356 - The Vasculitis Syndrome Flashcards
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
c-ANCA=___, common in ___
cytoplasmic Anti-neutrophil cytoplasmic antibody
Wegner’s
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
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
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
In GPA we can find multifocal _-lateral infiltrations in the lungs. URT gets damages especially in the ___ and ___.
bi
nasopharynx
sinuses
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)
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
Which systems can be involved in GPA? (6)
respiratory (90%) renal (72%) ocular (52%) cutaneous (46%) nervous (23%) cardiac (8%)
What are the clinical symptoms of the respiratory system in GPA (4)?
asymptomatic infiltration
cough
hemoptysis
dyspnea+ chest pain
What are the clinical symptoms of the ocular system in GPA (3)?
conjunctivitis
episcleritis
sclerites
What are the clinical symptoms of the cutaneous system in GPA (5)?
papule vesicles palpable rash ulcers nodules
What are the clinical symptoms of the cardiac system in GPA (3)?
pericarditis
coronary arteries vasculitis
cardiomyopathy
What are the clinical symptoms of the nervous system in GPA (3)?
cranial neuritis
mononeuritis multiplex
brain vasculitis/granuloma
Non specific GPA symptoms such as (4) may also appear when the disease is active.
fever
arthralgia
lethargy
weight loss/anorexia
In GPA patients there is an increase in ___ ___ events. Therefor we must pay attention to __ and ___
venous
thromboembolic
PE
DVT
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
Lab results of GPA patients may include an increase in __, ___, ___and ___, ____ (especially IgA)
ESR anemia leukocytosis thrombocytosis hypergammaglobulinemia
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
Name 5 different disease in the DDx of GPA:
anti GBM (Goodpasture’s syndrome)
URT/lung malignancies
lymphoma
infectious disease (histoplasmosis/leishmania)
GPA treatment includes two phases:
1- Induction to remission
2- Maintenance
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
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
Microscopic polyangiitis is a __ and ___vessel disease of ___ vasculitis type. ___ Ab are common.
small
medium
necrotizing vasculitis
ANCA
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)
