging __ formation. In the blood vessel we will see ___ cells leading to damage of the internal elastic ___ layer

T macrophages giant lamina

Temporal arteritis is more common in patients > __ with general symptoms. When cranial arteries are involved the main complain is ___ together with a sensitive and thicken ___.

50 | artery

In temporal arteritis, ischemic cranial complications may include ___ and ___ claudication. The ___ nerve may also be ischemic- leading to a sudden ___. Before those complain appear the patient will suffer from other head/eyes complains.

scalp tongue optic loss of sight

The lab results we will typically see in temporal arteritis are: (5)

``` ESR normochromic anemia liver functions damage (ALP) IgG hypercomplementemia ```

In temporal arteritis there is an increased risk for thoracic aorta aneurysm (X__). The main goal of treatment is to reduce events of loss of ___.

18 | sight

``` Temporal arteritis treatment includes ___. If there are ocular symptoms we should consider giving ___. We should also add ___ to reduce cranial events. Adding ___ (IL6 receptor antagonist) can be complementary to GC treatment. ```

prednisone methyl prednisone aspirin tocilizumab

Takayasu arteritis is an inflammation of the __ and __ arteries with a tendency to involve the ___ and the ___ artery. More common in female___ and in ___

``` medium large aorta pulmonary teenagers Asians ```

In Takayasu there is an ___ of inflammatory cells to the blood vessels- ___ in the intima, ___ of the media layer. As a result the lumen will ___ and sometimes even by ___.

``` invasion fibrosis scarring narrow thrombi ```

Takayasu clinical manifestation include ___ symptoms , followed by the loss of ___ in the involved blood vessels (especially in the ___ artery

general pulse subclavian

Takayasu should be suspected in ___ women with a decreased peripheral ___ and with abnormal ___ and arterial ___.

young pulse HTN murmurs

Using ___ in Takayasu helps to confirm the diagnosis. The least likely artery to be affected is the ___ artery

arteriography | coronary

Histological examination of arteries of Takayasu patients will show: (3) in the media.

lymphocytes granulomas giant cells

IgA vasculitis= ____

Henoch Schonlein

IgA vasculitis is a ___ vessel inflammation, characterized with palpable ___ in the ___ and lower extremities areas together with arthralgia, GI symptoms and ___.

small rash buttocks GN

Henoch Schonlein is more common in children between the ages of ___ but also in infants and adults.. It is more common during the ___ season

4-7 | spring

The common abdominal pain in IgA vasculitis is ___, together with other GI symptoms (3)

colic diarrhea/constipation vomiting blood/mucus in PR

The renal involvement in IgA vasculitis (10-50% of patients) includes light GN (3) which will resolve spontaneously

proteinuria microscopic hematuria RBC casts

The lab results of Henoch Schonlein includes: (3)

leukocytosis eosinophilia Increased IgA

Diagnosing IgA vasculitis is mostly based on ___ and symptoms. Skin biopsy may show vasculitis with __ and __ deposition

clinical presentation IgA C3

Henoch Schonlein usually resolves ____. In order to reduce the edema we can use __ but it does not improve cutaneous symptoms or joint pain.

spontaneously | prednisone

Cryoglobulins are Ab that become sediments in __ temperature

cold

Cryoglobulins can be found in the following disorders: (5)

``` lymphoproliferative disorders MM connective tissue disorders infections hepatic disorders ```

The most common causes for essential mixed cryoglobulinemia (__/__ mix sediments)is ___

IgM IgG chronic hepatitis infection

The most common clinical presentation of cryoglobulinemia vasculitis is ___. Other manifestations include: (3)

cutaneous vasculitis arthritis peripheral neuropathy GN

Less common clinical manifestations of cryoglobulinemia vasculitis include: (2)

RPGN | CNC/cardiac vasculitis

When examining the lab results of patients with cryoglobulinemia vasculitis, we can see: (5)

``` cryoprecipitates RF ESR anemia hypocomplementemia ```

In every cryoglobulinemia vasculitis patient we should look for ___ infection

HCV

Treatment for cryoglobulinemia include ___ treatment and ___ which will improve symptoms but will not cure. Another drug is ___

anti viral GC rituximab (anti CD20)