Different types of vasculitis have characteristic patterns of blood vessel involvement.However vasculitis is a systemic illness.The symptoms of vasculitis depend on the particular blood vessels that are involved by the inflammatory process

1. 1/20/2017
GOOD MORNING
GREETINGS FROM
SHARJAH

2. SYSTEMIC VASCULITIS
AN OVERVIEW
DR.K.V.RISHIKESAN MD,Dip.NB
SPECIALIST PHYSICIAN
1/20/2017

3. LEARNING OBJECTIVES
• DEFINE VASCULITIS
• DEFINE TYPICAL FEATURES S/o VASCULITIS
• DESCRIBE THE MODERN CLASSIFICATION SYSTEM
• IDENTIFY A PROBABLE VASCULITIS IN A PATIENT WITH
A TYPICAL PRESENTATION
• TREATMENT OVERVIEW and finally
• TAKE HOME MESSAGE
1/20/2017

4. VASCULITIS
A heterogeneous group of clinical syndromes
characterized by inflammation and necrosis of
blood vessels
Normal Artery
Artery: WBC in media and
adventitia
1/20/2017

5. WHAT IS VASCULITIS?
VASCULITIS IS A DIVERSE CATEGORY OF INFLAMMATORY
DISEASES OF BLOOD VESSELS.
• RANGE IN SEVERITY FROM SELF LIMITED DERMATOLOGIC
CONDITIONS TO ACUTE AND RAPIDLY FATAL MULTISYSTEM
DISEASES.
• ALL FORMS OF VASCULITIS ARE CHARECTERISED BY
ENDOTHELIAL DAMAGE, INTIMAL PROLIFERATION,
THROMBOSIS AND EVENTUAL VASCULAR OCCLUSION.
• VASCULITIS CAN AFFECT EVERY ORGAN SYSTEM.
1/20/2017

6. WHAT IS VASCULITIS?
VASCULITIS IS A DIVERSE CATEGORY OF
INFLAMMATORY DISEASES OF BLOOD
VESSELS.
• RANGE IN SEVERITY FROM SELF LIMITED
DERMATOLOGIC CONDITIONS TO ACUTE
AND RAPIDLY FATAL MULTISYSTEM
DISEASES.
• ALL FORMS OF VASCULITIS ARE
CHARECTERISED BY ENDOTHELIAL
DAMAGE, INTIMAL PROLIFERATION,
THROMBOSIS AND EVENTUAL VASCULAR
OCCLUSION.
• VASCULITIS CAN AFFECT EVERY ORGAN
SYSTEM.
1/20/2017
Can be difficult to
diagnose and may
be life-threatening

7. 1/20/2017

8. 1/20/2017

9. FIBRINOID
NECROSIS
Fibrinoid necrosis is a
form of necrosis, or
tissue death, in which
there is accumulation
of amorphous, basic,
proteinaceous material
in the tissue matrix
with a staining pattern
reminiscent of fibrin.
1/20/2017

10. 1/20/2017
Systemic
Fever, sweats,
weight loss
Skin
Palpable
Purpura
Neurologic
Mononeuritis
Multiplex
Musculoskeletal
Arthralgia /
arthritis
Muscle pain /
claudication
Respiratory
Sinusitis /
Epistaxis
Pulmonary
infiltrates
Gastrointestinal
Abdominal
Pain
Bloody stools
Renal
Glomerulo-
nephritis
Hypertension

11. WHEN SHOULD VASCULITIS BE SUSPECTED?
• MULTISYSTEM inflammatory
disease
• Sign. CONSTITUTIONAL
SYMPTOMS
• RAPIDLY PROGRESSIVE
organ dysfunction
• HIGH ESR
SEVERE anemia
PLATELETS > 500K
• Low serum ALBUMIN
CLINICAL FEATURES
PARTICULARLY S/O
of small vessel inflammation:
• SKIN: palpable purpura
• LUNGS: pulmonary infiltrates /
hemoptysis
• KIDNEY: active urinary sediment
• NEURO: foot drop
1/20/2017

12. TAKE THESE CLUES…..
• Palpable purpura =
small vessel leukocytoclastic
vasculitis
• Hepatitis B PAN
• Wrist/foot drop= PAN
• Hepatitis C =
cryoglobulinemia
• Oral & genital ulcers =
Behcet’s
Upper/lower airway disease and
glomerulonephritis =
Wegener’s
• Septal perforation,
epistaxis
• Recurrent sinus infections
Asthma and eosinophilia=
CSV
• Young female with arm/leg
fatigue and HTN =
Takayasu’s
1/20/2017

13. 1/20/2017
Unexplained ischemia,
such as claudication,
limb ischemia,
angina, transient
ischemic attack, stroke,
mesenteric ischemia
and cutaneous
ischemia, particularly in
a young patient or a
patient without risk
factors for
atherosclerosis
When should
vasculitis be
suspected?

14. CLINICAL FEATURES HIGHLY S/o VASCULITIS
1/20/2017

15. CUTANEOUS MANIFESTATION
Palpable
Purpura
Caused by
extravasation of RBCs
from small vessels that
have been occluded by
immunecomplexes.
They are heavy and tend
to occur in the most
gravity dependent area
of the body
1/20/2017

16. LIVEDO RETICULARIS
All of us develop this
“lacy” vascular pattern
when we are cold.
However livedo
reticularis is caused by
relative ischemia of the
capillary beds.
Vasculitis is one of the
causes of livedo.
1/20/2017

17. SPLINTER HEMORRHAGES
Splinter hemorrhages can be found in the distal nail
bed and/or in the periungual area.
1/20/2017

18. NEUROLOGIC
Mononeuritis multiplex:
check for FOOT DROP
Sural n. biopsy showing vasculitis
Note that the nerve tissue is seen at the
bottom of the picture. The vessel infiltration
with white blood cells occurs in the vasa
nervorum.
nerve tissue
vasa nervorum
1/20/2017

19. RESPIRATORY: UPPER/LOWER
• SINUSITIS
• EPISTAXIS
Or……..
.Pulmonary infiltrates
• Nodules
• Cavities
1/20/2017

20. GENITOURINARY
• Glomerulonephritis
• Hypertension
• Hematuria
• RBC casts
• Testicular pain
(especially PAN)
Cellular and Fibrous Crescent Formation in
glomerulonephritis. Testicular pain is
uncommon in systemic vasculitis, but when it
occurs, it is relatively specific for PAN.
1/20/2017

21. OCULAR
http://www.uveitis.org/images/sa
rcoid6.jpg
Retinal Vasculitis
http://webmedia.unmc.edu/eye/iritis.jpg
Iritis
Scleritis
eyelearn.med.utoronto.ca/.../RedE
ye/10Sclera.htm
1/20/2017

22. GASTROINTESTINAL
• Mesenteric ischemia which
may result in :
• pain 30 minutes after
eating
• bloody diarrhea
• bowel perforation
• hepatitis
• pancreatitis
• cholecystitis
1/20/2017

23. CLASSIFICATION
CONFUSING and UNSATISFYING DUE TO
1.OVERLAPPING PRESENTATIONS
2.MIX OF PRIMARY AND SECONDARY FORMS
3.EVOLVING UNDERSTANDING OF PATHOPHYSIOLOGY
4.EVOLVING CONSENSUS REGARDING USE OF EPONYMS
5.VARIED OPINION ON WHETHER CLASSIFCATION SHOULD BE BASED
PRIMARILY ON SIZE OF AFFECTED VESSELS,OR ON UNDERLYING
PATHOLOGIC PROCESS.
6.DIFFERENT SOURCES MAY PRESENT SLIGHTLY DIFFERENT
CLASSIFICATION SCHEMES
1/20/2017

24. CLASSIFICATION
No universally accepted
classification system.
• Vessel size
• Histopathology
• Dominant organ involvement.
The clinical features of primary
vasculitis syndromes often
OVERLAP, and many patients
do not fit neatly into a well-
defined type of vasculitis
1/20/2017

25. CHAPEL HILL
CLASSIFICATION
Large vessels : Aorta and its
major branches and the
analogous veins.
Medium vessels: The main
visceral arteries , veins and
their initial branches.
Small vessels :
Intraparenchymal arteries,
arterioles, capillaries, venules,
and veins.
The kidney is used to
exemplify medium and small
vessels.
1/20/2017

26. CLASSIFICATION BASED ON Vs.SIZE
• LARGE Vs: GIANT CELL ARTERITIS,
TAKAYASU ARTERITIS.
• MEDIUM Vs: PAN, KAWASAKI DISEASE
• SMALL Vs: ANCA POSITIVE
MICROSCOPIC POLYANGIITIS
WEGENERS, CHURG STRAUSS
ANCA NEGATIVE
HENOCH SCHONLEIN
PURPURA , MIXED ESSENTIAL
CRYOGLOBULINAMIA
1/20/2017

27. CLASSIFICATION
SOME TYPES PREVIOUSLY HAD DIFFERENT
EPONYM BASED NAMES
OLD NAME
• WEGENER’S GRANULOMATOSIS
(WG)
• CHURG STRAUSS VASCULITIS
(CSV)
• HENOCH SCHOENLEIN PURPURA
NEW NAME
GRANULOMATOUS POLYANGIITIS
(GPA)
EOSINOPHILIC GRANULOMATOSIS
WITH POLY ANGIITIS
(EGPA)
IgA VASCULITIS
1/20/2017

28. GENERAL APPROACH TO VASCULITIS
HISTORY: PATIENT’S
STORY
PHYSICAL EXAM: BODY’S
STORY
LABORATORY: BEHIND
THE-SCENES STORY
Basic CBC, CHEMS, LFTs,
UA/micro, CXR
……..MORE PROBLEMS
CREATE PROBLEM LIST………
LIST EVERYTHING [split
don’t lump]
PRIORITIZE PROBLEM
LIST………
WORK the PROBLEM LIST
1/20/2017

29. LABS
• Determine organ involvement
• Exclude other diseases
• Routine labs: CBC, BMP, UA,
ESR, CRP, LFTs
• Infection : cultures, viral
serologies (HBV, HCV, HIV)
• Autoimmune serologies:
ANA, RF, ANCAs, ENA,
ds- DNA, C3/C4
• Misc: CK, anti-GBM, SPEP,
Cryoglobulins
1/20/2017

30. VASCULITIS MIMICS
•Systemic illness – must
exclude alternative
diagnoses:
•Sepsis
•Drug toxicity
•Malignancy
•Coagulopathy
• INFECTIOUS DISEASES
• Endocarditis
• HIV
• DRUGS
• Cocaine
• Methamphetamine
• CHOLESTEROL EMBOLI
• ANTIPHOSPHOLIPID
ANTIBODY SYNDROME
1/20/2017

31. APPROACH TO DIAGNOSIS: ANCAs
• Antibodies directed against neutrophil granule
constituents
• c-ANCA
• Stains cytoplasm (hence “c”)
• Main target antigen: proteinase-3
• Highly specific (>90%) for Wegener’s
• p-ANCA
• Stains perinuclear (hence “p”)
• Main target antigen: myeloperoxidase
• A/w MPA and Churg-Strauss
1/20/2017

32. VASCULITIS: ADDITIONAL TESTING
Imaging studies
Sinus CT scan
Chest CT scan
Mesenteric
angiogram
Tissue biopsy
Temporal artery
Sural nerve
Muscle
Lung
Renal
1/20/2017

33. TISSUE BIOPSY “GO WHERE THE MONEY IS”
Blind biopsy generally low
yield of < 20%.
Vasculitic lesions tend to be
focal and segmental.
If a patient is over age 50 Y.
and presents with a new,
unexplained headache and
elevated ESR, with or without a
tender or abnormal temporal
artery, a temporal artery
biopsy would be indicated.
Similarly, in a patient who
presents with a multisystem
illness and testicular pain and
swelling,
a testicular biopsy should be
considered.
Sural nerve biopsy may be
indicated in a patient with
numbness and tingling in a
lower extremity.
If the urine sediment is
abnormal, a renal biopsy might
be obtained.
1/20/2017

34. ANGIOGRAM
• If biopsy is impractical
• Important in large vessel
vasculitis
• Patient with abdominal
pain
• Renal or mesenteric
vasculitis
If visceral involvement is suspected (and in the absence of a surgical abdomen), a visceral angiogram
to include the celiac, mesenteric, renal and, perhaps, hepatic arteries should be performed .
1/20/2017

35. TEMPORAL ARTERITIS(GCA)
Anaemia of chronic disease.
Blindness (ischemic optic
neuropathy) .
Claudication jaw/tongue
Dramatic response to
steroids
ESR high
Fever, wt. loss
Head ache new onset.
Scalp tenderness
PMR symptoms (proximal
muscle pain)
10% with large vessel
involvement (e.g. subclavian
artery)
1/20/2017

36. GIANT CELL ARTERITIS
ACR CRITERIA (3 OF 5)
• Age > 50
• New onset headache
• ESR (Westergren)  50
• Abnormal artery biopsy
(mononuclear cell infiltrate,
granulomatous inflammation,
usually multinucleated giant cells)
• Temporal artery abnormality
(tender or decreased pulse)
1/20/2017

37. GCA: THERAPY
• Corticosteroids mainstay
of therapy (~1 mg/kg)
• Calcium and vitamin D
• Consider bisphosphonate
• Try to prevent visual loss
with therapy:
Treat, then biopsy!
Necrosis of Intima and media
with disruption of Internal
Elastic Lamina
1/20/2017

38. QUESTIONS
• In the ACR diagnostic criteria
for Giant Cell Arteritis
(Temporal Arteritis), a patient
needs to be greater than what
age?
A. > 40 years
B. > 50 years
C. > 60 years
D. > 70 years
E. > 80 years
B. > 50 YEARS
Almost all are > 60
Average age is 70
1/20/2017

39. TAKAYASU’S
• Large vessel
• Unknown etiology
• Aorta/branches
• “Pulseless Disease”
• Women in reproductive years
• 10X more than men
• Asia, Eastern Europe, Latin America
• Granulomatous Panarteritis: 98%
have stenotic lesions, 27%
aneurysms
• Subclavian & aortic arch most
common, 93%
• 40- 80% renal artery stenosis
1/20/2017

40. TAKAYASU’S
• Arterial stenoses/organ ischemia
• Claudication
• Transient cerebral ischemia/ stroke
• Renal artery hypertension
• CHF
• Angina
• MI
• Mesenteric vascular insufficiency
• In the absence of complications
(retinopathy, HTN, aortic v. insuff), 15 yr
survival 95%
• Most respond to steroids alone
• 40% will need cytotoxics
1/20/2017
Aortogram of a 15-year-old girl with Takayasu
arteritis. Note large aneurysms of descending
aorta and dilatation of innominate artery.

41. POLY ARTERITIS NODOSA
• Necrotizing vasculitis of
medium & small arteries
• Age ~ 40s; M > F
• Constitutional symptoms are
common
• fever 50%
• weight loss 50%
• Vasculitis can be variable in
distribution making diagnosis
difficult
• Mononeuritis multiplex:50%
• Renal involvement: 60%
(renal arteries, interlobular arteries)
• Hypertension (more common)
• Glomerulonephritis (uncommon)
• Abdominal involvement 45%
• Arthralgias/ Myalgias / Myositis 64%
• Testicular pain 25%
• Pulmonary involvement
rare
1/20/2017

42. CASE
• A 29yo woman presents with a 4yr
h/o skin ulcers on her lower
extremities. A previous punch
biopsy 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.
• Which of the following is the most
likely diagnosis:
A. LYMPHOMA WITH A PARANEOPLASTIC
SYNDROME
B. TAKAYASU’S ARTERITIS
C. SLE
D. PAN
E. KAWASAKI DISEASE
1/20/2017

43. PAN : CLINICAL FEATURES
MYALGIA, ARTHRALGIA
FEVER , WT LOSS
PALPABLE PURPURIC RASH,
ULCERATION, INFARCTION AND
LIVEDO RETICULARIS
ARTERITIS OF VASA NERVORUM
LEADS TO A SYMMETRICAL
SENSORY AND MOTOR
NEUROPATHY.
RENAL INFARCTIONS MAY LEAD
TO SEVERE HTN AND RENAL
IMPAIRMENT.
1/20/2017

44. POLY ARTERITIS NODOSA
Hepatitis B (surface
antigen) is risk factor for
PAN.
Classic PAN
is NOT associated with
ANCA
ANCA
1/20/2017

45. KAWASAKI DISEASE
1/20/2017

46. KD : THE MUCOCUTANEOUS LYMPH NODE
SYNDROME
1/20/2017

47. KAWASAKI DISEASE
1/20/2017

48. KAWASAKI DISEASE : F E B Ri L E
Kawasaki disease (KD) is an
acute febrile vasculitic
syndrome of early
childhood that, (although it
has a good prognosis with
treatment) can lead to
death from coronary artery
aneurysm (CAA) in a very
small percentage of
patients.
CRITERIA
• Fever
• Enanthem
• Bulbar conjunctivitis
• Rash
• Internal organ involvement (not
part of the criteria)
• Lymphadenopathy
• Extremity changes
1/20/2017

49. ANCA ASSOCIATED VASCULITIS
• They have been classified together in the recent 2012 Chapel
Hill classification system.
• GPA and MPA have been considered together in clinical and
treatment studies because they share many clinical features.
• Renal involvement is identical in both diseases with a pauci
immune FSGN. Granulomatous involvement, of the upper
respiratory tract, is a characteristic feature of GPA, but not of
MPA
• Eosinophilia, asthma and atopy typically occur in EGPA
1/20/2017

50. TYPES OF ANCA ASSOCIATED VASCULITIDES
There are 3 forms of ANCA-
associated vasculitis.
• Granulomatosis with polyangiitis
(GPA)
• Formerly known as Wegener's
granulomatosis
• Microscopic polyangiitis (MPA)
• Eosinophilic granulomatosis with
polyangiitis (EGPA)
• Formerly known as Churg-
Strauss syndrome
1/20/2017

51. WEGENER’S GRANULOMATOSIS (GPA)
• Necrotizing vasculitis that affects the
small vessels of the respiratory tract
and renal system : PULMONARY-
RENAL SYNDROME
• Age ~ 40s: M > F 2:1
• Sinusitis
• Nasal septal ulceration
• Pneumonitis
• few symptoms until late
• usually no mediastinal
lymphadenopathy
• nodules that can cavitate
ACR CRITERIA (3 OUT OF 5)
1.Nasal or oral inflammation
(oral ulcers or bloody nasal drainage)
2.Abnormal chest radiograph (nodules,
fixed infiltrates, cavities)
3.Urinary sediment
(> 5 RBC/ hpf or casts)
4.Abnormal Biopsy:
showing vasculitis
5.Proteinase-3 antibodies
1/20/2017

52. 1/20/2017

53. WEGENER’S GRANULOMATOSIS (GPA)
A 65yo. woman c/o 6 months of
malaise, 9 lb weight loss, recurrent
sinusitis, and a persistent cough.
On exam, she is afebrile, the mid-
portion of the nasal bridge has a
flattened appearance, and both
sides of the nasal septum are
ulcerated.
RF is positive and ESR is 66.
• Which of the following tests
would be most helpful in
determining the diagnosis:
A. Nasal septum biopsy
B. Chest radiograph
C. Measurement of ANCA
antibodies
D. Sputum culture
E. Measurement of anti-GBM
antibodies
1/20/2017

54. GRANULOMATOUS POLYANGIITIS
WEGENER’S : ANCA
• AntiNeutrophil Cytoplasmic Antibody
• C (cytoplasmic staining) ANCA
• Proteinase 3 (C is the 3rd letter)
• Pulmonary-renal disease
• sensitivity of 95%
• specificity of 95%
• Limited disease…
• lower sensitivity and specificity
1/20/2017

55. GRANULOMATOUS POLYANGIITIS
(GPA) : RX
• Prior to cyclophosphamide, 80-90% mortality at 5 years
• With cyclophosphamide, 5-10% mortality
• Concern about long-term toxicity of PO cyclophosphamide
(bladder especially)
• IV CYTOXAN no significant bladder risk
• Rituximab: very effective for induction & maintenance
• Azathioprine for maintenance
1/20/2017

56. WEGENER’S GRANULOMATOSIS: OUTCOMES
INTERVENTION SURVIVAL
NONE 50% AT 5 MONTHS
GLUCOCORTICOIDS 50% AT 1 YEAR
GCS+CYTOXAN 80% AT 8 YEARS
1/20/2017

57. CHURG-STRAUSS VASCULITIS (EGPA)
• Necrotizing, granulomatous
vasculitis of small arteries and
venules
• Prior asthma
• Allergic rhinitis
• Eosinophilia
• Pulmonary infiltrates.
Classical presentation as a triad
of skin nodules, mononeuritis
multiplex and eosinophilia
• Intra/extravascular
granulomas
• Confusion with Wegener’s
• Nasal/sinus DZ is NON-
destructive
• Pulmonary nodules less
common
• p-ANCA (MPO): 70%
• More responsive to steroids
alone
1/20/2017

58. CHURG STRAUSS
THE BASTARD CHILD
OF WEGENER’S AND
POLYARTERITIS
NODOSA.
CSS SHARES FEATURES
OF BOTH WEGENER’S
AND PAN.
PATIENT WITH CSS
ALWAYS HAS ASTHMA
1/20/2017

59. 1/20/2017

60. CHURG STRAUSS VASCULITIS(EGPA) WEGENERS(WG)
ASTHMA +++ UNCOMMON
EOSINOPHILS +++ OCCASIONAL/MODEST
ATOPY +++ UNCOMMON
UPPER AIRWAY DESTRUCTION :
UNCOMMON
+
PULMONARY NODULES: OCCASIONAL ++
RENAL FAILURE : + ++
ANCA + +
1/20/2017

61. MICROSCOPIC
POLYANGIITIS (MPA)
• CLASSICALLY PRESENTS WITH
RAPIDLY PROGRESSIVE
GLOMERULONEPHRITIS OFTEN
ASSOCIATED WITH
PULMONARY ALVEOLAR
HAEMORRHAGE.
• CUTANEOUS INVOLVEMENT
SIMILAR TO PAN IS COMMON
• PATIENTS ARE USUALLY
p-ANCA POSITIVE
1/20/2017

62. CASE: WHAT IS THE MOST LIKELY CAUSE OF
RENAL DISEASE IN THIS PATIENT ?
• A previously healthy 22yo. male
college student had an URI 2 weeks
ago, RX with PCN
• He develops abdominal pain,
bilateral ankle pain & swelling with
raised purpuric lesions over lower
extremities
• Labs:
• creatinine 3.0 mg/d L, BUN 46 mg/dL
• Urinalysis: 4+ proteinuria, 2+ RBC’s,
• sev. RBC casts/ hpf
1/20/2017

63. HSP MANIFESTATIONS
Renal involvement (10-50%)
• Renal disease more
severe in adults
• Determines prognosis
• Many recover with no
therapy
• Asymptomatic hematuria
 proteinuria & renal
insufficiency (cresentic
GN)
• < 0.5% progress to ESRD
Small vessels, post capillary venules
Palpable purpura, Arthralgias,
GI INVOLVEMENT IN 85%
Abdominal pain, N&V,hemorrhage
Intussusception in children as the major
life threatening complication
Males=females
Mean age 5 yrs.
Preceding URI in 2/3 (1-3 weeks)
Tissue deposition of IgA-containing
immune complexes (skin, kidneys,
bowel)
1/20/2017

64. HSP: IgA VASCULITIS
Usually single episodes
< 4 weeks duration.
Glomerulonephritis
indistinguishable from IgA
nephropathy may occur.
Uncommon:
#Testicular involvement
#Pulmonary hemorrhage
#CNS complications
40% recurrence rate after
period of wellness
Treatment
• Supportive measures
• Corticosteroids for GI
vasculitis and
hemorrhage
• ? CS early in nephritis
1/20/2017

65. CRYOGLOBULINAEMIA
PATTERN OF
ORGAN
INVOLVEMENT:
• Constitutional
• Cutaneous
• Raynaud’s
• Articular
• Vascular
• Neurologic
1/20/2017
Rheumatoid factor
Complement C4 ↓ ↓ ↓
Cryoglobulin (cryocrit)

66. CRYOGLOBULINAEMIA
An immune-complex-mediated
small vessels Vasculitis.
A causative role of hepatitis C
virus in over 80% patients .
Cryoprecipitate Hep C Ag – Ab
Complement fixing:
C4 consumption
C4 levels VERY low
Therapeutic strategies
include
Aetiologic (antiviral),
Pathogenetic
(cyclophosfamide,
rituximab), or
Symptomatic (steroids,
plasmapheresis)
1/20/2017

67. BEHCET DISEASE
Behçet disease:
A rare vasculitic disorder.
Characterized by a triple-symptom
complex of recurrent oral aphthous ulcers,
genital ulcers, and uveitis
AUTOIMMUNE DISEASE.
The pathergy test is helpful but is not
sensitive or specific for the diagnosis of
Behcet disease.
1/20/2017
Oral aphthous ulcers secondary
to Behçet disease.

68. 1/20/2017
RARE VASCULITIS WHICH TARGETS
VENULES.
TREATMENT: CORTICOSTEROIDS,
THALIDOMIDE,
IMMUNOSUPPRESSIVE

69. VASCULITIS: PRINCIPLES OF TREATMENT
ORAL GLUCOCORTICOIDS
IV METHYL PREDNISOLONE
METHOTREXATE
AZATHIOPRINE
CYCLOSPORINE
RITUXIMAB-ANTI CD20Abs
ANTI TNF THERAPIES
INFLIXIMAB
ADALIMUMAB
ETANERCEPT
CERTULIZUMAB
1/20/2017

70. CHOICE OF THERAPY…….
DEPENDS ON :
SEVERITY OF ORGAN DAMAGE
EXTEND OF MULTISYSTEM INVOLVEMENT
THE VASCULAR BED INVOLVED:
(RENAL, OCCULAR,CORONARY.)
GLUCOCORTICOIDS + CYCLOPHOSPHAMIDE PREFERRED
FOR SERIOUS AND SEVERE COMPLICATIONS.
GLUCOCORTICOIDS ALONE WILL SUFFICE FOR ISOLATED MILD
VASCULITIS LIKE IDIOPATHIC CUTANEOUS VASCULITIS
1/20/2017

71. TREATMENT OVERVIEW
LIMITED CUTANEOUS
VASCULITIS
OBSERVATION…
ANTIHISTAMINE….
SHORT COURSE
PREDNISONE.
MILD SYSTEMIC
VASCULITIS
PREDNISONE
RAPIDLY PROGRESSIVE
VASCULITIS
PREDNISONE+
CYTOTOXIC
(CYCLOPHOSPHAMIDE).
CYC. TRANSITIONED TO
LESS TOXIC AGENTS
(AZA, METHOTREXATE)
AFTER REMISSION IS
ACHIEVED.
1/20/2017

72. VASCULITIS: ACTIVITY Vs. DAMAGES
Disease Activity
Disease Damage
1/20/2017
Even after achieving disease remission, patients will
continue to suffer from the chronic, irreversible
consequences of both the disease and its therapies

73. TAKE HOME MESSAGE
• The systemic vasculitides are chronic diseases, usually
autoimmune , characterized by relapse and remission
• Ultimate diagnosis is almost always biopsy
• Achieving remission requires intense monitoring by an expert
multidisciplinary team
• Therapy of choice usually involves corticosteroids and
sometimes an adjunctive cytotoxic
• Pain and fatigue are common consequences independent of
disease activity and generally fail to respond to
immunosuppression
1/20/2017

74. Questions?
1/20/2017