VASCULITIS INTRODUCTION.
TYPES OF VASCULITIS
DIAGNOSING CRIERIA
TREATMENT AND GUIDELINES
DRUGS USED IN BURGER'S DISEASE
A CASE ON VASCULITIS
APPROACH OF TREATMENT
PATIENT COUNSELLING FOR THE PARTICULAR PATIENT
2. VASCULITIS :
Vasculitis is a general term for a group of uncommon diseases that feature
inflammation of the blood vessels and generally damage to these blood
vessels occur. The blood vessels of the body are referred to as the
vascular system. The blood vessels are comprised of:
1. arteries- passes oxygen-rich blood to the tissues of the body
2. veins- return oxygen-depleted blood from the tissues to the lungs for
oxygen.
Vasculitis is defined by certain patterns of distribution of blood vessel , its
involvement, particular organ involvement, and laboratory test
abnormalities. As a group, these diseases are referred to as vasculitides.
The word vasculitis is derived from the Latin "vasculum", vessel + "- itis,"
inflammation.
When arteries are inflamed , the condition is also referred to as arteritis.
When the veins are inflamed, it is referred to as venulitis.
3. Comparison of major types of vasculitis
Vasculitis Affected organs Histopathology
Cutaneous small-vessel
vasculitis
Skin, kidneys
Neutrophils, fibrinoid
necrosis
Granulomatosis with
polyangiitis
Nose, lungs, kidneys Neutrophils, giant cells
Eosinophilic granulomatosis
with polyangiitis
Lungs, kidneys, heart, skin Histiocytes, eosinophils
Behçet's disease
Commonly sinuses, brain,
eyes and skin; can affect
other organs such as lungs,
kidneys, joints
Lymphocytes,
macrophages, neutrophils
Kawasaki disease Skin, heart, mouth, eyes
Lymphocytes, endothelial
necrosis
Buerger's disease
Leg arteries and veins
(gangrene)
Neutrophils, granulomas
"Limited" granulomatosis
with polyangiitis vasculitis
Commonly sinuses, brain,
and skin; can affect other
organs such as lungs,
kidneys, joints;
4. The actual cause of these vasculitis diseases is usually not known. However,
immune system abnormality and inflammation of blood vessels are common
features. Each form of vasculitis has its own characteristic pattern of
symptoms, much of which depends on what particular organs are affected.
Factors that may cause vasculitis are:
Infections (such as hepatitis B),
Exposure to chemicals (such as amphetamines and
cocaine),
Medications,
Cancers (such as lymphomas and multiple
myeloma), and
Rheumatic diseases (such as rheumatoid arthritis
and systemic lupus erythematosus).
ANCA antibodies
Tobacco
5. Buerger's disease
Thromboangiitis obliterans, also
known as Buerger, is a recurring
progressive inflammation and
thrombosis (clotting) of small and
medium arteries and veins of the hands
and feet. It is an autoimmune disease
triggered by various factors as
mentioned above among which tobacco
is the leading cause for this disease.
6. Signs and symptoms:
There is a recurrent acute and chronic inflammation and
thrombosis of arteries and veins of the hands and feet.
The main symptom is pain in the affected areas, at rest and
while walking (claudication).
The impaired circulation increases sensitivity to cold.
Peripheral pulses are diminished or absent.
There are color changes in the extremities (from cyanotic
blue to reddish blue)
Skin becomes thin and shiny. Hair growth is reduced.
Ulcerations and gangrene in the extremities are common
complications, often resulting in the need for amputation of
the involved extremity.
7. Pathophysiology:
There are characteristic pathologic findings of acute
inflammation and thrombosis (clotting) of arteries and
veins of the hands and feet (the lower limbs being more
common).
The mechanisms underlying Buerger's disease are still
largely unknown, but smoking and tobacco consumption
are major factors associated with it.
It has been suggested that the various immunological
antigen may trigger an immune response in susceptible
persons which could incite an inflammatory reaction of the
vessel wall.
This eventually leads to vasculitis and ischemic changes
in distal parts of limbs.
8. DIAGNOSIS
Clinical diagnostic criteria generally include the
following:
(1) history of smoking or tobacco abuse;
(2) age of onset less than 45 to 50 years;
(3) intrapopliteal, segmental arterial occlusions with
sparing of the proximal vasculature;
(4) frequent distal upper extremity arterial
involvement (Raynaud's syndrome or digital
ulceration);
(5) superficial phlebitis; and
(6) exclusion of arteriosclerosis, diabetes, true
arteritis, proximal embolic source, and
hypercoagulable states.
9. Erythrocyte sedimentation rate (ESR)
C–reactive protein (CRP)
CT Scan/MRIs
ANCA tests- specific to granulomatous
vasculitis
10. Treatment approach
Medications to dilate blood vessels, improve blood flow or
dissolve blood clots, generally fibrinolytics and
anticoagulants(not well established) are preffered
Intermittent compression of the arms and legs to increase
blood flow to your extremities
Spinal cord stimulation
Cortico-steriods are prescribed for reducing inflammatory
status.
Surgery to cut the nerves to the affected area (surgical
sympathectomy) to control pain and increase blood flow,
although this procedure is controversial and long-term
results haven't been well-studied
Amputation, if infection or gangrene occurs
11. In acute cases, anti-platelet therapy and
heparinization may be beneficial to
prevent progressive thrombosis.
Thrombolytic therapy has been reported
beneficial but yet its uncertain.
Anti-inflammatory are recommended
Infected lesions should be treated with
appropriate antibiotics to control infection.
For noninfected lesions, “benign neglect”
with pain control and local cleansing is
recommended
Sympathectomy, particularly lumbar, has
been used in patients with nonhealing
ischemic lesions, but the results are
unclear.
Under trials:
Three relatively new forms of therapy have
been attempted in patients with refractory
Buerger’s disease over the last decade:
(1) implantation of a spinal cord stimulator;
(2) administration of a prostacyclin
analogue with vasodilatory and platelet
inhibitory properties; and
(3) intramuscular gene transfer of vascular
endothelial growth factor (VEGF).
12. •Name: Mrs. X
•Age: 25 years
•I. P. No.: 21891823
•Department: surgery
•Date of admission: 21/7/2018
•Date of discharge: 24/7/2018
13. PATIENT COMPLAINTS
• Leg swelling,
•Redish colourisation
• Pain in calf muscle
PAST MEDICAL HISTORY
• H/O rt brachial artery
occlusion with middle finger
gangrene
• T.Aspirin 75mg – 7 months
•T.Atorvas 20mg- 7 months
•C. cephalexin-5 days
•T.dilnip 10mg- 2months
•T.prednisolone-2 months
•T.Acton(para) 50Omg
•T. Pregabalin
•T.Amitrypyiline
PRESENT ILLNESS
:h/O lower limb clauduation
PAST MEDICATION HISTORY
14. VITAL SIGNS
• BP: 120/70 mm Hg
•PR: 86 bpm
• Temperature: 98.4° F
• CVS: S1S2 +ve
15. CLINICAL
HEMATOLOGY
Observed value Normal value
Hemoglobin 10.3 12-16 mg/dL
Total leukocyte
count
6900 3.2-9.8×10³/mm³
Neutrophils 71 40-70%
Lymphocytes 22 25-33%
Eosinophils 7 0-6%
PARAMETERS Observed value Normal value
Random BS
UREA
Sr.creatinine
92
21
0.7
<200 mg/Dl
8-21 mg/dl
0.6-1.4 mg/dl
16. •OTHER INVESTIGATION:
CT LOWER LIMB
IMPRESSION-
RLL-filling defect is seen in mid superficial femoral artery for a
segment of 2.9cm. There is a distal reformation with muscular
branches
LLL- Thinning is seen in mid SFA for a segment of 2 cm with
surrounding tortuous vessels, suggestive of chronic oclussion
17. From the above subjective and
objective evidence, the patient is
diagnosed with
Buerger's
19. Clopidogrel+Apixaban
Both increase anticoagulation. Risk of bleeding
•aPTT
REFFERERD TO GH for further treatment with a reference of fem-pop
Surgery to remove the occlusion of lower limb arteries
PHARMACIST INTERVENTION:
1. Fibrinolytic like alteplase and tenecteplase must be considered in
complications if therapeutic treatmment in required.
2. ACR (American college of Rheumatology) does not recommend
prophylactic treatment of Atorvastatin in this type of vasculitis since
arterosclerosis is ruled out.
3.Clopidogrel is generally avoided with aspirin as both the drug together
exposes the patient to severe GI bleeding.
20. PATIENT COUNSELLING
REGARDING DISEASE:
Buerner disease is an immune mediated
vasculitis. It does not have a permanent
therapy but conservative therapy
Improves quality of life in such patients
REGARDING DRUGS
1.APIXABAN : Patient should inform physician if any bleeding occurs( gums,nose
etc)
2. Prednisolone:
These drugs are not subjected to be self-withdrawn if patients symptoms are
decreased, as these may cause flare-ups.
3.Anti-platelet drugs:
Side effects like GI disturbance or bleeding , nausea, vomiting should be checked.
If any dose is missed next dose should be taken only after 12 hrs, it should not be
taken with the next consequent dose,
21. REGARDING LIFESTYLE :
•Unrefined plant foods are preferred, rather than on
animal products or highly refined foods
•Foods higher in dietary fiber must be included.
•Drink plenty of water, prefer olive oil and garlic
22. •Move your feet and calf muscles as an exercise
and avoid heavy workout
•Use compression stockings
•Lift your foot off the floor and make circles in the air
with your toes
•Dressing of wounds properly.
•Not moving around barefoot.