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Indian Journal of Rheumatology 2012 May

Review Article
Volume 7, Number 1 (Suppl); pp. 92–98

Henoch–Schonlein purpura: An update

Nutan Kamath1, Suchetha Rao2

ABSTRACT

Henoch–Schonlein purpura (HSP), the commonest childhood vasculitis, is characterised by non-thrombocytopaenic

palpable purpura, arthritis or arthralgias, gastrointestinal and renal involvement. The peak incidence is between the
ages of 4 years and 8 years with a male preponderance. Though the diagnosis is usually clinical, a tissue biopsy
revealing leucocytoclastic vasculitis is helpful when the presentation is atypical. Renal involvement in the form of an
immune complex glomerulonephritis is the most serious long-term complication. The aetiopathogenesis, classifica-
tion, clinical features, relevant Indian data, and a stepwise management approach with corticosteroids and immuno-
suppressive agents per the renal histology are discussed.
Keywords: Arthritis, children, nephritis, palpable purpura, vasculitis

INTRODUCTION AND NOMENCLATURE between the ages of 3 years and 15 years and is more common
in males. In an international cohort of 827 patients with HSP,
Henoch–Schonlein purpura (HSP) is a multisystem, small- the mean age at onset was 6.9 ± 3 years and 51% were boys.2
vessel, immunoglobulin A (IgA) immune complex-mediated Bagga et al. studied 47 children with HSP in North India.
leucocytoclastic vasculitis. It was originally known as The mean age at onset was 8.5 years and the male:female
Heberden–Willan disease as described in 1801 by William ratio was 2.6:1.3 Grover et al. reviewed 30 children with HSP,
Heberden. In 1837, Johann Lukas Schonlein recognised the also in North India. The mean age was 10.5 years and the
association between purpura and arthritis as “peliosisrheu- male:female ratio was 1.7:1.4 Henoch–Schonlein purpura
matica” and his pupil Edouard Heinrich Henoch subsequently was diagnosed in 209 children (155 males, 54 females,
reported a case in 1874 that included bloody diarrhoea, ratio 2.87:1) analysed over a period from 1993 to 2008, by
abdominal pain and renal involvement with purpuricrash. Singh et al. The median age at diagnosis was 6.9 ± 2.98
The term anaphylactoidpurpura was applied by Gairdner years (range 1–17 years) and the disease exhibited a sea-
in 1948.1 sonal trend in the distribution with a noticeable peak in the
This review highlights the new classification criteria of months of October and November.5 Seasonal variation with
HSP and evolving knowledge regarding the pathogenesis, most cases occurring in winter has been reported in western
diagnosis and management. data as well.1

EPIDEMIOLOGY AETIOLOGY AND PATHOGENESIS

Henoch–Schonlein purpura is predominantly a childhood The aetiology of HSP is unclear but is associated with
vasculitis, rarely reported in adults. Worldwide, Asians have infections, medications (methotrexate, anti-tumour necrosis
the highest incidence. As this disease is self-limited, its true factor agents), vaccination, tumours (non-small-cell lung
incidence may be under-reported. It occurs most frequently cancer, prostate cancer, and haematological malignancies),

1
Professor, 2Associate Professor, Department of Paediatrics, Kasturba Medical College, Mangalore, Manipal University, Karnataka, India.
Correspondence: Dr. Nutan Kamath, email: [email protected]; [email protected]
doi: 10.1016/S0973-3698(12)60034-X
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HSP vasculitis Review Article 93

alpha-1-antitrypsin deficiency and familial Mediterranean of disease. Knowledge acquired from studies of patients
fever (MEFV). with IgAN may also provide important insights into the
• The infections implicated as a potential trigger are group-A pathogenesis of HSP.8
β-haemolytic Streptococci, Bartonella, Haemophilus
parainfluenzae, M. pneumoniae, varicella, rubella, rubeola,
parvovirus B-19, hepatitis A and B virus, adenovirus,
human immunodeficiency virus, and Helicobacter pylori.1,6 GENETIC BACKGROUND
• The term anaphylactoid purpura suggests allergy as the
basis for HSP, as is seen after insect bites and exposure to Genetic contribution is complex and polygenic in nature.10
drugs and dietary allergens. human leucocyte antigen (HLA) A2, A11, and B35 antigens
• The deposition of IgA suggests that HSP is an IgA- were associated with a significantly increased risk whereas
mediated dysregulated immune response to an antigen. It HLA A1, B49, and B50 antigens were associated with
may operate through the alternate complement pathway decreased risk for HSP in children, in a Turkish study.11
which leads to neutrophil accumulation, resulting in inflam- Mutations in the familial MEFV gene were seen in patients
mation and vasculitis without a granulomatous reaction. with HSP.12 The HSP has also been described with hetero-
• The vasculitis causes extravasation of blood and its com- zygous C2 complement component deficiency.
ponents into the interstitial spaces resulting in oedema and
haemorrhage. This can involve multiple systems includ-
ing the skin, gastrointestinal (GI) tract, kidney, and joints.
Serum levels of IgA anti-cardiolipin antibodies were ele- DIAGNOSIS
vated in the acute phase in a study by Yang et al.7 Galactose-
deficient IgA1 is recognised by anti-glycan antibodies, Henoch–Schonlein purpura is a clinical diagnosis but when
leading to the formation of circulating immune complexes the presentation is atypical, tissue biopsy may be helpful.
and their mesangial deposition, resulting in renal injury. Although new criteria are proposed by the European League
Renal expression of alpha-smooth muscle actin (α-SMA) Against Rheumatism (EULAR) and Pediatric Rheumatology
has also been associated with progression of renal injury.8 Society (PRES), most of the studies published to date have
Disorders of coagulation and its activation are also associ- used the older criteria proposed by American College of
ated with the development of HSP. Rapid decline in factor Rheumatology (Table 1).
XIII has been observed in patients with severe abdominal The EULAR/PRES criteria have been prospectively
involvement.9 As this decline is seen before the appearance validated through an international, web-based prospective
of rash, it may be useful as a diagnostic marker in patients data collection that included 827 patients with HSP and 356
with abdominal symptoms. Factor XIII also declines prior with other vasculitides. The sensitivity and specificity of
to the recurrence of HSP. the new classification criteria were 100% and 87%, respec-
Henoch–Schonlein purpura nephritis (HSPN) and IgA tively, with a kappa-agreement of 0.90 (95% confidence
nephritis (IgAN) share common pathogenetic mechanisms interval [CI] 0.84–0.96).2
and may represent different ends of a continuous spectrum Table 2 summarises the new criteria.

Table 1 Diagnostic criteria of Henoch–Schonlein purpura (European League Against Rheumatism/Pediatric Rheumatology Society
and American College of Rheumatology)
EULAR/PRES criteria, 200613 ACR criteria, 199014
Mandatory criterion:
Palpable purpura Two or more of the following criteria are needed:

Plus at least one of the following criteria:

1. Diffuse abdominal pain 1. Age 20 years or less at disease onset
2. Immunoglobulin A deposition in any biopsy 2. Palpable purpura without thrombocytopaenia
3. Arthritis/arthralgias 3. Acute abdominal pain with gastrointestinal bleeding
4. Renal involvement (haematuriaand/or proteinuria) 4. Biopsy showing granulocytes in the walls of small arterioles/r venules
in the superficial layers of the skin
ACR = American College of Rheumatology; EULAR = European League Against Rheumatism; PRES = Pediatric Rheumatology Society.
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94 Indian Journal of Rheumatology 2012 May; Vol. 7, No. 1 (Suppl) Kamath and Rao

Table 2 2010 Classification criteria for Henoch–Schönlein purpura (EULAR/PRES)2

Criterion Definition Sensitivity Specificity
(%) (%)
Purpura (mandatory) Purpura (palpable, in crops) or petechiae, with lower limb predominance, not related 89 86
to thrombocytopaenia

And at least one of four of the following:

Abdominal pain Diffuse, acute, colicky pain. May include intussusception and gastrointestinal bleeding 61 64
Histopathology Leucocytoclastic vasculitis with predominant IgA deposit; or proliferative 93 89
glomerulonephritis with predominant IgA deposit
Arthritis or arthralgias Arthritis. Acute joint swelling or pain with limitation on motion 78 42
Arthralgia. Acute joint pain without joint swelling or limitation on motion
Renal involvement Proteinuria, > 0.3 g/24 hr; spot urine albumin to creatinine ratio > 30 mmol/mg; or
≥ 2 + on dipstick
Haematuria, red cell casts. Urine sediment showing > 5 red cells per high power 33 70
field or red cell casts
EULAR = European League Against Rheumatism; IgA = immunoglobulin A; PRES = Pediatric Rheumatology Society.

CLINICAL FEATURES Renal abnormalities follow the onset of typical rash

within 1–3 months. The spectrum of manifestations ranges
Henoch–Schonlein purpura is characterised by a classic tet- from microscopic haematuria and mild proteinuria to the less
rad of non-thrombocytopaenic palpable purpura, arthritisor common nephrotic syndrome, acute nephritic syndrome,
arthralgias, GI and renal involvement. hypertension, or renal failure. Gross haematuria and mild
Cutaneous involvement is the most common presenta- proteinuria following an upper respiratory tract infection
tion. The onset is acute and symptoms may appear sequen- similar to IgA nephropathy is common in HSP.6,8
tially. Cutaneous lesions start as maculopapular eruptions Arthralgia or arthritis involving only a few joints occurs
that become palpable and purpuric. Dependent areas and in 50–80% of children with HSP. Typically, non-migratory,
pressure points, especially lower extremities and buttocks, non-destructive symmetrical polyarthralgias involving the
may favour the localisation of rashes. The lesions fade leaving knees and ankles are seen. Joint involvement is more com-
a brownish colour that persists for weeks. Other morpho- monly seen in adults than in children. The joint disease
logical patterns, such as vesicles, erythema multiforme-like resolves within a few days to a week. Occasionally, arthritis
lesions and haemorrhagic bullae, may also be seen. Lesions may precede the appearance of the rash by 1 or 2 days.15,16
on the face and ears are common in infants and they may Central nervous system and the lungs are rarely
develop marked oedema of the face, scalp and extremities. involved.
The disease seems to be mediated by the activation of alternate Indian data on the major clinical features of HSP are
pathway of complement by large IgA containing immune summarised in Table 3.
complexes. This association may explain the predilection of
skin lesions for the lower legs and buttocks in ambulatory
children and the sacrum, buttocks and ears in infants who
often lie supine as gravity causes immune complexes to PATHOLOGY
deposit and incite inflammation in dependent areas.15,16
Gastrointestinal manifestations occur usually within a Leucocytoclastic vasculitis is the pathognomic lesion of
week after the onset of rash and are due to vasculitis involving HSP and is seen in the dermal capillaries and postcapillary
the splanchnic circulation. Abdominal pain is the most com- venules in the skin. Deposition of IgA (principally IgA1) is
mon symptom. Other symptoms include nausea, vomiting, characteristic. If the biopsy is obtained from the centre of a
haematemesis, melena, and haematochezia. Rare manifes- lesion, it may stain negative for IgA due to the presence of
tations such as intussuception (ileoileal), intestinal perfora- proteolytic enzymes.1 In a study by Murali et al. on 71 chil-
tion, massive GI bleeding, acute acalculous cholecystitis, dren with HSP, the sensitivity of histopathology was 80.4%
haemorrhagic ascites, pancreatitis, and biliary cirrhosis may and was not influenced by the duration of the lesion. The
occur.6 direct immunofluorescence test was a useful adjunct to
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HSP vasculitis Review Article 95

Table 3 Major clinical manifestations of Henoch-Schonlein purpura in Indian children

Clinical feature Bagga et al.3 Kumar et al.17 Bagai et al.18 Grover et al.4 Sarkar et al.19
n = 47 n = 45 n = 36 n = 30 n = 90
Purpura (%) 95.7 100 100 100 –
Joint pain (%) 46.8 60 72.2 86.7 –
Gastrointestinal (%) 63.8 78 58.3 80 7.7
Renal (%) 51.1 31 47.2 30 10

histopathology, especially if done within 48 hours.20 In the outcomes of 46 adults and 116 children with HSP, cutane-
kidneys, glomerulonephritis ranges from focal and segmen- ous lesions were the principal initial manifestation in both
tal lesions to severe crescentic disease. The principal lesion groups. However, adults had a lower frequency of abdomi-
is an endocapillary proliferative glomerulonephritis with an nal pain and a higher frequency of joint symptoms and
increase in the endothelial and mesangial cells. All gradations severe renal involvement. Adults also required aggressive
of severity may be present in the same biopsy. Fluorescence therapy with glucocorticoids or cytotoxic agents, or both.
microscopy confirms the deposits of immunoglobulin, prin- There was complete recovery in 94% of children and 89%
cipally IgA, in the most involved glomeruli. Dense deposits of adults.22
in the mesangium and occasionally in the subendothelial and Lin and Huang reported that leucocytosis, thrombocy-
paramesangial regions are present on electron microscopy.1 tosis and elevated levels of serum C-reactive protein were
more common in children, whereas elevated serum IgA and
cryoglobulin levels were more common in adults in a
Chinese population. Henoch–Schonlein purpura in adults
DIFFERENTIAL DIAGNOSIS may represent a more severe form of the disease with a
higher frequency of significant renal involvement and risk
Immune thrombocytopaenic purpura, acute poststreptococcal of progressive kidney disease, but without the other mani-
glomerulonephritis, leukaemia, systemic lupus erythemato- festations of HSP.23
sus, septicaemia, disseminated intravascular coagulation, the
haemolytic–uraemic syndrome and other types of vasculitis
should be considered in the differential diagnosis. The MEFV
can also mimic or occur with HSP.1,15 INVESTIGATIONS
Common causes of an acute surgical abdomen with
abdominal pain and GI tract bleeding must be considered. Henoch–Schonlein purpura is a clinical diagnosis. Labora-
Intussusception can be considered if there is a tender abdom- tory investigations include complete blood count. The plate-
inal mass, and abdominal tenderness with an elevated serum let count is normal or increased. Leucocytosis of up to
amylase level may suggest acute pancreatitis. 20,000 white blood cells/mm3 (20 × 109/L) with a left shift
Infantile acute haemorrhagic oedema (Finkelstein– is seen in some children. Normochromic anaemia is often
Seidlmayer syndrome) affects children below 2 years of age related to GI blood loss, confirmed by a positive stool guaiac
and is characterised by acute onset of fever, purpura, ecchy- examination in 80% of the children who have abdominal
moses and inflammatory oedema of the limbs, ears, and complaints. Antinuclear antibody, anti-neutrophilic cytoplas-
face without visceral involvement. Attacks may recur. Leuco- mic antibodies and rheumatoid factor are not characteristic.
cytoclastic vasculitis with occasional demonstration of the Urinary abnormalities usually demonstrate a direct correla-
perivascular IgA deposition is noted in the biopsy.21 tion with the severity of the renal proliferative changes.
Proteinuria, sometimes severe enough to result in hypoalbu-
minaemia, may occur.8,16,24
C1q, C3 and C4 are usually normal. Activation of the
HENOCH–SCHONLEIN PURPURA IN ADULTS alternative complement pathway during the acute illness
is confirmed by the presence of C3d, low levels of total
Henoch–Schonlein purpura is uncommon in adults, with a haemolytic complement and decreased concentrations of
reported incidence of 0.12 cases per 100,000 persons. There properdin and factor B in the serum. Plasma levels of Von
is no gender predilection. In a study of clinical features and Willebrand factor antigen are elevated, indicating endothelial
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96 Indian Journal of Rheumatology 2012 May; Vol. 7, No. 1 (Suppl) Kamath and Rao

cell damage. Circulating IgA containing immune complexes 2 mg/kg/day for 12 weeks and classes IV and V received
and cryoglobulins may be present.25 azathioprine for 9 months subsequent to the treatment for
Plain radiographs may show dilated loops of bowel in class III. All patients received angiotensin converting enzyme
children with abdominal involvement. Specific GI abnor- (ACE) inhibitors regardless of their blood pressure values.
malities in children with abdominal complaints and testicu- Eighteen patients presenting with severe HSPN, defined
lar torsion can be confirmed by ultrasound. Intussusception as heavy proteinuria and/or decreased renal function, were
can be identified on a barium study and relieved by it if evaluated. At presentation, seven of the patients had impaired
performed early in the course. Magnetic resonance imaging renal function with glomerular filtration rate (GFR) below
and magnetic resonance angiography can define the extent 75 mL/min/1.73 m2. With the presented treatment schema,
of cerebral vasculitis. Punch biopsy of a cutaneous lesion all GFR returned to normal at the end of 4 years of follow-
may assist in the diagnosis of difficult cases by demonstrat- up. There was no proteinuria in any of the patients; only
ing a leucocytoclastic vasculitis characterised by the depo- eight had microscopic haematuria. This preliminary study
sition of IgA and C3. suggests a stepwise treatment according to the renal histol-
A renal biopsy is indicated only in children with persis- ogy. The excellent results with complete disappearance of
tent or significant renal manifestations. Indications for diag- proteinuria and normal renal function justify the use of
nostic renal biopsy in children with HSP are hypertension, the aforementioned immunosuppressive protocol with ACE
oliguria, raised creatinine, nephritic/nephrotic presentation, inhibition.30
heavy proteinuria (Ua:Ucr persistently > 100 mg/mmol) on Renal transplantation has been successful in some chil-
an early morning urine sample at 4 weeks and persistent dren with renal failure.1
proteinuria (not declining) after 4 weeks.8

COURSE OF THE DISEASE AND PROGNOSIS

TREATMENT
Henoch–Schonlein purpura runs its entire course within
The acute, active phase of HSP resolves spontaneously in 4 weeks of the onset in most cases. One half of the patients
most patients and the primary goal of the physician is to have at least one recurrence, each episode is usually similar,
reassure the family about the benign nature of the disease but briefer and milder.15 Exacerbations may be spontaneous
and to provide symptomatic treatment. The patient should or coincide with repeated respiratory tract infections. The
be monitored for complications which usually occur within severity of the cutaneous leucocytoclastic vasculitis does
4 weeks of initial presentation. Joint pain and painful soft not correlate with visceral involvement.
tissue oedema usually respond to acetaminophen or non- Prognosis is excellent for most children. Significant
steroidal anti-inflammatory drugs. Prednisone, 1–2 mg/kg morbidity or mortality is associated with GI tract lesions in
per day, may be necessary to hasten their resolution. Anti- the short-term and renal disease in the long-term. The devel-
inflammatory agents should be avoided in patients with opment of renal disease within the first 6 months after onset
extensive renal involvement. Corticosteroids may lead to the or the occurrence of numerous exacerbations associated with
resolution of abdominal pain within 24 hours without seri- nephropathy suggests a poor prognosis for renal function.
ous complications. Pulmonary haemorrhage is an extremely Additional poor prognostic factors are decreased factor XIII
rare and sometimes fatal complication. This requires aggres- activity; hypertension; renal failure at onset; and if a renal
sive immunosuppressive treatment, combining IV methyl- biopsy had been performed, an increased number of glomer-
prednisolone and cyclosporin (or another immunosuppressive uli with crescents; macrophage infiltration; and tubulointer-
agent) and supportive care.26–28 stitial disease. The worst outcome is associated with the
Chartapisak et al. systematically reviewed all published presence of the nephrotic or nephritic syndrome at onset.31
randomised controlled trials for the prevention or treatment Henoch–Schonlein purpura accounts for less than 1% of
of renal involvement in HSP and suggested that prophylac- children with renal failure from all causes. Patients who
tic therapy with corticosteroids did not prevent the onset of have had clinical nephritis should be followed closely for at
HSPN but was effective in treating it if present.29 least 5 years.32–34
Altugan and coworkers studied all patients with severe The Alder Hey HSP monitoring pathway was developed
renal involvement who were biopsied and a treatment plan was for the screening of renal outcome in HSP, after following
assigned: Class II received oral steroids, class III (with cres- up a cohort of 102 patients for 5 years. A normal urine anal-
centic nephritis) received additional oral cyclophosphamide ysis on day 7 had a 97% (CI 90–99%) negative predictive
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HSP vasculitis Review Article 97

value in predicting a normal renal outcome. A 6-month Gastroenterol Res Pract 2010; 2010: 597–648. [Epub 2010,
monitoring period for all patients presenting with HSP was May 23.]
devised according to the urine findings on day 7 and those 7. Yang YH, Huang MT, Lin SC, Lin YT, Tsai MJ, Chiang BL,
patients with a high-risk of developing HSPN were inten- et al. Increased transforming growth factor-beta (TGF-beta)-
sively monitored.34 secreting T cells and IgA anti-cardiolipin antibody levels during
acute stage of childhood Henoch–Schönlein purpura. Clin Exp
Immunol 2000; 122: 285–90.
8. Lau KK, Suzuki H, Novak J, Wyatt RJ. Pathogenesis of
CONCLUSION Henoch–Schönlein purpura nephritis. Pediatr Nephrol 2010;
25: 19–26.
Henoch–Schonlein purpura is the commonest acute vasculi- 9. Kawasaki K, Komura H, Nakahara Y, Shirashi M, Higashida
tis of childhood, typified by purpuric rash on the extremities M, Ouchi K, et al. Factor XIII in Henoch–Schönlein purpura
and buttocks, joint and abdominal pain. It is a self-limiting, with isolated gastrointestinal symptoms. Pediatr Int 2006; 48:
single episode illness in one half of cases. Patients must be
413–5.
followed up for at least 6 months for potential HSPN and its
10. Dudley J, Afifi E, Gardner A, Tizard EJ, McGraw ME.
complications and for 5 years if nephritis is documented.
Polymorphism of the ACE gene in Henoch–Schönlein pur-
Aggressive and early treatment of HSPN can preserve renal
pura nephritis. Pediatr Nephrol 2000; 14: 218–20.
function.
11. Peru H, Soylemezoglu O, Gonen S, Cetinyurek A, Bakkaloğlu
SA, Buyan N, et al. HLA class 1 associations in Henoch
Schönlein purpura: increased and decreased frequencies. Clin
ACKNOWLEDGEMENT Rheumatol 2008; 27: 5–10.
12. Gershoni-Baruch R, Broza Y, Brik R. Prevalence and sig-
Source of funding: None. nificance of mutations in the familial Mediterranean fever
Conflict of interest: None. gene in Henoch–Schönlein purpura. J Pediatr 2003; 143:
658–61.
13. Ozen S, Ruperto N, Dillon M, Bagga A, Barron K, Davin JC,
et al. EULAR/PRES endorsed consensus criteria for the clas-
REFERENCES sification of childhood vasculitides. Ann Rheum Dis 2006;
65: 936–41.
1. Brogan P, Bagga A. Leukocytoclastic vasculitis. In: Textbook of 14. Mills JA, Michel BA, Bloch DA, Calabrese LH, Hunder GG,
Pediatric Rheumatology 6th ed. Cassidy et al., eds. Philadelphia Arend WP, et al. The American College of Rheumatology
Saunders 2011: 483–97. 1990 criteria for the classification of Henoch–Schönlein pur-
2. Ozen S, Pistorio A, Iusan SM, Bakkaloglu A, Herlin T, Brik R, pura. Arthritis Rheum 1990; 33: 1114–21.
et al. Paediatric Rheumatology International Trials Organisation 15. Dedeoglu F, Sundel RP. Vasculitis in children. Pediatr Clin
(PRINTO). EULAR/PRINTO/PRES criteria for Henoch– North Am 2005; 52: 547–75.
Schönlein purpura, childhood polyarteritis nodosa, childhood 16. Palit A, Inamadar AC. Childhood cutaneous vasculitis: a
Wegener granulomatosis and childhood Takayasu arteritis: comprehensive appraisal. Indian J Dermatol 2009; 54: 110–7.
Ankara 2008. Part II: Final classification criteria. Ann Rheum 17. Kumar L, Singh S, Goraya JS, Uppal B, Kakkar S, Walker R,
Dis 2010: 798–806. et al. Henoch–Schonlein purpura: the Chandigarh experience.
3. Bagga A, Kabra SK, Srivastava RN, Bhuyan UN. Henoch– Indian Pediatr 1998; 35: 19–25.
Schonlein syndrome in northern Indian children. Ind Pediatr 18. Bagai A, Albert S, Shenoi SD. Evaluation and therapeutic
1991; 28: 1153–7. outcome of palpable purpura. Indian J Dermatol Venereol
4. Grover N, Sankhyan N, Bisht JP. A five year review of clini- Leprol 2001; 67: 320–3.
cal profile in HSP. J Nepal Med Assoc 2007; 46: 62–5. 19. Sarkar S, Mondal R, Nandi M, Ghosh A. Trends of childhood
5. Singh S, Aulakh R. Kawasaki disease and Henoch Schonlein vasculitides in eastern India. Indian Pediatr 2011; 48: 814.
purpura: changing trends at a tertiary care hospital in north 20. Murali NS, George R, John GT, Chandi SM, Jacob M,
India (1993–2008). Rheumatol Int 2010; 30: 771–4. Jeyaseelan C, et al. Problems of classification of Henoch
6. Sohagia AB, Gunturu SG, Tong TR, Hertan HI. Henoch– Schönlein purpura: an Indian perspective. Clin Exp Dermatol
Schonlein purpura—a case report and review of the literature. 2002; 27: 260–3.
[Downloaded free from http://www.indianjrheumatol.com on Sunday, July 21, 2019, IP: 223.228.56.62]

98 Indian Journal of Rheumatology 2012 May; Vol. 7, No. 1 (Suppl) Kamath and Rao

21. Goraya JS, Kaur S. Acute infantile hemorrhagic edema and Henoch–Schönlein purpura in a child. Clin Rheumatol 2008;
Henoch–Schönlein purpura: is IgA the missing link? J Am 27: 803–5.
Acad Dermatol 2002; 47: 801–2. 29. Chartapisak W, Opastiraku S, Willis NS, Craig JC, Hodson
22. Blanco R, Martinez-Taboada VM, Rodriguez-Valverde V, EM. Prevention and treatment of renal disease in Henoch–
García-Fuentes M, González-Gay MA. Henoch–Schönlein Schönlein purpura: a systematic review. Arch Dis Child 2009;
purpura in adulthood and childhood: two different expres- 94: 132–7.
sions of the same syndrome. Arthritis Rheum 1997; 40: 30. Altugan FS, Ozen S, Aktay-Ayaz N, Güçer S, Topaloğlu R,
859–64. Düzova A, et al. Treatment of severe Henoch–Schönlein
23. Lin SJ, Huang JL. Henoch–Schönlein purpura in Chinese nephritis: justifying more immunosuppression. Turk J Pediatr
children and adults. Asian Pac J Allergy Immunol 1998; 16: 2009; 51: 551–5.
21–5. 31. Ronkainen J, Ala-Houhala M, Huttunen NP, Jahnukainen T,
24. Muller D, Greve D, Eggert P. Early tubular proteinuria and Koskimies O, Ormälä T, et al. Outcome of Henoch–Schönlein
the development of nephritis in Henoch–Schönlein purpura. nephritis with nephrotic-range proteinuria. Clin Nephrol 2003;
Pediatr Nephrol 2000; 15: 85–9. 60: 80–4.
25. Casonato A, Pontara E, Bertomoro A, Ossi E, Vincenti M, 32. Kawasaki Y, Suzuki J, Sakai N, Nemoto K, Nozawa R, Suzuki S,
Girolami A, et al. Abnormally large von Willebrand factor et al. Clinical and pathological features of children with
multimers in Henoch–Schönlein purpura. Am J Hematol 1996; Henoch–Schönlein purpura nephritis: risk factors associated
51: 7–11. with poor prognosis. Clin Nephrol 2003; 60: 153–60.
26. Rees L, Webb NJA, Brogan P. Vasculitis. In: Paediatric 33. Goldstein AR, White RH, Akuse R, Chantler C. Long-term
Nephrology (Oxford handbook). Rees L, Webb NJA, Brogan follow-up of childhood Henoch–Schönlein nephritis. Lancet
PA, eds. Oxford: Oxford University Press 2007. 1992; 339: 280–2.
27. Gedalia A. Henoch Schonlein Purpura. Curr Rheumatol Rep 34. Watson L, Richardson AR, Holt RC, Jones CA, Beresford MW.
2004; 6: 195–202. Henoch Schonlein purpura—a 5-year review and proposed path-
28. Matsubayashi R, Matsubayashi T, Fujita N, Yokota T, way. PLoS One 2012; 7: e29512. [Epub 2012, Jan 3. PubMed
Ohro Y, Enoki H. Pulmonary hemorrhage associated with PMID: 22235302; PubMed Central PMCID: PMC3250434.]