An approach to vasculitis

1. APPROACH TO VASCULITIS
DR. KRISHNA

2. • Vasculitis- Inflammation of blood vessels characterised by leucocytic
infiltration of the vessel walls
• Different patterns of vessels’ involvement indifferent entities
• Vessel lumen compromised--- ischemia of thecorresponding organ

3. Pathogenesis
• main groups of pathogenetic mechanisms behind vasculitis-
1.Immune complex formation
2.ANCA mediated

4. Immune complex formation
• Deposition of immune complexes in the blood vessels activation of
Complements destruction of vessel wall (acute & chronic
inflammation)
• Henoch Schonlein purpura- IgA mediated
• Polyarteritis Nodosa- Hepatitis B ag
• Essential Mixed Cryoglobinemia- Hepatitis C virion

5. ANCA
• Aberrant expression of proteinase 3 and MPO over the surface of the
neutrophils formation of antibodies destruction of
neutrophils vessel wall damage
• P-ANCA (anti-proteinase 3)
• C-ANCA (anti-MPO)
• - Churg Strauss vasculitis
• - Microscopic Polyangiitis
• - Wegener’s granulomatosis

6. Common featuresof cutaneous vasculitis
• Painful palpable purpura
Caliber or involved vessel Morphology of lesion
Small Petechiae
Palpable purura
Macular purpura
Urticarial papules
Vesicles
Targetoid papules and plaque
Medium Livedo reticularis
Retiform purpura
Ulcers
Subcutaneous nodules
Digital necrosis
Large Erythematous or cyanotic skin,purpura ,
ender nodules, ulceration and /or gangrene
Pyoderma gangrenosum like lesion

7. History of a patient with cutaneous vasculitis that
may give clues indicating systemic disease
• Weight loss, fatigue, fever
• Arthralgia, myalgia
• Red eye, eye pain, vision loss
• Nasal or sinus congestion
• Ear pain
• Oral/nasal ulcers
• Chest pain/dyspnoea
• Abdominal pain, blood in faeces
• Blackouts, weakness, fi ts

8. Pattern of purpura Diseases to consider
Pinpoint, cayenne
pepper macular
purpura, typically
<5 mm
Capillaritis, exercise‐induced purpura (’runners’ legs’),
coughing, ligatures
Purpura in contact dermatitis (e.g. rubber), venous
hypertension, suction induced,
cutaneous T‐cell lymphoma
Macular purpura of
any size
Purpura due to infections, platelet disorders and
thrombocytopenia, other clotting disorders, mild
small vessel vasculitis
Trauma/ artefact
Large macular purpura,
typically over 2 cm
Purpura of old age, topical, inhaled or systemic
corticosteroid‐induced purpura, scurvy
Painful palpable
purpura of any size
Cutaneous vasculitis of all types, pityriasis lichenoides,
thrombo‐occlusive disorders of all types
Livedo pattern purpura Antiphospholipid syndromes, vasculitis in medium‐sized
blood vessels (e.g. polyarteritis, ANCA vasculitides),
thrombo‐occlusive disorders of all types including
cryoglobulins, chilblains

9. RULE OUT SECONDARY CAUSES
OF VASCULITIS
- where vasculitis is one of the
clinical manifestations of
the respective disease

10. Association Incidence diesease
Idiopathic 50%
Infection 15-20% Bacterial
Beta-hemolytic streptococci,
especially group A
Mycobacterium leprae
Neisseria meningococcus
Mycobacterium tuberculosis
Atypical mycobacteria
Disseminated fungal infections
(immunocompromised hosts)
Candida
Aspergillus
Fusarium
Mucor
Viral
Upper respiratory tract infection
Hepatitis C > B A, including
vaccines
HIV
Parvovirus B19
Neisseria gonorrhoea
Staphylococcus aureus
Rickettsiae
Gram-negative rods
-Escherichia coli
-Klebsiella
-Pseudomonas

11. Inflammatory
disorders
15-20% Autoimmune connective tissue
diseases
– Rheumatoid arthritis**
– SLE
– Sjögren syndrome
Inflammatory bowel disease
Behçet disease
Hypergammaglobulinemic purpura of Waldenström
Drug
exposure
10-15% Antibiotics
Penicillins
Cephalosporins, esp. cefaclor
Sulfonamides
Minocycline‡
Quinolones
Macrolides
Neoplasms 2-5% Plasma cell dyscrasias
– Monoclonal gammopathies
– Multiple myeloma
Myelodysplasia
Myeloproliferative disorders
Lymphoproliferative disorders
Hairy cell leukemia
Thiazides
Hydralazine‡
Quinidine
Allopurinol
D-penicillamine
G-CSF
NSAIDs
Propylthiouracil
Streptokinase

12. THE PATTERN OF VESSEL
INVOLVEMENT

15. CHARACTERISTIC
PRESENTATIONS OF EACH
VASCULITIS

16. Giant cell arteritis
A/K/A Temporal arteritis
• Elderly persons more than 50 yrs of age
• More common in female
• Headache, tender palpable pulseless artery
• BLINDNESS-most serious complication
• Jaw claudication, Scalp pain, Scalp Tenderness

17. Takayasu’s Arteritis
• Pulseless Disease
• Middle aged females
• Aorta and its branches mainly involved
• Subclavian vessels, Carotid vessels, Mesentricvessels
• Chronic and Relapsing course

18. • Headache, malaise and fever
• Hypertension, pyrexia and pulseless disease are common findings in
children
• erythema nodosum, pyoderma gangrenosum, ulcerated subacute nodular
lesions, papulonecrotic eruptions
• Renal artery stenosis, increased arterial stiffness and increased sensitivity of
the carotid sinus reflex all contribute to the hypertension.

19. • Renal dysfunction,
• Abdominal pain, bleeding or perforation may result from ischaemia
• Involvement of the aortic arch and its branches can lead to the ’aortic arch
syndrome’ with arm claudication, absent radial or brachial pulses (pulseless
disease’)
• Aortic regurgitation, coronary artery ischaemia with angina or myocardial
infarction, pulmonary hypertension, stroke, syncope and visual disturbances
can occur

20. Poly Arteritis Nodosa
• multisystem vasculitis characterized by segmental necrotizing vasculitis that involves
predominantly medium-sized blood vessels.
• 40 and 60 years of age.
• Renal arteries most commonly involved leading to renovascular hypertension
• Pulmonary vessels NEVER involved
• Association with patients of
Hepatitis B
Hairy cell leukemia
streptococcal (especially in children), parvovirus B19, and
HIV,
minocycline.

21. • Cutaneous PAN, a “skin-limited” form of the disease represents about 10% of
PAN
• tender subcutaneous nodules on the lower extremities, livedo
racemosa,retiform purpura ,cutaneous necrosis, and puchedout ulcers are
commonly seen.
• Lesions may resolve with persistent hyperpigmentation, sometimes in a
retiform pattern.
• Cutaneous PAN is the most common form in children, often associated with
streptococcal infections.

23. • classic PAN-- constitutional symptoms, such as weight loss and fever, as well
as multi-organ involvement.
• Common symptoms-- arthralgias, paresthesias ,myalgias, abdominal pain,
and shortness of breath
• Involvement of the kidneys occurs at the level of the interlobar renal
arteries, resulting in renovascular hypertension and renal failure, but not
glomerulonephritis

24. • In male patients, orchitis frequently occurs, especially in association
with HBV infection
• Gastrointestine-- mesenteric ischemia, a poor prognosis
• Microaneurysms of the renal, mesenteric, and/or celiac arteries on
magnetic resonance angiography are highly suggestive of classic PAN.

25. Kawasaki’s Vasculitis
• MucoCutaneous Lymph node syndrome
• Children < 5 years of age mostly
• Desquamative erythematous rashes involving the skin, mucus
membranes, cervical lymphadenopathy
• 25 % develop coronary artery aneurysms in the convalescent stage of
the illness

26. • At least 5 days of fever, irritability, vomiting,anorexia, cough, diarrhoea,
runny nose, weakness and abdominal and joint pain.
• fever is typically spiking and unresponsive to paracetamol.
• There is acral and perianal erythema and acral oedema, bilateral
conjuncitivitis with anterior uveitis, fi ssured lips and a strawberry tongue,
and cervical lymphadenopathy.

27. • febrile stage- 2 weeks
• second phase- 4-6 weeks
• Convalescent phase– upto 3 months
• Incomplete Kawasaki disease should be considered in children who
do not have all the features of the full disease

28. • Deaths may occur due to myocarditis, dysrhythmias, pericarditis, rupture of
aneurysms and occlusion of coronary arteries
• Harada score has been used in some countries as an indication immunoglobulin
therapy
• Four of following seven criteria are needed:
(i) white blood count >12 000/ mm;
(ii) platelet count <35 × 10 4 /mm;
(iii) CRP >3;
(iv) haematocrit<35%;
(v) albumin <3.5 g/dL;
(vi) age <12 months;
(vii) male sex.

29. ANCA Vasculitis
• Usually Pulmonary capillaritis PLUS Glomerulonephritis
• Granulomas +, Asthma + = Churg Strauss
• Granulomas +, NO asthma= Wegener’s
• NO granulomas, NO asthma= Microscopic Polyangiitis

30. Microscopic Polyangiitis
• systemic vasculitis that involves small and medium-sized arteries, with
cutaneous, pulmonary, and renal involvement
• fever, weight loss, arthralgias and myalgias, for months to years
before other symptoms occur.
• Palpable purpura , livedo racemosa , splinter hemorrhages, urticarial
plaques, and ulcers.

32. • Renal involvement occurs in nearly all patients, pauci-immune,
crescentic, necrotizing glomerulonephritis is the primary pathology.
• Pulmonary capillaritis (with dyspnea and pulmonary infiltrates) ,it can
result in diffuse alveolar hemorrhage.
• Neurologic involvement presenting as a peripheral neuropathy or
mononeuritis multiplex

33. Wegener’s Granulomatosis
• Classical triad = URT & LRT
Necrotizing small vessel vasculitis
Pauci-immune glomerulonephritis.
• age of onset- 45–65 years.
• Palpable purpura, oral ulcers.
• Gingival tissue is often red, friable, and hyperplastic (“strawberry gums”).
• Painful subcutaneous nodules and ulcers that resemble pyoderma
gangrenosum
• Papulonecrotic lesions are common and usually occur on the extremities
(particularly the elbows)

35. • The upper or lower respiratory tracts are involved in up to 90% of patients
• Suggestive symptoms signs include recurrent epistaxis, mucosal
ulcerations, nasal septal perforation, and saddle nose deformity.
• Patients with pulmonary involvement typically present with dyspnea,
cough, hemoptysis, or pleuritis, and chest X-rays demonstrate irregular
infiltrates or nodules.
• Renal disease is present in only 20% of patients at presentation, but
eventually develop glomerulonephritis.

36. Existing European criteria (3 of 6 required)
• Upper respiratory tract inflammation;
• Typical radiologic features on chest radiograph or chest CT scan;
• Abnormal urinalysis
• Biopsy-proven granulomatous inflammation;
• Airway stenosis (especially in children);
• Serologic findings (e.g. anti-PR3 ANCAs).

37. Eosinophilic Granulomatosis With Polyangiitis
• Churg Strauss Vasculitis
• distinguished by an association with asthma and eosinophilia.
• mean age at diagnosis is 48 years
• various triggering factors, vaccination, leukotriene inhibitors, and
rapid discontinuation of corticosteroids

38. • The clinical presentation divided into three successive phases:
(1) first phase – symptoms of allergic rhinitis, nasal polyps, and asthma,
which may persist for years;
(2) second phase – peripheral eosinophilia, respiratory tract infections, and
gastrointestinal symptoms;
(3)third phase – systemic necrotizing vasculitis with granulomatous
inflammation,which can occur several years to decades after the initial
symptoms.
• Palpable purpura is seen most commonly, typically on the lower
extremities and often with necrosis.
• Subcutaneous nodules may be seen on the scalp or extremities,
• urticaria, livedo racemosa, and papulonecrotic lesions

39. • Neurologic and cardiac involvement, presenting as mononeuritis
multiplex and cardiomyopathy or pericarditis.
• The cardiac manifestations are due to granulomatous inflammation,
and are the leading cause of death.

40. CUTANEOUS SMALL VESSEL VASCULITIS
• involves primarily the dermal postcapillary venules and is characterized
histologically by LCV.
• often idiopathic in nature, but may be secondary to an underlying cause
such as an infection or medication
• onset is between 36 and 56 years, slight male prepon
• CSVV is a single‐organ vasculitis and therefore by definition does not have
systemic manifestations

41. • The American College of Rheumatology (ACR) classifi cation criteria for
CSVV.
• The presence of three of the following five criteria have 84% specifi city for
CSVV:
(i) age greater than 16 years at disease onset;
(ii) history of taking a medication at onset that may have been a recipitating
factor;
(iii) the presence of palpable purpura;
(iv) the presence of a maculopapular rash;
(v) a biopsy demonstrating granulocytes around an arteriole or venule

42. • crop of lesions consisting of palpable purpura, erythematous papules,
urticarial lesions, or hemorrhagic vesicles that range in size from 1 mm to
several centimeters
• Occasionally pustules, ulcerations, and targetoid lesions are seen.
• CSVV favors dependent areas, as well as areas affected by trauma
(Koebner phenomenon) or under tight-fitting clothing.
• exercise, in particular walking or hiking in hot weather, can induce CSVV on
the lower extremities.
• lesions can be associated with burning, pain, or pruritus.
• Residual postinflammatory hyperpigmentation may persist for months

44. • Constitutional symptoms, such as fevers, weight loss and myalgias, may
accompany flares of CSVV
• 90% of patients will have spontaneous resolution of cutaneous lesions
within several weeks or a few months, while another 10% will have
chronic or recurrent disease at intervals of months to years.

45. IgA vasculitis
• typically affects children following a respiratory tract infection, but may
also occur in adults.
• The classic tetrad= palpable purpura,
arthritis,
abdominal pain,
Microscopic hematuria
• occur in children <10 years
• slight male predominance in both children and adults.

47. The ACR criteria in 1990: if any two of
the following four criteria
(i) palpable purpura;
(ii) bowel angina;
(iii) age <20 years at onset; and
(iv) the presence of granulocytes in the
vessel wall
on biopsy
Paediatric Rheumatology Society for classifying
childhood‐onset vasculitis.
The presence of any one of the following four
features
in the presence of palpable purpura satisfi es a
classifi cation of IgA vasculitis:
(i) diffuse abdominal pain
(ii) any biopsy demonstrating predominant IgA
deposition
(iii)any acute arthritis or arthralgia; and
(iv)renal involvement in the form of
haematuria or proteinuria.

48. • Urticaria, vesicles, bullae, targetoid lesions, and foci of necrosis can also be
seen.
• Typically, lesions are symmetrically distributed on the buttocks and lower
extremities, but may also involve the trunk, upper extremities, and face.
• Individual lesions usually regress within 10 to 14 days.
• In pediatric patients, risk factors for the development nephritis include age
>8 years at onset, abdominal pain, and recurrent disease
• Poor prognostic factors include renal failure at the time of onset, nephrotic
syndrome, hypertension, and decreased factor XIII activity.

49. Acute Hemorrhagic Edema of Infancy
• children 4 to 24 months of age.
• etiology of AHEI is unknown, 75% of patients have an associated infection, drug
exposure, or immunization.
Infections
– Coxsackievirus
– Cytomegalovirus
– Epstein–Barr virus
– Herpes simplex virus
– Hepatitis A virus
– Measles
– Rotavirus
– Streptococcus spp.
Medications
– Acetaminophen
– Penicillins
– Cephalosporins
– Trimethoprim–sulfamethoxazole
– NSAIDs

50. • onset of disease is 1–2 weeks
• large erythematous patches or urticarial plaques that then evolve into
annular, targetoid purpuric plaques.
• favor the cheeks, ears, and extremities, truncal
• Lesions may be asymptomatic, painful as does healing with atrophic scars
• .

51. • Tender, nonpitting edema of the face, ears, extremities (including the
hands and feet), and scrotum is characteristic.
• oral petechiae, conjunctival injection, abdominal pain, arthralgias,
glomerulonephritis, and intussusception (<1%) may occur
• The course is benign, with spontaneous and complete resolution
without sequelae within 1 to 3 weeks

53. Urticarial Vasculitis
• Persistent urticarial lesions that demonstrate the histopathologic features of LCV
• The peak incidence is during the fifth decade, and 60–80% of patients with
urticarial vasculitis are female.
• The hypocomplementemic form occurs almost exclusively in women
• From 70–80% of cases of urticarial vasculitis are normocomplementemic and
they follow a benign course with an average duration of 3 years

54. • Associations
Autoimmune connective tissue diseases
(Sjögren syndrome, SLE)
Serum sickness
Cryoglobulinemia
Infections
– Hepatitis B virus
– Hepatitis C virus
– Epstein–Barr virus
– Lyme disease
Medications
– Cocaine
– Diltiazem
– Etanercept
– Infliximab
– Methotrexate
– NSAIDs
Hematologic malignancies
– Plasma cell dyscrasias (IgM,
IgG, IgA)
– Leukemias
– Lymphomas

55. • erythematous, indurated wheals , with or without angioedema, that favor the trunk,
proximal extremities
• Urticarial vasculitis is distinguished from chronic urticaria by individual lesions that
persist beyond 24 hours, are associated with burning and pain rather than pruritus,
and resolve with postinflammatory hyperpigmentation.
• most important prognostic feature is the
presence or absence hypocomplementemia.
• Patients with normal complement levels
tend to have skin-limited disease,with
hypocomplementemia are much more likely
to have systemic manifestations

56. • The hypocomplementemic urticarial vasculitis syndrome (HUVS) is a more severe
syndrome defined by specific diagnostic criteria:
• two major criteria: (1) urticaria for 6 months and
(2) hypocomplementemia – plus –
• two or more minor criteria: (1) vasculitis on skin biopsy;
(2) arthralgia or arthritis;
(3) uveitis or episcleritis;
(4) glomerulonephritis;
(5) recurrent abdominal pain; or
(6) positive C1q precipitin test with a low C1q level

57. • Patients with hypocomplementemia who do not meet the criteria for
HUVS are considered to have hypocomplementemic urticarial
vasculitis (but not HUVS).
• Musculoskeletal involvement is the most common extracutaneous
manifestation Arthralgias of the hands, elbows, knees, ankles, and
feet occur in half of all patients with urticarial vasculitis,
• up to 50% of patients with HUVS have frank arthritis.

58. • Renal involvement, manifesting as proteinuria or microscopic hematuria,
occurs in 5–10% of patients with HUVS
• Gastrointestinal manifestations (abdominal pain, nausea, vomiting,
diarrhea) occur in up to 30% of patients
• HUVS shares features with SLE, but distinctive clinical findings in HUVS
include ocular inflammation (30%; conjunctivitis, episcleritis, iritis, uveitis),
angioedema(>50%), and COPD-like symptoms (50%).

59. Erythema Elevatum Diutinum
• rare, chronic dermatosis characterized by red–violet to red–brown papules,
plaques, and nodules that favor extensor surfaces.
• Histopathologic features consist of LCV in early lesions, followed by fibrotic
replacement of the dermis in older lesions
• Middle aged and older adults (ages 30–60 years).
• An earlier onset occurs more often in the setting of HIV infection.

60. • typical lesions are violaceous, red–brown or yellowish papules, plaques, or
nodules that are symmetrically distributed.
• They favor acral and periarticular sites, in particular the extensor surfaces of the
elbows, knees, ankles, hands, and fingers
• Additional sites- the face, retroauricular area,
trunk, axillae, buttocks, and genitalia.
• Initially, the lesions are erythematous, but with
time become red–brown or violaceous in color
as well as doughy or firm to palpation due
to fibrosis

61. • lesions are asymptomatic, can be associated with a burning sensation or
pruritus.
• Nodular lesions, primarily palmoplantar, that progress to form bulky masses are
characteristic of EED in the setting of HIV infection
• There has been an increasing number of
reports of associated peripheral keratitis, nodular
scleritis, panuveitis, and blindness
• The disease is chronic and has a relapsing and
remitting course; the majority of cases resolve
spontaneously over a period of 5 to 10 years,
but the disease can last up to 40 years.

62. • In patients presenting with EED, evaluation for an associated infection
(e.g. streptococcal, viral hepatitis, HIV), monoclonal gammopathy
(serum immunofixation electrophoresis), or autoimmune disorder
should be considered.

63. Cryoglobulinemic Vasculitis
• Cryoglobulins are cold-precipitable immunoglobulins that can be divided into
three subtypes
Subtype Molecular composition Associations Pathophysiology Manifestations
I Monoclonal IgM > IgG Plasma cell dyscrasias,
lymphoproliferative
disorders
Vascular occlusion Raynaud
phenomenon,
retiform
purpura, gangrene,
acrocyanosis
II
III
Monoclonal IgM† (>IgG†)
against
polyclonal IgG
Polyclonal IgM† against
polyclonal IgG
HCV ≫ HBV, HIV,
autoimmune connective
tissue diseases (e.g.
rheumatoid arthritis),
lymphoproliferative
disorders (e.g. B-cell
non-Hodgkin
lymphoma, CLL)
Vasculitis Palpable purpura,
arthralgias,
peripheral
neuropathy,
Glomerulonephritis

64. • typically affects the skin, peripheral nervous system, and kidneys.
• palpable purpura of the lower extremities
• erythematous papules, ecchymoses, and dermal nodules; rarely, urticaria, livedo
reticularis, necrosis, ulcerations, and bullae are observed
• extracutaneous findings- arthritis ,
peripheral (typically sensory)neuropathy
gastrointestinal disease or hepatitis
membranoproliferative glomerulonephritis

65. Diagnosis
• investigations are necessary for two main purposes.
• First, it is important to establish if the vasculitis is primary or
secondary.should be directed to identify underlying rheumatological
disease, malignancy, infection or a primary vasculitis.
• Second, should be carried to demonstrate the presence of vasculitis
involving internal organs.

66. • Complete Blood Counts
• Mild Anemia – Anemia of Chronic Disease
• Differential Leucocyte Count:
Predominant eosinophils- Churg Strauss, HSP
• Thrombocytopenia may cause purpura
• ESR
• Non specific, But useful test to suggest presence of underlying inflammatory
process ,raised in systemic vasculitis, infection and malignancy

67. • C‐reactive protein May be raised in infections
• Urea and electrolytes Raised creatinine and urea in renal
involvement
• IgE Churg stauss
• Liver function HBV, HCV
• R- factor EMC

68. • Urinalysis Haematuria and proteinuria in renal involvement
HSP, Wegener’s, churg strauss
Chest X ray /HRCT
thorax
• Xray Para Nasal
Sinuses
-
Pulmonary infiltrates- small vessel
vasculitis
Pulmonary cavities- Wegener’s
granulomatosis
Sinusitis of Wegener’s
mucosal thickening- Churg strauss

69. • Viral Markers Hep. B= Poly Arteritis Nodosa
Hep.C= Essential Mixed Cryoglobulinemia
• Antinuclear
antibodies
present in autoimmune connective tissue disease
• Antineutrophil
cytoplasmic
antibody(ANCA
P-ANCA: Wegener’s Granulomatosis
C-ANCA: Microscopic polyangiitis, Churg Strauss, Wegener’s
vasculitis

70. • Immunoglogulin
levels
- Usually hyper gammaglobulinemia seen
- Elevated IgA levels= Henoch Sconlein Purpura
• Cryoglobulins Essential Mixed Cryoglobulinemia
• Complement
levels
reduced in immune compex mediated diseases - EMC, HSP,
huv
• Direct
immunofluorescence
Small vessel vasculitis
pan

71. • ARTERIOGRAPHY
Helps specially in in arteries
that cannot bebiopsied easily
like Aorta, Coronary artery,
Mesentric vessels
Presence of vascular patency,
Aneurysms
• Aortic Angiography- Takayasu’s
• Cerebral Angiography- Isolated CNS vasculitis
• Renal Angiography- PAN
• Coronary Angiography- Kawasaki’s
• Lower limb arteriography-Buerger’s Disease
(TAO)

72. • Skin Biopsy- to detect “leukocytoclasis” in cutaneous vasculitis--- all small vessel
and secondary vasculitides.
• Angiocentric segmental inflammation
with endothelial swelling,
• a neutrophilic infiltrate with
leukocytoclasia, red blood cell
extravasation,
• fibrinoid necrosis of blood vessel
walls.
• The neutrophils are present around
and within the walls of the small
dermal blood vessels

73. • LCV +granuloma- wegener’s
• Eosinophilic infiltration, extra vascular granulomas, vasculitis- Churg strauss
• LCV + Papillary and perifollicular neutrophilic infiltrate– EED
-- chronic lesion- angiocentric eosinophilic fibrosis, infiltration of plsma cell, -
macrophage , lymphocytes
-- Cholesterol deposit in histiocytes and extracellular tissue (extracellular
cholesterolosis)
• Temporal artery biopsy --- giant cell arteritis– gold standard

74. • Renal Biopsy- to detect glomerulonephritis especially in small
vessel vasculitis
• FSGN- seen in pauci immune vasculitis- micropan
• MPGN- seen in EMC
• Muscle, kidney testicle biopsy – PAN

75. Treatment
• Triggering drugs should be stopped
• Underlying infections treated
• Malignancy or associated rheumatological disease should be
managed

76. CUTANEOUS SMALL VESSEL VASULITIS
• Corticosteroids tapered over 2-3 weeks
• Colchicine – 0.6 mg twice daily
• Dapsone - 50-150 mg daily
• Cytotoxic agents –
• Azathioprine (1-2mg/kg/day)
• Cyclosporine (2.5-4mg/kg/day)

77. ERYTHEMA ELEVATUM DIUTINUM
• Treatment of associated disorder – HIV and para-proteinemia
• Dapsone – relapse on stopping may occur
• Niacinamide
• High potency topical/intralesional corticosteroids
• 5% topical dapsone gel

78. IgA VASCULITIS
• Supportive
• Self-limiting
• Dapsone and colchicine
• Glucocorticoids, Pulsed intravenous methylprednisolone, ciclosporine
, cyclophosphamide, MMF– a/w abd pain, arthritis, renal involvement

79. CRYOGLOBULINEMIC VASCULITIS
• Treatment of underlying cause
• Hepatitis C – combination of Glucocorticoids, antiviral therapy
• Interferon a in combination with Ribavirin – beneficial, relpases may
occur.
• Rituximab may be of benefit.
• Non- hepatitis C cryoglobulinaemic vasculitis- CS+ Cyclophophamide

80. HYPOCOMPLEMENTAEMIC URTICARIAL
VASCULITIS
• SYSTEMIC CORTIOSTEROIDS
• Drugs effective in urticarial vasculitis –
dapsone (100-200 mg OD)
colchicine (0.6 mg twice to thrice a day)
HCQs (200mg OD or BD)
• For refractory cases – cyclophosphamide / MMF

81. MICROSCOPIC POLYANGITIS
• European league of Against Rheumatism and British society of
Rheumatology
• Remission Induction
• Pulsed intravenous cyclophosphamide (15 mg/kg every 2-3 weeks) or
daily oral cyclophosphamide (2 mg/kg/day)
• Oral prednisolone (1 mg/kg/day) to maximum of 60 mg – adjunct to
cyclophosphamide
• Aim- is to reduce the dose upto 15 mg/day at 3 month

82. • Methyl prednisolone can be added 1 gm /day for 3 days
• Severe renal disease – plasmapheresis
• Relapsing and refractory disease-
• Rituximab 375 mg/m^2 per week for 4 weeks > pulsed IV
cyclophosphamide
• Remission maintenance
• Post – cyclophosphamide – azathioprine (2 mg/kg/ day)
after 6 pulses or 6 months of oral cyclophosphamide
• Post- rituximab – rituximab at 4-6 monthly interval

83. GRANULOMATOSIS WITH POLYANGITIS
• European League of Rheumatism and British Society for
Rheumatology
• Localized – methotrexate (15-25 mg/week) with prednisolone ( 1
mg/kg/day in tapering doses upto 15 mg / day)
• Higher risk of relapse
• Non- localized – cyclophosphamide – mainstay
• Pulsed intravenous cyclophosphamide (15 mg/kg every 2-3 weeks)
for 6 pulse or
daily oral cyclophosphamide (2 mg/kg/day) for 6 months

84. • Oral prednisolone (1 mg/kg/day) to maximum of 60 mg – adjunct to
cyclophosphamide
• Methyl prednisolone can be added 1 gm /day for 3 days
• Severe renal disease – plasmapheresis
• Rituximab 375 mg/m^2 per week for 4 weeks
• Remission - azathioprine (2 mg/kg/ day)
after 6 pulses or 6 months of oral cyclophosphamide
• Methotrexate
• Leflunamide
• MMF

85. EOSINOPHILIC GRANULOMATOSIS WITH
POLYANGITIS
• LOCALIZED - Oral prednisolone (1 mg/kg/day) ENOUGH TO INDUCE
REMISSION
• In patients without organ involvement / life-threatening condition
Methotrexate 20- 25 mg/week
• SYSTEMIC DISEASE – Pulsed intravenous cyclophosphamide (15
mg/kg every 2-3 weeks – six pulse with - Oral prednisolone (1
mg/kg/day)
• REFRACTORY - Rituximab

86. • Remission maintenance-
• Post – cyclophosphamide – azathioprine (2 mg/kg/ day)
• Post- rituximab – rituximab as per need
• Post – methotrexate – continuing long-term methotexate

87. POLYARTERITIS NODOSA AND CUTANEOUS
POLYARTERITIS NODOSA
• Screen for infection
• Discontinue medication that predates disease
• HBV – antiviral + immunosuppressive
• NSAIDS and salicylates – for cPAN
• High dose corticosteroids with tapering doses over 3-6 months
• Strong association with streptococcal infection- Penicillin – treatment
and prophylaxis

88. • sulfapyridine
• Pentoxifylline
• Dapsone
• Low-dose weekly methotrexate (7.5 – 20 mg/week) – is unresponsive
to corticosteroids
• Chronic leg ulcers – GM- CSF

89. KAWASAKI DISEASE
• Intravenous Immunoglobulin and Aspirin 100mg/kg/day until fever
has settled
• Then reduce to 3-5 mg/kg/day for 6-8 weeks – in no cardiac
abnormality and longer with coronary aneurysm
• IVIg - single dose – 2 g/kg over 12 hour
• If patient febrile after 1st dose of IVIg – repeat 2g/kg
• High dose Prednisolone – 2 kg/kg/day – tapered after normalization
of CRP

90. GIANT CELL ARTERITIS
• Treatment ASAP – To avoid complication
• Visual loss – methylprednisolone 500 – 1000 mg daily for 3 days followed
by oral prednisolone
• Aspirin
• Prednisolone – 40- 60 mg/ day – taper slowly over months after CRP/ESR
levels remain controlled
• Treatment over 2 years
• weekly methotrexate (15 – 20 mg/week)

91. TAKAYASU ARTERITIS
• No proven treatment
• Oral prednisolone (1 mg/kg/day)
• Oral prednisolone (1 mg/kg/day) + azathioprine
• Cyclophosphamide
• Infliximab
• Tocolizumab