This document provides an overview of vasculitis, including classification, pathophysiology, clinical manifestations, investigations, and management approaches. It discusses several specific large vessel vasculitides - giant cell arteritis, Takayasu arteritis, and polyarteritis nodosa. Giant cell arteritis commonly involves temporal arteries and causes headaches. Takayasu arteritis primarily affects the aorta and its branches. Polyarteritis nodosa preferentially involves the skin, nerves, gastrointestinal tract and kidneys. Diagnosis relies on tissue biopsy and imaging. Treatment focuses on glucocorticoids, with additional immunosuppressants for severe or refractory disease.

1. Under supervision of : Dr.Assad Mubarak
By :

2. 1-Overview & approach to vasculitis…..by (wafaa shiker)
2-Larger vessels vasculitis……..by (mohammed abdulbast)
3-Middle vessels vasculitis …..by (ahmed salahdeen)
4- Small vessels vasculitis……..by (haneen hammed)
5- Cases and questions ………..by (mohammed abdulbast )

3. Introduction
Vasculitis : is a clinicopathologic process characterized by inflammation of and damage to blood vessels
 The vessel lumen is usually compromised, and this is associated with ischemia of corresponding organs.
 Different patterns of vessels involvement in different entities.
 These are a heterogeneous group of diseases characterized by inflammation and necrosis of blood
vessel walls, with associated damage to skin, kidney, lung, heart, brain and gastrointestinal tract.
 There is a wide spectrum of involvement and disease severity, ranging from mild and transient disease
affecting only the skin, to lifethreatening fulminant disease with multiple organ failure

4. PATHOPHYSIOLOGY AND PATHOGENESIS
 3 main groups of pathogenetic mechanisms behind vasculitis
1. immune complex formation
2. antineutrophil cytoplasmic antibodies (ANCA) mediated.
3. pathogenic T lymphocyte responses and granuloma
formation
some of vasculitis cases remain of unknown causes.

5. immune complex formation
 antigen-antibody complexes are formed in antigen excess and
are deposited in vessel walls
 . The deposition of complexes results in activation of
complement components, particularly C5a, which is strongly
chemotactic for neutrophils.
 These cells then infiltrate the vessel wall, phagocytose the
immune complexes and release their intracytoplasmic
enzymes, which damage the vessel wall
 The actual antigen contained in the immune complex has only
rarely been identified
 hepatitis B virus antigen has been identified in polyarteritis
nodosa
 hepatitis C virus antigen has been identified in the
Cryoglobulinemic vasculitis

6. immune complex formation

7. (ANCA) mediated mechanism
ANCA are antibodies directed against certain proteins in the cytoplasmic
granules of neutrophils and monocytes.
 These autoantibodies are present in a high percentage of patients with
(Wegener’s granulomatosis) and (microscopic polyangiitis)
 lower percentage of patients with eosinophilic granulomatosis with (Churg-
Strauss).
 they called ANCA-associated vasculitis.
 However, as these diseases possess unique clinical phenotypes in which ANCA
may be absent,
 The absolute height of the antibody titers does not correlate well with
disease activity.

9. Pathogenic T Lymphocyte Responses And
Granuloma Formation
 Vascular endothelial cells can express HLA class II
molecules following activation by cytokines such as
interferon (IFN) γ. This allows these cells to participate
in immunologic reactions such as interaction with
CD4+ T lymphocytes in a manner similar to antigen-
presenting macrophages.
 Endothelial cells can secrete IL-1, which may activate
T lymphocytes and initiate or propagate immunologic
processes within the blood vessel.

11. Classification
 Classification of Vasculitis :
No universally accepted classification but can be classifed according to
 Systemic
 Vessel size
 Histopathology
 Dominant organ involvement
 Etiology

12. Classification
Etiological classification of Vasculitis:
Primary or Secondary?
 Primary: Vasculitis is the principal feature
of the disease
 Secondary: Vasculitis is a complication
of another disease or toxin (e.g. RA,
infection, malignancy)

15. According To size and pathology

17. Clinical manifestation
 The clinical features result from a combination of local tissue ischaemia
(due to vessel inflammation and narrowing) and the systemic effects of
widespread inflammation.
 Systemic vasculitis should be considered in any patient with fever, weight
loss, fatigue, evidence of multisystem involvement, rashes, raised
inflammatory markers and abnormal urinalysis

19. Approach to vasculitis
Step 1 : When suspect vasculitis by recognize the common feature
of highly suggestive vasculitis include:
 palpable purpura,
 pulmonary infiltrates and microscopic hematuria
 chronic inflammatory sinusitis
 mononeuritis multiplex
 unexplained ischemic events
 glomerulonephritis with evidence of multisystem disease.

21. Step 2 : to exclude other diseases that produce clinical
manifestations that can mimic vasculitis
Conditions That Can Mimic Vasculitis includes :
 Infectious diseases
 Bacterial endocarditis
 Disseminated gonococcal infection
 Pulmonary histoplasmosis
 Coccidioidomycosis
 Syphilis
 Lyme disease
 Rocky Mountain spotted fever
 Whipple’s disease

22.  Coagulopathies/thrombotic microangiopathies
 Antiphospholipid syndrome
 Thrombotic thrombocytopenic purpura
 Neoplasms
 Atrial myxoma
 Lymphoma
 Carcinomatosis
 Drug toxicity
 Cocaine , Levamisole , Amphetamines, Ergot alkaloids, Methysergide , Arsenic
 Sarcoidosis
 Atheroembolic disease
 Antiglomerular basement membrane disease
(Goodpasture’s syndrome)
 Amyloidosis
 Migraine
 Reversible cerebral vasoconstrictive syndrome

23. Step 3 : Establishing the diagnosis of vasculitis : usually
made based on biopsy of involved tissue and/or
arteriogram

24. Step 4 : determine the pattern of vessel involvement :
(large vessel, medium vessel, small vessels)

25. Step 5 : treat vasculitis and underlying cause

26. Large vessels vasculitis
1- GIANT CELL ARTERITIS AND POLYMYALGIA RHEUMATICA
2- TAKAYASU ARTERITIS

27. GIANT CELL ARTERITIS AND
POLYMYALGIA RHEUMATICA
 Definition : Giant cell arteritis, historically referred to as
temporal arteritis, is an inflammation of medium- and
large-sized arteries.
It characteristically
 involves one or more branches of the carotid artery,
particularly the temporal artery.
 However, it is a systemic disease that can involve arteries
in multiple locations, particularly the aorta and its main
branches.

28. What is the association between PMR and
GCA? Giant cell arteritis is closely associated with polymyalgia
rheumatica, which is characterized by
stiffness, aching, and pain & weakness in the muscles of the neck,
shoulders, lower back, hips, and thighs.
 Most commonly, polymyalgia rheumatica occurs in isolation, but it
may be seen in 40–50% of patients with giant cell arteritis.
In addition, ∼10–20% of patients who initially present with features
of isolated polymyalgia rheumatica later go on to develop giant cell
arteritis.
 This strong clinical association together with data from
pathophysiologic studies has increasingly supported that giant cell
arteritis and polymyalgia rheumatica represent differing clinical
spectrums of a single disease process.

29. PATHOLOGY AND PATHOGENESIS Histopathologically, the disease is a panarteritis with
inflammatory mononuclear cell infiltrates within the vessel
wall with frequent giant cell formation.
 There is proliferation of the intima and fragmentation of
the internal elastic lamina.
Pathophysiologic findings in organs result from the
ischemia related to the involved vessels
1- 70-80% their will be true granuloma ( giant cell around
the external lamina )
2- 20-30 % atypical granuloma mononuclear cell around
the media

30. SYMPTOMS

31. SYMPTOMS & SIGN Giant cell arteritis is most commonly characterized
clinically by: the complex of
1- fever
2-anemia(PALLOR)
3-headaches in a patient over the age of 50 years.
Other phenotypic manifestations include features of
systemic inflammation including malaise, fatigue,
anorexia,weight loss, sweats, arthralgias, polymyalgia
rheumatica.

32. SYMPTOMS & SIGNIn patients with involvement of the cranial arteries, headache is
the predominant symptom and may be associated with a tender,
thickened,
or nodular artery, which may pulsate early in the disease but
may become occluded later.
Scalp pain and claudication of the jaw and tongue may occur if
maxillary & fascial artery are involved .
A well-recognized and dreaded complication of
giant cell arteritis, particularly in untreated patients, is
ischemic optic neuropathy, which may lead to serious visual
symptoms, even sudden blindness in some patients.
tip:- mono neuritis muliplex ???

33. SYMPTOMS & SIGN
Manifestations of large-vessel disease can
 include subclavian artery stenosis that can present as
arm claudication
 or aortic aneurysms involving the thoracic and
to a lesser degree the
 abdominal aorta, which carry risks of rupture or
dissection
and physical examination will show diminished pulses or
bruits.

35. Just to remember

36. Investigation Characteristic laboratory findings
1-elevated ESR and CRP
2- normochromic or slightly hypochromic
anemia.
3- Levels of enzymes indicative of muscle
damage such as serum creatine kinase are not
elevated.
 The diagnosis is confirmed by multiple
biopsy's of the temporal artery.

38. D.Ultrasonography of the temporal artery

39. In this regard, it has been reported that temporal artery biopsies may show
vasculitis even after ∼14 days of glucocorticoid therapy. A dramatic clinical
response to a trial of glucocorticoid therapy can further support the
diagnosis.
 Large-vessel disease may be confirmed by vascular imaging, most
commonly through magnetic resonance or computed tomography.

40. Management
 Acute disease-related mortality directly from giant cell arteritis is very
uncommon, with fatalities occurring from cerebrovascular events or
myocardial infarction. However, patients are at risk of late mortality
from aortic aneurysm rupture or dissection as patients with giant cell
arteritis are 18 times more likely to develop thoracic aortic aneurysms than
the general population.
 The goals of treatment in giant cell arteritis are
 1-to reduce symptoms and,
 2- most importantly, to prevent visual loss.

41. Management1- Giant cell arteritis and its associated symptoms are
exquisitely sensitive to glucocorticoid therapy.
Treatment should begin with prednisone,
40–60 mg/d for ∼1 month, followed by a
gradual tapering.
2- When ocular signs and symptoms occur, consideration
should be given for the use of methylprednisolone 1000 mg
daily for 3 days to protect remaining vision.
The ESR can serve as a useful indicator of inflammatory disease
activity in monitoring and tapering therapy and can be used to
judge the pace of the tapering schedule.

42. Management Aspirin 81 mg daily has been found to reduce the
occurrence of cranial ischemic complications in giant
cell arteritis and should be given in addition to
glucocorticoids in patients who do not have
contraindications
 Both methotrexate as a glucocorticoid-sparing agent
and Infliximab has no rule in Mx

43. TAKAYASU ARTERITIS DEFINITION: Takayasu arteritis (pulseless disease) is
an inflammatory and stenotic disease of medium and
large-sized arteries characterized by a strong
predilection for the aortic arch and its branches .
INCIDENCE AND PREVALENCE
 Takayasu arteritis is an uncommon disease with an
estimated annual incidence rate of 1.2–2.6 cases per
million. It is most prevalent in adolescent girls and
young women.

45. PATHOLOGY AND PATHOGENESIS The involvement of the major branches of the aorta is
much more marked at their origin than distally.
The disease is a panarteritis with inflammatory
mononuclear cell infiltrates and occasionally giant cells.
There are marked intimal proliferation and fibrosis,
scarring and vascularization of the media, and
disruption and degeneration of the elastic lamina.
Narrowing of the lumen occurs with or without
thrombosis.

46. CLINICAL AND LABORATORY
MANIFESTATIONS
 Takayasu arteritis is a systemic disease with generalized
as well as vascular symptoms.
 The generalized symptoms include malaise, fever, night
sweats, arthralgias, anorexia, and weight loss, which may
occur months before vessel involvement is apparent &
some cases may be asymptomatic.

Takayasu arteritis should be suspected strongly in a
young woman who develops a decrease or absence of
peripheral pulses, discrepancies in blood pressure, and
arterial bruits in the involved vessels, particularly the
subclavian artery.

48. Investigation The diagnosis is confirmed by arteriography,
 which includes irregular vessel walls, stenosis,
poststenotic dilation, aneurysm formation, occlusion,
and evidence of increased collateral circulation.

51. Prognosis & ManagementDisease-related mortality :
 most often occurs from congestive heart failure,
cerebrovascular events, myocardial infarction, aneurysm
rupture, or renal failure.
 Even in the absence of life-threatening disease, Takayasu
arteritis can be associated with significant morbidity.
 The course of the diseases variable, and although
spontaneous remissions may occur, Takayasu arteritis is
most often chronic and relapsing.
 Although glucocorticoid therapy in doses of 40–60 mg
prednisone per day alleviates symptoms.
.

52. Medium-Vessel Vasculitides

53. • Medium-Vessel Vasculitides
1. Polyarteritis nodosa
2. Buerger’s Disease
3. Kawasaki Disease

54.  Several different pathophysiologic mechanisms are operative among the
medium- and small-vessel vasculitides.
1. Immune Complex–Mediated Vascular Injury
2. Role of ANCAs
3. Antiendothelial Cell Antibodies
4. Superantigen Model

55. Polyarteritis Nodosa
 Polyarteritis nodosa has a striking predilection for certain organs,
particularly:-
1. Skin
2. peripheral nerves
3. gastrointestinal tract
4. Kidneys

56. Signs and symptoms

57. Symptoms
This disease usually begins with
nonspecific symptoms like:-
• malaise,
• fatigue,
• fever,
• myalgias,
• and arthralgias

59. Signs
Skin lesions of polyarteritis nodosa

60.  A majority of patients with polyarteritis nodosa (>80% in some series)
have vasculitic neuropathy (symmetrical and affects both sensery and
motor function )

61.  The classic gastrointestinal manifestation of polyarteritis nodosa is
“intestinal angina,”
 It can also affect individual gastrointestinal tract organs such as the
 gallbladder
 or appendix,
So presenting as cholecystitis or
appendicitis

62.  The typical renal manifestation of polyarteritis nodosa is :-
1. renin-mediated hypertension
2. renal infarctions.

64.  Cardiac lesions, which usually remain subclinical, may lead to myocardial
infarction or congestive heart failure.
 Polyarteritis nodosa usually spares the lungs.

65. Diagnosis
The diagnosis of polyarteritis nodosa
requires either
• a tissue biopsy
• an angiogram that demonstrates
microaneurysms

67.  nerve and muscle biopsies (e.g., sural nerve and
gastrocnemius muscle) have a high yield if there is
clinical suspicion of vasculitic neuropathy.
 Symptoms suggestive of a neuropathy can be confirmed
by electrodiagnostic studies that demonstrate a
sensorimotor axonal neuropathy, often in a
mononeuritis multiplex pattern

68. Treatment
 Approximately half of patients with polyarteritis nodosa
achieve remissions or cures with high doses of
glucocorticoids alone.
 Cyclophosphamide (2 mg/kg/day, adjusted for renal
dysfunction) is indicated for patients whose disease is
refractory to glucocorticoids or who have serious involvement
of major organs
 In recent years, therapeutic regimens involving lamivudine or
entecavir and plasma exchange have substantially improved
the treatment of hepatitis B virus (HBV)–associated
polyarteritis nodosa

70. Buerger’s Disease
 Buerger’s disease, also known as thromboangiitis obliterans
 strong association with cigarette smoking.
 Buerger’s disease does not occur in the absence of exposure to tobacco.
 The vessels affected by Buerger’s disease are the distal medium-sized
arteries and veins, particularly vessels at the levels of the ankles and wrists
 . The disease is characterized by thrombotic obliterations that begin
distally and proceed proximally.
 Arterial obliteration leads to the development of collateral vessels with a
“corkscrew” appearance on angiography.

72.  Vascular occlusion in Buerger’s disease often leads to the loss of digits and,
if smoking persists, to the loss of greater amounts of tissue (e.g., hands or
feet).
 Despite the intense involvement of the extremities in Buerger’s disease,
internal organ disease almost never occurs.

74.  Complete abstinence from tobacco is essential to the treatment of Buerg-
er’s disease. Failure to stop smoking is associated with a dramatic increase
in the risk of limb loss by amputation.
 No other therapeutic interventions, including glucocorticoids and
anticoagulation, have dramatic effects on Buerger’s disease.

77. Kawasaki Disease
• Kawasaki disease occurs exclusively in young
children.
• Because of its striking mucocutaneous findings
and lymphadenopathy, Kawasaki disease is also
known as mucocutaneous lymph node
syndrome

78.  Features of Kawasaki disease include
1. high fevers,
2. cervical adenopathy,
3. conjunctival congestion,
4. buccal erythema, prominence of the tongue papillae (“strawberry
tongue”),
5. polymorphous truncal rash,
6. erythema of the palms and soles, and desquamation of skin from the
fingertips

80.  In a small number of patients with Kawasaki disease, panvasculitis in the
coronary vessels leads to acute cardiac complications. Coronary arteritis
leads to narrowing of the vessel lumen by the migration of myointimal
cells from the media through the fragmented internal elastic lamina
 Direct complications include aneurysmal dilation and thrombosis of the
coronary arteries
 Late mortality from myocardial infarction may occur from the thrombosis
of coronary artery aneurysms formed during the initial inflammatory
stage.

81. Daignosis
• By sign and symptoms
• lab test

82.  The recommended therapeutic regimen in Kawasaki disease is the
combination of intravenous immune globulin (IVIG; 400 mg/kg/day on 4
consecutive days) and acetylsalicylic acid (100 mg/kg/day, lowered to 3 to
5 mg/kg/day after resolution of the fever). IVIG prevents the formation of
coronary aneurysms in most cases.
 Glucocorticoids are reserved for salvage therapy in patients in whom
treatment with IVIG and acetylsalicylic acid has failed. 3

85. Antineutrophil cytoplasmic antibody
(ANCA)associated vasculitis is a life-
threatening disorder characterised by:
inflammatory infiltration of small blood
vessels
 fibrinoid necrosis
And the presence of circulating
antibodies to ANCA.
The combined incidence is
about 10– 15/1 000 000.

86. Granulomatosis with
polyangiitis
(Wegener’s granulomatosis
(WG)
The vasculitis is characterized by
granuloma formation, mainly
affecting the nasal passages, airways
and kidney.
A minority of patients present with

87. The most common
presentation of WG is with:
Patients often present with severe upper
respiratory tract findings such as :
 paranasal sinus pain
 Purulent or bloody nasal discharge
with or without nasal mucosal ulceration .
 Nasal septalperforation may follow,
leading to saddle nose deformity
Epistaxi
s
saddle-nose
deformity

88. Ears:
conductive hearing
loss due to auditory
tube dysfunction
sensorineural hearing
loss (unclear
mechanism)

89. Eye:
o Pseudotumor:
o proptosis may occur because of
inflammation of the retroorbital
tissue.
o This causes diplopia due to
entrapment of the extraocular
muscles, or loss of vision due to
o optic nerve compression.
o Disturbance of colour vision is an
early feature of optic nerve
compression.
o scleritis, conjunctivitis, uveitis,
episcleritis

90. Oral cavity:
 strawberry gingivitis
 underlying bone destruction
with loosening of teeth
 non-specific ulcerations
 throughout oral mucosa.

91. Trachea:
subglottal stenosis
Lungs:
pulmonary haemorrhage
causing haemoptysis, and
rarely bronchial stenosis.
pulmonary nodules (referred
to as "coin lesions"),
infiltrates (often interpreted
as pneumonia), cavitary
lesions.

92. Skin:
 subcutaneous nodules
(granulomas) on the elbow
 purpura .
Kidney:
rapidly progressive glomerulonephritis
(75%), leading to chronic kidney
failure.
Nervous system:
occasionally sensory neuropathy (10%)
and rarely mononeuritis multiplex.

93. Diagnosis:
Patients with active disease usually:
have a leucocytosis
with an elevated CRP and ESR
in association with raised ANCA levels.
proteinase3 (PR3) antibody positive.
Complement levels are usually normal
or slightly elevated.

94. Imaging of the upper airways or
chest with MRI can be useful in
localising abnormalities but,
where possible, the diagnosis
should be confirmed by biopsy
of the kidney or lesions in the
sinuses and upper airways.

95. Microscopic polyangiitis
(MPA)
Is a necrotising small vessel vasculitis
found with rapidly progressive
glomerulonephritis, often in
association with alveolar haemorrhage.
Cutaneous and gastrointestinal
involvement is common and other
features include neuropathy (15%) and
pleural effusions (15%).

96. Signs and symptoms
Clinical features may include constitutional
symptoms like :

97. Glomerulonephritis occurs in
at least 79% of patients and
can be rapidly progressive,
leading to renal failure.
Hemoptysis may be the first
symptom of alveolar
hemorrhage, which occurs in
12% of patients.

98. Because many different
organ systems may be
involved, a wide range of
symptoms are possible in
MPA.
Purpura and livedo
racemosa may be present.
Other manifestations
include mononeuritis
multiplex.

99. Diagnosis
Laboratory tests may reveal :
leukocytosis
an increased sedimentation
rate ESR
elevated CRP
anemia and elevated creatinine
due to kidney impairment.
and protein and red blood cells
in the urine.

100. An important diagnostic test is the presence of
perinuclear antineutrophil cytoplasmic
antibodies (p-ANCA) with myeloperoxidase
specificity
(a constituent of neutrophil granules).
In patients with neuropathy, electromyography
may reveal a sensorimotor peripheral neuropathy.
Differential diagnosis
Upper airway disease and pulmonary nodules are
not typically found in microscopic polyangiitis
and , if present, suggest granulomatosis with
polyangiitis (Wegener’s).

101. Churg–Strauss syndrome
Churg–Strauss syndrome (CSS) is a small vessel vasculitis
with an incidence of about 1–3 per 1 000 000.
Eosinophilic granulomatosis with polyangiitis (Churg-
Strauss) was described in 1951 by Churg and Strauss and
is characterized by asthma, peripheral and tissue
eosinophilia , extravascular granuloma formation, and
vasculitis of multiple organ systems.

102. Some patients have a prodromal
period for many years,
characterized by :
allergic rhinitis
nasal polyposis
and late onset asthma that is often difficult to
control.

103. The typical acute presentation is
with a triad of:
 skin lesions (purpura or nodules)
asymmetric mononeuritis multiplex
eosinophilia.
Pulmonary infiltrates and pleural or
pericardial effusions due to serositis may
be present.
Up to 50% of patients have abdominal
symptoms provoked by mesenteric
vasculitis.

104. Diagnosis:
Patients with active disease have:
raised levels of ESR and CRP and an
eosinophilia.
Although antibodies to MPO or PR3 can
be detected in up to 60% of cases, CSS is
considered to be a distinct disorder from
the other ANCA associated vasculitides.

105. Biopsy of an affected site reveals a
smallvessel vasculitis with
eosinophilic infiltration of the vessel
wall .
In order to be diagnosed with
(Churg-Strauss), a patient should
have evidence of asthma a, peripheral
blood eosinophilia , and clinical
features consistent with vasculitis.

106. Management of MPA, WG and CSS
Treatment should be instituted as early as
possible to prevent irreversible damage, even in
advance of biopsy confirmation if there is life-
threatening or critical organ involvement.
Remission can be induced either with oral high-
dose prednisolone (1 mg/kg daily) and
continuous oral cyclophosphamide (2 mg/kg
daily) or with bolus i.v. methylprednisolone (10
mg/kg) and cyclophosphamide (15 mg/kg),
monthly.

107. Once remission has been induced (3-6 months) the
dose of oral prednisolone is rapidly reduced and
cyclophosphamide is usually replaced with
azathioprine.
Co-trimoxazole is usually given at a prophylactic
dose (960 mg thrice weekly) in conjunction with
cyclophosphamide to prevent Pneumocystis
pneumonia, unless there is a history of drug
allergy.
Mesna is used with bolus cyclophosphamide to
reduce the risks of haemorrhagic cystitis.

108. Behçet’s syndrome
This is a vasculitis of unknown aetiology
that characteristically targets small
arteries and venules.
It is rare in Western Europe but more
common in ‘Silk Route’ countries around
the Mediterranean and Japan, where there
is a strong association with HLAB51.

109. Oral ulcers are universal.
Unlike aphthous ulcers, they are
usually deep and multiple, and
last for 10–30 days.
Genital ulcers are also a common
problem, occurring in 60–80% of
cases.

110. The usual skin lesions are
:

111. Ocular involvement is common and may
include:
Eye :
 anterior or posterior uveitis or retinal vasculitis.
Neurological involvement :
occurs in 5% and mainly involves :
the brainstem
although the meninges, hemispheres and cord can also be affected
causing pyramidal signs, cranial nerve lesions, brainstem
symptoms or hemiparesis.
Recurrent thromboses also occur.
Renal involvement is extremely rare.

112. Criteria for diagnosis of Behçet’s syndrome

113. The diagnosis is primarily made on clinical grounds, but
one characteristic feature that can be of diagnostic value
is the :

114. Treatment
Oral ulceration can be managed with topical
steroid preparations (soluble prednisolone
mouthwashes, steroid pastes).
Colchicine can be effective for erythema
nodosum and arthralgia.
Thalidomide (100–300 mg per day for 28 days
initially) is very effective for resistant oral and
genital ulceration but is teratogenic and
neurotoxic.
Steroids and immunosuppressants are
indicated for uveitis and neurological disease.

115. Henoch–Schönlein purpura
Henoch–Schönlein purpura (HSP) is a small vessel
vasculitis caused by immune complex deposition
following an infectious trigger.
It is predominantly a disease of children and young
adults

116. Nephritis can also occur and may
present up to 4 weeks after the
onset of other symptoms.

117. Diagnosis :
Biopsy of affected
tissue shows a
vasculitis with IgA
deposits in the
vessel wall.

118. Treatment
HSP is usually a self-limiting
disorder Corticosteroids and
immunosuppressive therapy may
be required in patients with more
severe disease, particularly in the
presence of nephritis.

119. Cryoglobulinemic
vasculitis
This is a small vessel
vasculitis
that can develop in some
patients with circulating
cryoglobulins,
which are
immunoglobulins that
precipitate out in the cold.

120. Cryoglobulins are classified into three types
and types II and III are associated with
vasculitis
•Type I Cryoglobulinemia: caused by monoclonal
immunoglobulin. This type is usually related to cancerous
conditions of the blood or the immune systems.
•Types II Cryoglobulinemia: caused by IgM, IgG and IgA
monoclonal immunoglobulin. These types of Cryoglobulinemia
are most commonly found in people who have chronic
inflammatory conditions like hepatitis C.
•Types III Cryoglobulinemia: caused by IgM and IgG
polyclonal immunoglobulin.

121. The typical
presentation is with:
a vasculitic rash over the
lower limbs
Arthralgia
Raynaud’s phenomenon
and neuropathy.
Some cases are secondary
to hepatitis infection and
others are secondary to
other autoimmune
diseases.

122. Affected patients
should be screened
for evidence of
hepatitis B and C
infection, and if the
results are positive,
these should be
treated appropriately
.

123. Treatment
There is no consensus as to how
best to treat cryoglobulinaemic
vasculitis in the absence of an
obvious trigger.
Corticosteroids and
immunosuppressive therapy are
often used empirically but their
efficacy is uncertain.

125. The systemic vasculitis is wide spectrum
disease associated with signs and symptoms of
chronic inflammation.
Vasculitis can affect virtually any organ system;
many of these diseases have typical patterns of
involvement that are recognizable by
experienced clinicians.
The diagnostic value of a biopsy, performed
early in the course of illness, cannot be
overstated.
Drugs used to treat vasculitis depend on the
severity of the clinical manifestations.

126. Case 1
• Case A previously healthy 22yo male medical student had an
URI 2 weeks ago, RX with Augmentin
• He develops abdominal pain, bilateral ankle pain & swelling
with raised purpuric lesions over lower extremities
• Labs:
– creatinine 3.0 mg/dL, BUN 50 mg/dL
– Urinalysis: 4+ proteinuria, 2+ RBC’s, serval RBC casts/ hpf

127. CASE 1
• What is the most likely cause of renal disease & purpura in this
patient ?

128. Case 2
• A 55 yo woman c/o headache for the past 8 days and notes the
onset of double vision and blurring, lasting 15min before
resolving. She has lost 5 kg over the past 2 months with no Hx
of migraine .
• Which is the best next step in her management ?
• What is the most accurate test to diagnosis ?

129. Case 3
• A 27 yo Asian woman work as house keeper in
Kirkuk presents with a 7 kg weight loss, low-grade
fevers and arthralgia's.
• She notes pain in her arms with any prolonged
activity.
• Recent labs are notable for an ESR of 130.
her vital sign :HR100 bpm
her BP is 60/40 mmhg in the left arm
And in the right arm is 80/60 mmhg
• What is the next step in her management?
• What's the most likely diagnosis ?

130. Case 4
• A 34 yo Paramedic staff presents with a 2 yr h/o skin
ulcers on his lower extremities.
• A previous punch biopsy in dermatology clinic showed
thrombotic lesions in small blood vessels of the dermis.
• Treatment has mostly focused on wound care.
• On exam the ulcers are noted, as is livedo reticularis, a
decreased right hand grip, and a right foot drop.
Lab: WBC 12 (with neutrophilia)
• What is the probable diagnosis?

131. Case 4

132. Case 5
• 50yo woman presents with 1 week of fever, chills, chest pain,
cough, dyspnea and paresthesias in Lower extrimites .
• PMHx: bronchial asthma (7 yrs), allergic rhinitis
• PE: 36C , HR 98, BP 120/70, RR 16/min
– wheezes/ rhonchi bilaterally
– mild peripheral weakness in LE
– sensory dysesthesia in stocking distribution bilaterally
Lab: WBC 12.8 (N 30%, L 25%, Eos 40%)
Blood/sputum cultures: negative
ANA neg, p-ANCA + 1:20
CXR: patchy bilateral infiltrates
Ct scan :multiple, bilateral, and cavitary infiltrates

133. Case 5
• She is treated with empiric antibiotics over 3 days with no improvement in
symptoms.
• What is the most likely diagnosis?