Judging the Relevance and worth of ideas part 2.pptx
D. Fadhil Vasculitis-7 (Muhadharaty) (1).pptx
1.
2.
3.
4.
Vasculitides constitute a
spectrum of diseases
characterized by inflammation
& necrosis of blood vessels with
resulting ischemia of those
tissues supplied by the affected
vessels. These are rare diseases
& have late diagnosis.
10.
3- aortic type= Takayosu
disease, polyarteritis nodosa(in
the latter, the subclavian artery
is spared ).
4- pulmonary type= Wegener's
granulomatous, Churg- Strauss
syndrome
11. GIANT CELL ARTERITIS(GCA)/
POLYMYALGIA RHEUMATICA
GCA also called temporal
arteritis or cranial arteritis &
this is the most common type
of systemic vasculitis.
12. GIANT CELL ARTERITIS(GCA)/
POLYMYALGIA RHEUMATICA
It especially involve
branches of carotid artery
of patients over the age of
50, often female patients.
13.
C/F : headache not
responding to analgesia, scalp
tenderness, visual
disturbances, jaw
claudication, arthralgia&
monocular blindness which is
irreversible. 90% of patients
have high ESR.
14.
15.
Biopsy of a segment of
temporal artery; reveals
characteristic findings of
inflammation &
destruction of internal
elastic lamina with
GIANT cells present.
16. POLYMYALGIA RHEUMATICA(PMR)
Characterized by pain , stiffness
of shoulders & hip girdles &
proximal extremities. It also
associated with high ESR&
anemia.
It commonly occurs in female
over 50 years.
17.
Diagnosis is made on clinical
grounds& rapid response to
steroids.
PMR& GCA are likely part of
same disease spectrum & some
patients may have symptoms of
both diseases.
18.
In GCA, corticosteroids
treatment is obligatory
because they significantly
reduce the irreversible visual
loss and other focal ischemic
lesions, but much higher
doses are needed than in
PMR.
19.
Treatment should not
be delayed, especially if
there have already been
episodes of visual loss
or stroke.
20.
Starting doses of prednisolone
are:-
PMR: 10-15 mg as a single dose
in the morning.
GCA: 60-100 mg, usually in
divided doses.
The dose should be reduced
gradually in weekly or monthly
steps.
21.
Calcium and vitamin D
supplement and sometimes
bisphosphonates are necessarily
needed to prevent osteoporosis
while high dose of steroids are
given. In PMR, the response
appears in a matter of few days.
22.
The outcome of both
disease is excellent , but
relapse may occur
during treatment or
years after cessation of
treatment.
23.
TAKAYASU'S ARTERITIS is
a large vessel disease that
affect young female but can
be seen in both male &
female up to the age of 50. It
also known as pulse less
disease.
24.
TA results in stenosis of aorta &
its branches like cerebral ,
brachiocephalic, renal ,
mesenteric , femoral & coronary
arteries.
Stenosis of proximal aorta& its
proximal branches is the most
common finding.
25.
26.
27.
Clinically limb claudication ,
lightheadedness, malaise, fever
& arthralgia , all are
experienced by such patients.
There was a delay in diagnosis
for years even if the stenosis is
sever because most patients are
asymptomatic.
28.
Diagnosis is made by
angiography. Treatment
includes chronic steroids. Some
patients may respond to
immunosuppressive drugs like
methotrexate&
cyclophosphamide. Some
patients require corrective
vascular angioplasty.
29.
POLYARTERITIS
NODOSA(PAN) is a medium
vessel inflammatory vasculitis
involving segmental necrotizing
lesions often at arterial branch
points leading to stenosis,
thrombosis, hemorrhage or
infarction of the involved artery.
30.
Classical PAN is rare condition
which, unlike other vasculitic
diseases, usually occurs in middle-
age men. It is accompanied by sever
systemic manifestations, and its
occasional association with
hepatitis B antigenaemia suggest a
vasculitis secondary to the
deposition of immune complexes.
34. CLINICAL FEATURES::
These include fever,
malaise, weight loss and
myalgia. These initial
symptoms are fallowed by
dramatic acute features
that are due to organs
infarction.
35.
* Neurological features-
mononeuritis multiplex
* Abdominal pain due to arterial
involvement of abdominal viscera,
mimicking acute cholecystitis,
pancreatitis and appendicitis.
* Renal- presents with hematuria
and proteinuria. Hypertension and
acute /chronic renal failure occur.
36.
* Cardiac- Coronary arteritis
causes myocardial infarction
and heart failure.
* Skin –subcutaneous
hemorrhage and gangrene
occur.
* Lung involvement is rare.
37. INVESTIGATIONS::
-blood count. Anemia, leukocytosis
and high ESR.
* biopsy of involved artery.
-angiography. Demonstrate
microaneurysms in hepatic,
intestinal or renal vessels.
Most patients are undiagnosed
until postoperatively for ischemic
events.
39.
Churg-Strauss syndrome also
known as allergic
granulomatosis& agiitis& this is
a medium & small vessel
vasculitis that has extravascular
manifestations which can
distinguish it from other types
of vasculitis.
40.
The typical presentation is a
middle age person with
chronic asthma who develops
pulmonary infiltrates,
vasculitis& eosinophilia. The
vasculitis involves the skin,
peripheral nerves& GIT, but
other organs may be affected.
41.
Biopsy shows
microgranulomas& eosinophilic
deposits. The pulmonary
infiltrates are patchy but rapidly
resolve with steroid treatment.
Recent reports showed that anti-
leukotriene drugs used in
treatment of asthma may cause
CSS.
42.
Diagnosis is made depending on
clinical findings but biopsy is often
needed. Eosinophilia is present in
almost all patients but rapidly
returns to normal on giving
steroids. Anti-neuotrophil
cytoplasmic auto antibodies(P-
ANCA) are positive in most
patients.
43.
Treatment is based on
steroids, but
immunosuppressive drugs
may be needed in some
patients. The prognosis is
fairly good but relapse rate is
common.
44.
WEGENER'S GRANULOMATOSIS(WG)
small& medium vessel vasculitis with
many extra vascular manifestations.
Although any anatomic area can be
affected, the three most common sites
of involvement are : the sinuses&
upper airways, the lungs, & the
kidneys.
50.
The patients may only
diagnosed after months or years
of subtle symptoms. However
WG can be presented with
alveolar hemorrhage & or
rapidly progressive GN, both of
which account for much of the
mortality from the disease.
51.
Destruction of nasal sinus
tissues may result in facial
deformities. Pseudo tumor
can occur everywhere but
commonly in lungs & retro
orbital spaces. Skin ,
peripheral nerves & eye
involvement are common.
52.
Diagnosis is made on tissue
biopsy. ANCA of C-
ANCA(cytoplasmic) is positive
in 90% of patients with renal
disease&70% without renal
disease. P-ANCA also can be
found.
The mortality of untreated WG
approaches 100%.
53.
Treatment is steroids, but this is
inadequate. Therefore
combination with
immunosuppressive drugs
cyclophosphamide &
methotrexate is helpful. Relapse
is common.
54. HENOCH SCHONLEIN PURPURA(HSP)
is a small vessel vasculitis
affecting children & young
adults. Classical clinical triad
of palpable purpura at
buttocks, arthritis&
abdominal pain is found in
80% of patients.
55. HENOCH SCHONLEIN PURPURA(HSP)
Fever & glomerulonephritis are
also common. Affected vessels have
IG &complement deposition&
serum IgA is often elevated. IgA
deposition in renal lesions is
characteristic. Diagnosis is
confirmed depending on clinical &
laboratory findings.
56.
57.
Although patients with HSP
may develop bowel
perforation or significant
renal disease, the majority
improve without sequelae.
Most patients improve within
weeks or months.
58.
Ddx: drug induced purpura, SLE,
gonococcal arthralgia, keratoderma
blennorhagica& secondary syphilis.
Treatment is supportive with NSAIDs
& analgesia.
Steroids are kept for more symptomatic
patients. Those with chronic renal
diseases require steroids &
immunosuppressive drugs.
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