Rheumatology I 05 Vasculitis

Rheumatic Diseases
Vasculitis
With Kartik Rangaraj MD
Wen Jie Lau, j.wenjie93@hotmail.com

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Vasculitis
(Large, Middle and small Vessel)

Vasculitis
• Vasculitis is immune-mediated damage to vessel walls,

inducing necrosis.
• The best way to think about vasculitis is that it is a common

endpoint to numerous disease entities.
• Idiopathic
• Connective tissue disease
• Infectious (esp. viral)

Definition • Malignant

Vasculitis
Of the idiopathic vasculitides, the most common way to characterize them is by size of vessel
involvement.
Large vessel Medium vessel Small vessel

Takayasu’s arteritis Polyarteriitis nodosa ANCA-associated vasculitis
Giant-cell arteritis Buerger’s disease Henoch-Schönlein purpura
(temporal arteritis) (thrombangiitis obliterans)
Cutaneous leukocytoclastic
angiitis
Essential mixed
cryoglobulinemia
So, while each of these entities end up with vasculitis, they differ in the clinical presentation.

Vasculitis – Large Vessel Vasculitides
Clinical manifestation Diagnostic studies Treatment
Takayasu Fever, weight loss, arthralgias, pain and Elevated ESR, High-dose steroids, add
arteritis tenderness of the vessels, unequal MRI/MRA of the cyclo-phosphamide if
pulses, asymmetric blood pressures in vessels showing severe
young Asian women abnormalities
(aortic arch),
carotid doppler
can also be done
Giant-cell Fever, weight loss, fatigue, myalgias, Elevated ESR, Corticosteroids as soon
arteritis tender temporal arteries, unilateral temporal artery as clinically suspected
(Temporal headache, amaurosis fugax, jaw biopsy → giant cell even before performing a
arteritis) claudication, polymyalgia rheumatica and mononuclear temporal artery biopsy to
seen in 50 % of patients infiltrates in vessel prevent permanent
walls blindness

Vasculitis
Narrowing of brachiocephalic, carotid, and Temporal artery enlargement

subclavian arteries in Takayasu
Vinay Kumar & Abul K. Abbas & Jon C. Aster, Robbins & Cotran Pathologic Basis of Disease, 9th Edition, 2015, p. 509, Fig. 11-25, p. 508, Fig. 11-24 C,
(Elsevier)
Vasculitis – Medium Vessel Vasculitides

Polyarteritis Does not affect the Biopsy of vessel gold standard Steroids for mild
Nodosa venous circulation, → necrosis and inflammation of disease, add
multisystem vessel walls; cyclophosphamide
inflammatory process: Mesenteric and renal for organ-threatening
fever, malaise, weight angiography can show disease;
loss, mesenteric microaneurysms if associated with
ischemia, tender Hepatitis B → treat
cutaneous ulcers, renin- *PAN most confused by with lamivudine,
mediated hypertension infectious vasculitis steroids, and
but not GN, plasmapheresis
mononeuritis multiplex

Vasculitis
One last thought before discussing the

individual vasculitides:
Angiography is a very good way to visualize

vessels and those affected by vasculitis are
characterized by alternating strictures and
aneurysms.
Gekka M, Sugiyama T, Nomura M, https://openi.nlm.nih.gov/detailedresult.php?img=PMC4578610_12883_2015_429_Fig2_HTML&query=

angiography+vasculitis&it=xg&lic=by&req=4&npos=60, CC BY 4.0, cropped
Vasculitis – Medium Vessel Vasculitides

Kawasaki Children, self-limiting Fever > 5 days and 4 of 5 of Steroids;
Disease Fever, conjunctivitis, the following: IVIG has significantly
mucositis, rash • Bilateral conjunctivitis reduced coronary
(erythema and • Oral changes (strawberry manifestations
desquamation of palms tongue, lip fissures)
and soles); • Erythema/desquamation of
Most feared palms or soles
complication is cardiac • Polymorphous rash
disease (esp. coronary
artery aneurysms) • Cervical lymphadenopathy

Vasculitis – Small Vessel Vasculitides
Generally separated into ANCA-associated and non-ANCA-associated

• Remember: ANCA stands for Anti-Neutrophil Cytoplasmic Antibody
Since this affects small vessels, these diseases affect certain organs:
• Skin
• Muscle/nerves
• Lungs
• Kidneys

ANCA-associated vasculitis
• Two forms of ANCA: p- (peripheral) and c- (cytoplasmic) ANCA
• p-ANCA targets a neutrophilic protein called myeloperoxidase (MPO)
• c-ANCA targets a neutrophilic protein called proteinase 3 (PR3)
• These antibodies are thought to be pathogenic.
• Non-immune complex mediated vessel damage


Granulomatosis Sinusitis, hearing loss PR3-ANCA positive Steroids and
with Polyangiitis (new ear tubes in Biopsy shows either: methotrexate for non-
(Wegener) elderly pts means WG!), • Granulomas vasculitis diseases;
saddle-nose deformity, Steroids, plasma-
purpura, mononeuritis • Pulmonary
hemorrhage pheresis, cyclo-
multiplex, hemoptysis, phosphamide for
hematuria • Pauci-immune GN organ or life-
threatening disease
Microscopic Identical to WG except MPO-ANCA positive Steroids,
Polyangiitis sinusitis, hearing loss, Biopsy will not show plasmapheresis,
(MPA) saddle-nose deformity granulomas cyclophosphamide for
organ or life-
threatening disease


Eosinophilic Elderly patient with new- Eosinophilia, abnormal Steroids for most cases
Granulomatosis onset asthma and/or CXR
with Polyangiitis allergic rhinitis is CSS 50 % MPO-ANCA Severe disease will
(Churg-Strauss) unless otherwise proven! positive also require
Skin nodules/rash, Surgical lung biopsy cyclophosphamide
pericarditis, mononeuritis gold standard →
multiplex eosinophilic infiltrates,
necrosis, granulomas

Leukocytoclastic Vasculitis
Wong SF, Newland L, John T, White SC, Painful, palpable rash with hemorrhagic bullae of hands, https://openi.nlm.nih.gov/detailedresult.php?img=
PMC3443657_1752-1947-6-261-2&query=leukocytoclastic+vasculitis&it=xg&lic=by&req=4&npos=26%20CC%20BY%202.0, resized, CC BY 2.0
Vasculitis
Purpuric rash Saddle-nose deformity
E. Goljan, Rapid Review Pathology E-Book, 4th Edition, 2013, p. 235, Fig. 10-15 E F, Mosby (Elsevier)
Vasculitis
(Non-ANCA-associated Vasculitis)

Vasculitis – Non-ANCA-associated Vasculitis

Henoch- More prevalent in Skin biopsy of the Self-limited disease,
Schönlein children purpura will treatment is supportive
Purpura Palpable purpura, demonstrate IgA Short courses of
glomerulonephritis, deposition steroids may be
mesenteric ischemia, needed for
and arthritis symptomatic relief
Cutaneous Skin lesions: purpura Immunofluorescence Stop the inciting agent,

Leukocytoclastic (both palpable and non- testing of skin biopsy short course of
angiitis palpable), urticaria; with leukocytoclastic corticosteroids
secondary to drug vasculitis
reactions


Essential Mixed Seen in Hep C patients (+) Cryoglobulins Steroids
Cryoglobuli- Palpable purpura, Low serum C3 and C4 Cases associated with
nemia immune-complex GN, Rheumatoid factor, Hep hepatitis C: treat with
digit infarction, C particles usually interferon and ribavirin
mononeuritis multiplex, present, but sometimes Severe cases: steroids,
occasional hemoptysis are absent during flares cyclophosphamide,
as they are part of the plasmapheresis
cryocrit


Hereditary Blanching skin lesion, Clinical Experimental
Hemorrhagic recurrent epistaxis, avm,
Telangiectasi GI-bleeding, hematuria

Four types of Seronegative Spondyloarthropathies
These diseases are:

• Ankylosing spondylitis
• Reactive arthritis (Reiter's syndrome)
• Psoriatic arthritis
• Enteropathic arthritis (Crohn’s and ulcerative colitis)

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Rheumatic Diseases

With Kartik Rangaraj MD

Wen Jie Lau, j.wenjie93@hotmail.com

Wen Jie Lau, j.wenjie93@hotmail.com

• Vasculitis is immune-mediated damage to vessel walls,

• The best way to think about vasculitis is that it is a common

• Connective tissue disease

• Infectious (esp. viral)

Wen Jie Lau, j.wenjie93@hotmail.com

Large vessel Medium vessel Small vessel

Wen Jie Lau, j.wenjie93@hotmail.com

Clinical manifestation Diagnostic studies Treatment

Wen Jie Lau, j.wenjie93@hotmail.com

Narrowing of brachiocephalic, carotid, and Temporal artery enlargement

Clinical manifestation Diagnostic studies Treatment

Wen Jie Lau, j.wenjie93@hotmail.com

One last thought before discussing the

Angiography is a very good way to visualize

Gekka M, Sugiyama T, Nomura M, https://openi.nlm.nih.gov/detailedresult.php?img=PMC4578610_12883_2015_429_Fig2_HTML&query=

Clinical manifestation Diagnostic studies Treatment

Wen Jie Lau, j.wenjie93@hotmail.com

Generally separated into ANCA-associated and non-ANCA-associated

Wen Jie Lau, j.wenjie93@hotmail.com

• p-ANCA targets a neutrophilic protein called myeloperoxidase (MPO)

• c-ANCA targets a neutrophilic protein called proteinase 3 (PR3)

• These antibodies are thought to be pathogenic.

• Non-immune complex mediated vessel damage

Wen Jie Lau, j.wenjie93@hotmail.com

Clinical manifestation Diagnostic studies Treatment

Wen Jie Lau, j.wenjie93@hotmail.com

Clinical manifestation Diagnostic studies Treatment

Wen Jie Lau, j.wenjie93@hotmail.com

Purpuric rash Saddle-nose deformity

Wen Jie Lau, j.wenjie93@hotmail.com

Clinical manifestation Diagnostic studies Treatment

Cutaneous Skin lesions: purpura Immunofluorescence Stop the inciting agent,

Wen Jie Lau, j.wenjie93@hotmail.com

Clinical manifestation Diagnostic studies Treatment

Wen Jie Lau, j.wenjie93@hotmail.com

Clinical manifestation Diagnostic studies Treatment

Wen Jie Lau, j.wenjie93@hotmail.com

These diseases are:

• Reactive arthritis (Reiter's syndrome)

• Enteropathic arthritis (Crohn’s and ulcerative colitis)

Wen Jie Lau, j.wenjie93@hotmail.com

Wen Jie Lau, j.wenjie93@hotmail.com

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