Vasculitides are rare autoimmune diseases characterized by inflammation of blood vessels, leading to potential organ damage and classified by vessel size. The document details large-, medium-, and small-vessel vasculitis, including specific conditions like giant cell arteritis, Takayasu arteritis, Kawasaki disease, and polyarteritis nodosa, along with their epidemiology, clinical features, and management strategies. Treatment generally includes glucocorticoids and immunosuppressants, with specific therapies based on the type and underlying causes.

1. Systemic vasculitis
(vasculitides)
By Dr Nwachukwu shalom

2. definitio
n
● Vasculitides are a heterogeneous group
of rare autoimmune diseases
characterized by blood vessel
inflammation (vasculitis). Inflammation
can lead to ischemia, necrosis, and/or
hemorrhage, with subsequent end-
organ damage.
● They are either primary (idiopathic) or
secondary to an underlying disease (e.g.,
HBV infection, cancer, systemic lupus
erythematosus) or drug use.
● They are further classified based on the
size of the affected vessels: small-,
medium-, or large-vessel vasculitis, or
variable vessel vasculitis.

3. Etiology
-Primary (idiopathic)
-Secondary to another disease or drug use, e.g.:
Infectious diseases
Viral infection: e.g., HBV, HCV, HIV
Infectious endocarditis
Tuberculosis
Syphilis
-Drugs: e.g., hydralazine, cocaine
-Malignancy: e.g., multiple myeloma, lymphoproliferative disorders
-Autoimmune diseases: e.g., systemic lupus erythematosus (SLE), Sjogren syndrome, sarcoidosis

4. Large-vessel vasculitis
Affects: aorta and great vessels

5. Giant cell arteritis
(Temporal arteritis, Horton disease)
Giant cell arteritis (GCA) is a type of
autoimmune vasculitis that causes chronic
inflammation of large and medium-sized
arteries, in particular the carotid arteries,
its major branches, and the aorta.
GCA is most common in women over the
age of 50 and of northern European
descent, and approximately 50% of
patients also have polymyalgia
rheumatica.

6. Presentation
● Fever
● weight loss
● night sweats
● Fatigue
● Malaise
● new-onset headache
● tender, hardened temporal
artery
● jaw claudication
● amaurosis fugax.

7. Management
If there is strong clinical suspicion for GCA, glucocorticoids should be administered
immediately, even prior to diagnostic workup if needed, to reduce the risk of permanent
vision loss and cerebral ischemia
Laboratory studies: ESR, specifically 50 mm/h (due to Rouleaux formation of RBCs),
↑ ≥ ↑
CRP
Duplex Ultrasound
Temporal artery biopsy

8. Takayasu arteritis
Takayasu arteritis (aortic arch syndrome) is a
systemic vasculitis of large vessels
characterized by granulomatous
inflammation of the aorta and its major
branches. It most commonly affects Asian
women < 40 years of age.
Patients typically present with constitutional
symptoms, decreased bilateral brachial and
radial pulses, angina, and syncope.
Epidemiology: 15–45 y.o, > (9:1)
♀ ♂

9. Management
MR angiography /CT angiography
High-dose glucocorticoids,
Methotrexate/Azathioprine/Cyclophosphamide/TNF inhibitor (infliximab)

10. Medium-vessel vasculitis
Affects: arteries with muscular walls

11. Kawasaki
disease
Kawasaki disease is an acute,
necrotizing vasculitis of unknown
etiology. The condition primarily
affects children under the age of
five and is more common among
those of Asian descent.
coronary artery aneurysms are
the most concerning possible
manifestation as they can lead to
myocardial infarction or
arrhythmias.

12. Clinical Features
fever for at least 5 days and one of the following
≥ 4 other specific symptoms
< 4 specific symptoms and involvement of the coronary
arteries
Specific symptoms include:
● Erythema and edema of hands and feet, including the palms
and soles (the first week)
● Possible desquamation of fingertips and toes after 2–3 weeks
● Polymorphous rash, originating on the trunk
● Painless bilateral “injected” conjunctivitis without exudate
● Oropharyngeal mucositis
● Erythema and swelling of the tongue (strawberry tongue)
● Cracked and red lips
● Cervical lymphadenopathy (mostly unilateral)

13. Treatment
● IV immunoglobulin (IVIG)
● High-dose oral aspirin
● IV glucocorticoids

14. Polyarteritis nodosa (PAN)
Polyarteritis nodosa (PAN) is a systemic vasculitis
of medium-sized vessels that most commonly
affects the skin, peripheral nerves, muscles,
joints, gastrointestinal tract, and kidneys, but
usually spares the lungs.
PAN is associated with certain viral infections,
most commonly hepatitis B virus (HBV) infection.

15. Clinical features
Nonspecific symptoms
-Fever, weight loss, malaise
-Muscle and joint pain
Organ involvement
-Renal involvement ( 60%): hypertension, renal
∼
impairment.
-Coronary artery involvement ( 35%); risk of
∼ ↑
myocardial infarction
-Skin involvement ( 40%): rash, ulcerations, nodules
∼
-Neurological involvement: polyneuropathy (mononeuritis
multiplex), stroke
-GI involvement: abdominal pain, melena, nausea,
vomiting
-Usually spares the lungs

16. Treatment
Immunosuppressants (e.g., glucocorticoids PLUS cyclophosphamide) are
indicated for all patients to achieve remission.
Antiviral therapy is indicated for HBV-associated PAN.

17. Small-vessel vasculitis
Affects: capillaries, arterioles and venules

18. Types of small vessel vasculitis
Cutaneous small vessel vasculitis
Occurs 7-10 days after certain
medications (penicillin,
cephalosporins, phenytoin,
allopurinol) or infections (eg, HCV,
HIV). Palpable purpura, no visceral
involvement.
Immunoglobulin A vasculitis
Most common childhood systemic vasculitis. Often
follows URI.
Classic triad of Henoch-Schönlein purpura
● Hinge pain (arthralgias)
● Stomach pain (abdominal pain associated
with intussusception)
● Palpable purpura on buttocks/legs
Associated with IgA nephropathy (Berger disease).
Treatment: supportive care, possibly
corticosteroids.

19. Granulomatosis with polyangiitis
Upper respiratory tract: perforation of nasal
septum, chronic sinusitis, otitis media,
mastoiditis.
Lower respiratory tract: hemoptysis, cough,
dyspnea.
Renal: hematuria, red cell casts.
Triad:
● Focal necrotizing vasculitis
● Necrotizing granulomas in lung and upper
airway
● Necrotizing glomerulonephritis
CXR: large nodular densities
Treatment: corticosteroids in combination with