Publish date: Posted on
Last updated: May 31, 2022
Keywords #
IgA vasculitis
Henoch-Schönlein purpura (HSP)
palpable purpura
leukocytoclastic vasculitis
Diagnosis #
IgA vasculitis (IgAV), also known as Henoch-Schönlein purpura, is a type of systemic vasculitis that typically manifests with palpable purpura, arthralgias, abdominal pain, and renal disease. IgAV is the most common systemic vasculitis seen in children and often has a relatively benign course. Although it is seen more frequently in children, adults can also be affected and have higher rates of complications, including renal failure.1 It is thought to be caused by an interplay of genetic, environmental, and immunologic factors leading to deposition of IgA immune complexes into vessel walls.[2]
Key Concepts #
- IgA vasculitis is the most common systemic vasculitis in children.
- It is characterized as a tetrad of palpable purpura, arthralgias, abdominal pain, and renal disease.
- It typically resolves spontaneously.
- Treatment is generally supportive; however, some patients may benefit from systemic steroids or dapsone.
- Patients must be checked at baseline and following diagnosis for renal complications (elevated creatinine, BUN or hematuria/proteinuria). If this is identified, referral to nephrology is warranted.
Epidemiology #
IgA vasculitis predominantly affects children, with estimates of the annual incidence ranging from 10 to 27 in 100,000 children compared to 1 to 5 per 100,000 in the adult population.[2,3,4,5] It is often precipitated by an upper respiratory infection and occurs most frequently in the winter.[3]
Clinical Features #
- IgA vasculitis is characterized as a tetrad of palpable purpura, arthralgias, abdominal pain, and renal disease.
- Palpable purpura and petechiae are the most common initial presenting symptom seen in IgA vasculitis. The lower extremities and buttocks are most commonly affected.[3] Other manifestations include joint involvement, manifesting as arthralgias, gastrointestinal involvement, manifesting as abdominal pain, and renal involvement, manifesting as hematuria.[3,6]
Rare but important complications of IgA vasculitis to be aware of include intussusception, which is seen more frequently in children, and renal failure, which is more common in adults with IgAV.[3,6]
Differential Diagnoses #
- Acute hemorrhagic edema of infancy
- Thrombocytopenic purpura
- Cryoglobulinemia
- Small Vessel Vasculitis
- Erythema elevatum diutinum
- Capillaritis
- Erythema multiforme
- Meningococcemia
Diagnostic Workup #
The diagnosis can be made clinically based on the presence of palpable purpura along with evidence of other organ involvement (i.e. hematuria, abdominal pain, or arthralgias). Biopsy of lesions for direct immunofluorescence (DIF) can be useful for confirmation and in cases where the diagnosis is not clear.
On microscopy, the appearance will be consistent with a leukocytoclastic vasculitis. IgAV can be differentiated from other vasculitides using DIF of lesional skin, which will display deposition of IgA and C3 complexes in the dermal blood vessels.6 This is considered the gold standard for diagnosis.
Urinalysis (UA) and complete metabolic panel (CMP) should be obtained at presentation to assess for renal involvement and to establish a baseline. It is also important to obtain a complete blood count (CBC) and PT/PTT to rule out thrombocytopenia and other coagulopathies in patients with petechiae and purpura. Abdominal ultrasound is recommended in patients with suspected intussiception.3 Patients should also have close follow-up with routine UA to monitor for worsening renal function in some cases for up to a year.[6]
Treatment #
Treatment of IgA vasculitis is generally supportive as it typically resolves without intervention. Nonsteroidal anti-inflammatory drugs, such as naproxen, can be useful in patients with arthralgias and abdominal pain.
Severe cases and those with extensive organ involvement may warrant the use of systemic steroids, which may reduce the risk of complications and hasten recovery. In cases refractory to oral steroids, the use of dapsone, colchicine, cyclosporine, and other immunosuppressive agents may be beneficial.[2,6]
References #
- Audemard-Verger A, Terrier B, Dechartres A, et al. Characteristics and Management of IgA Vasculitis (Henoch-Schönlein) in Adults: Data From 260 Patients Included in a French Multicenter Retrospective Survey. Arthritis Rheumatol. 2017. doi:10.1002/art.40178
- Audemard-Verger A, Pillebout E, Guillevin L, Thervet E, Terrier B. IgA vasculitis (Henoch-Shönlein purpura) in adults: Diagnostic and therapeutic aspects. Autoimmun Rev. 2015;14(7):579-585. doi:10.1016/j.autrev.2015.02.003
- Trnka P. Henoch-Schönlein purpura in children. J Paediatr Child Health. 2013. doi:10.1111/jpc.12403
- Penny K, Fleming M, Kazmierczak D, Thomas A. An epidemiological study of Henoch-Schönlein purpura. Paediatr Care. 2013. doi:10.7748/paed2010.12.22.10.30.c8135
- Hočevar A, Rotar Z, Ostrovršnik J, et al. Incidence of IgA vasculitis in the adult Slovenian population. Br J Dermatol. 2014;171(3):524-527. doi:10.1111/bjd.12946
- Audemard-Verger A, Terrier B, Dechartres A, et al. Characteristics and Management of IgA Vasculitis (Henoch-Schönlein) in Adults: Data From 260 Patients Included in a French Multicenter Retrospective Survey. Arthritis Rheumatol. 2017;69(9):1862-1870. doi:10.1002/art.40178
