Paediatric Vasculitis

Question 1

What is henoch shonlein purpura

Answer 1

acute immune complex-mediated small vessel vasculitis

often preceded by an upper respiratory tract infection

Sex: ♂ > ♀
More common in children
90% of cases < 10 years old
Peak incidence: 6 years

typically presents with a tetrad of symptoms: palpable purpura, arthritis/arthralgia, abdominal pain, and renal disease.

in individual patients only some of the classic tetrad of symptoms may be present.

Question 2

What are the terras of symptoms in HSP

Answer 2

palpable purpura,

arthritis/arthralgia,

abdominal pain,

renal disease.

Question 3

RF for HSP

Answer 3

1) Preceding viral or bacterial infection 1–3 weeks prior
- upper respiratory tract by (group A streptococcus)
- GI infections also possible

2) Genetic predisposition
3) Drugs ( antibiotics and antiarrhythmics)
4) vaccinations (e.g., yellow fever, cholera)

Question 4

PP of HSP

Answer 4

exposure to allergen/antigen (infection, drugs) → stimulation of IgA production → deposition of IgA immune complexes in vascular walls (skin, GI tract, joints, kidneys) → activation of complement → vascular inflammation and damage

Question 5

Clinical features of HSP

Answer 5

History
Recent history of URTI (1-3)was

Manifestations
Skin (∼ 100% of cases)
-Symmetrical,raised, erythematous macules or that may coalesce into a palpable purpura (non-blanching skin lesions)
-common sites: the lower extremities, buttocks, and other areas of pressure or constraint (diapers)

-Joints (∼ 75% of cases): arthritis/arthralgia, most common in the ankles and knees

-Gastrointestinal tract (∼ 60% of cases)
Colicky abdominal pain (mimic acute abdomen)
Bloody stools or melena
Nausea/vomiting

-Kidneys (∼ 50% of cases): HSP nephritis with signs and symptoms of nephritic syndrome

Question 6

Dx of HSP

Answer 6

Dx for purpura
-Hypersensitivity vasculitis: no IgA deposition on biopsy
-Small vessel vasculitides : no IgA deposition on biopsy, affects adults and is associated with specific antibodies
-Coagulopathies: abnormal coagulation studies
Immune thrombocytopenia;
hemolytic uremic syndrome: low platelet count, non-palpable purpura

Dx for arthritis/arthralgia

• Autoimmune diseases : no IgA deposition on biopsy and associated with specific antibodies
• Septic arthritis: arthritis typically in a single joint
• Reactive arthritis: no rash, abdominal pain, or renal symptoms

Dx for renal disease
-IgA nephropathy (Berger’s disease) : no rash, joint, or GI symptom and primarily affects (young) adult

Question 7

Rx of HSP

Answer 7

Mild disease

• Outpatient treatment
• Usually no treatment necessary
• NSAIDs for pain management, rest, and adequate
• hydration

Severe disease
-Inpatient treatment
-Systemic glucocorticoids for severe abdominal pain not relieved by NSAIDs—IV fluids to maintain hydration
severe renal disease: IV methylpredinosolone

Question 8

Complications of HSP

Answer 8

Renal
HSP nephritis may progress to nephrotic syndrome.

Serious complication: rapid-progressive glomerulonephritis (RPGN) with crescent formation

Gastrointestinal

1) Small bowel infarction or perforation
2) Intussusception

Question 9

What is Polyarteritis nodosa

Answer 9

systemic vasculitis
of the small and medium-sized vessels
which leads to tissue ischemia; most commonly involving
skin, peripheral nerves, muscles, joints, gastrointestinal tract, and kidneys

Peak incidence: ∼ 45–65 years
Sex: ♂ > ♀
Often associated with hepatitis B or C infections

Question 10

Clinical features of PAN

Answer 10

Clinical features
Nonspecific symptoms: fever, abdominal, muscle, and joint pain

Renal involvement : hypertension, renal impairment

Coronary artery involvement ; increased risk of myocardial infarction

Skin involvement: rash, ulcerations, NODULES

Neurological involvement: polyneuropathy & stroke

GI involvement: abdominal pain, nausea, vomiting

Question 11

How do you differentiate PAN from other vasculitides

Answer 11

Usually spares the lungs

Characteristic nodules on skin

Question 12

Dg of PAN

Answer 12

1)Blood tests
Hepatitis B serology
Hepatitis C serology
↑↑ ESR
Anemia, leukocytosis

2) Urine analysis: proteinuria, hematuria
3) Muscle biopsy: transmural inflammation of the arterial wall with leukocytic infiltration and fibrinoid necrosis
4) Angiography: aneurysms and stenosis of small and medium-sized vessels of the involved organs

Question 13

Rx of PAN

Answer 13

Immunosuppression: corticosteroids,cyclophosphamide

Antiviral therapy against HBV and HCV may be required.