Professional Documents
Culture Documents
Henoch-Schonlein Purpura Ig A Vasculitis: Dr. Nisha Singh Moderator: DR Sumidha Mittal
Henoch-Schonlein Purpura Ig A Vasculitis: Dr. Nisha Singh Moderator: DR Sumidha Mittal
Ig A Vasculitis
Dr. Nisha Singh
Moderator: Dr Sumidha Mittal
HSP/ IgA vasculitis
• Small vessel vasculitis with IgA-dominant
immune deposits that typically involves the
skin, gut and glomeruli, and is associated with
arthralgia and/or arthritis
European League Against Rheumatism ( EULAR)/Paediatric Rheumatology
International Trials Organisation (PRINTO)/Paediatric Rheumatology
European Society (PRES)
EPIDEMIOLOGY
• Incidence- 3 to 26.7 per 100, 000
• Age- Few months to late adulthood, More
common in 4-6 years
• M:F=2:1
• The clinical features are often atypical at
extremes of age
• More severe in adults
PRECIPITATING ORGANISM
• Group A b hemolytic streptococcus-
M.C.
• Hepatitis A and B
• CMV, HIV, Adenovirus,HSV
• Mycoplasma, H.pylori
• Toxacara canis, human parvovirus B19,
Varicella, and scarlet fever
• Vaccinations- MMR, pneumococcal,
influenza, meningococcal and hepatitis
B
CLINICAL FEATURES
• Many cases follow an upper respiratory tract
infection
Skin
• Palpable purpura-
– Lower limbs and buttocks.
– Symmetrically distributed
– Extensor dependent surfaces
– May involve the arms, face and ears
– Usually spares the trunk.
• Occur in crops for several months
• May be preceded by urticarial or erythematous, maculo-
papular lesions that usually disappears within 24 hours
• Rash may appear as bullae, necrotic lesions or deep
bruising
• Severe bullous lesions -2%
• Subcutaneous edema over the dorsa of the
hands and feet and around the eyes,
forehead, scalp, and scrotum may occur early
in the disease, particularly in the very young
child.
Gastrointestinal Involvement
• 50–75% of cases
• GI manifestations may precede purpura by up to 2
weeks in 20% of children
• Colicky abdominal pain- M.C., Periumbilical and/or
epigastric pain
• Vomiting
• GI haemorrhage
– Positive stools for occult blood/overt bleeding
– Due to oedema and haemorrhage of the bowel wall due to
vasculitis
• Gastrointestinal mucosal ulceration and purpura
• Gastritis
• Duodenitis
• Intussusception - rare complication
– Either ileo-ileal, or ileo- colic
• Protein losing enteropathy
• Pancreatitis
• Hydrops of the gall bladder
Joints
• Arthritis or arthralgia- presenting symptom in
15–25% of cases
• Up to 82% of patients have a degree of joint
involvement during the illness
• Large joints of the lower limbs including
knees, ankles, feet and hips
• Upper limbs may be affected too
Renal
• In 12–92%
• Develops within four weeks - 75– 80% and
– Within three months - 97–100%
• Haematuria
• Proteinuria
• Nephrotic syndrome/ Nephritis
• Renal impairment
• Hypertension.
Neurological
• Rare
• Nonspecific headache followed by subtle
encephalopathy with minimal changes in
mental status, such as labile mood, apathy
and hyperactivity
• Seizures
• Subdural haematoma, subarachnoid
haemorrhage, cerebellar haemorrhage,
intraparenchymal bleeding and infarction
Pulmonary
• Rare
• Diffuse alveolar haemorrhage- M.C.
• Interstitial pneumonia
• Interstitial fibrosis
Urological
• Orchitis -24%
• Genital swelling may also be due to more
generalised oedema secondary to
hypoalbuminaemia from renal or
gastrointestinal involvement
PATHOLOGY
• Skin Biopsy - Leucocytoclastic Vasculitis with
perinuclear infiltration of polymorphs and
mononuclear cells.
• IgA is found in most purpuric lesions and can
also be found in non-affected areas
• Renal Biopsy
• The renal lesion of HSP nephritis- indistinguishable
from that of IgA nephropathy
• Focal and segmental proliferative
glomerulonephritis.
• The proliferation of extracapillary cells may result
in crescent formation.
• IF reveals mesangial IgA with IgG, C3 and fibrin.
• It is the non-renal clinical features that
differentiate IgA nephropathy from HSPN.
INVESTIGATIONS
• No specific test
• Anemia, Leukocytosis, Increased ESR
• Thrombocytosis- Severe disease
• Abdominal USG – to exclude intussception
• IgA is elevated in 50-70%; however there is no
correlation with disease severity.
• C3,C4 maybe low
• Site of infection- blood cultures, swabs, urine C/S
and CXR
• Evidence of recent streptococcal infection-
Raised ASO and anti-DNAse B titres
• Viral investigations (serology and throat swabs)
• Skin Biopsy
• Renal Workup and Biopsy
Skin Biopsy- SHARE GUIDELINES-
European consensus based recommendations
• Skin biopsy should include specific immunofluorescence
staining for IgA
• Leucocytoclastic Vasculitis
• Skin biopsy-
– not required for typical lesions
– for atypical rash (such as extensive lesions or diffusely distributed
lesions) to exclude alternative diagnoses.
– should be of the most recent lesions.
– if taken in the centre of the necrotic lesion, IgA deposition may be
falsely negative due to the presence of proteolytic enzymes
– absence of IgA staining does not exclude the diagnosis of IgAV.
– is also important to exclude other forms of vasculitis such as
ANCA-associated vasculitis, particularly in older children
• Corticosteroids
• Immunosuppresants : AZT, MMF, CYC,
Ciclosporin
SHARE Guidelines-Use of analgesia
• Adequate analgesia should be prescribed for
IgAV -associated arthropathy
• NSAIDs are not C/I if renal function is normal
• Adequate analgesia should be prescribed for
IgAV -associated abdominal pain
• Use of NSAID is relatively C/I in the presence
of active gastrointestinal bleeding
Use of Corticosteroids
• Indications:
• Orchitis, Cerebral Vasculitis, Pulmonary haemorrhage
• Other severe organ- or life-threatening vasculitis
manifestations
• Can be considered: Severe abdominal pain and/or
rectal bleeding
• Dose of prednisolone : 1-2 mg/kg/day
• If CS are indicated, pulsed IV Methylprednisolone (e.g.
10-30 mg/kg with a maximum of 1 g/ day on 3
consecutive days) may be considered for severe cases
• Prophylactic CS treatment to prevent the
development of IgAV-associated nephritis is not
indicated
IgA V Nephritis