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Year : 2021  |  Volume : 9  |  Issue : 2  |  Page : 120-122

Adult-onset immunoglobulin A vasculitis

Department of General Medicine, MR Medical College, Kalaburagi, Karnataka, India

Date of Submission28-Jan-2020
Date of Decision09-Mar-2020
Date of Acceptance09-May-2020
Date of Web Publication21-Apr-2021

Correspondence Address:
Dr. Megha Amarapur
D/O Basavaraj Amarapur, Basava Nivas, Plot No. 178/A, Maka Layout, Jewargi Colony, Kalaburagi - 585 103, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/AJIM.AJIM_6_20

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We describe a case of adult-onset immunoglobulin A vasculitis (IgA vasculitis) presenting with cutaneous, renal and gastrointestinal manifestations without any evidence of articular involvement. Injection rituximab therapy led to complete resolution of the symptoms. IgA vasculitis is more common in children than in adults. However, in adults, it frequently takes aggressive course as worse renal outcomes compared to children. Systemic steroids are recommended for moderate-to-severe IgA vasculitis. However, upcoming evidence suggests good response with B cell-directed therapies such as rituximab.

Keywords: Adult onset, immunoglobulin A vasculitis, injection rituximab, palpable rash

How to cite this article:
Amarapur M, Biradar S. Adult-onset immunoglobulin A vasculitis. APIK J Int Med 2021;9:120-2

How to cite this URL:
Amarapur M, Biradar S. Adult-onset immunoglobulin A vasculitis. APIK J Int Med [serial online] 2021 [cited 2021 Jun 12];9:120-2. Available from: https://www.ajim.in/text.asp?2021/9/2/120/314198

  Introduction Top

Immunoglobulin A vasculitis (IgA vasculitis [IgAV]), formerly called Henoch–Schonlein purpura [HSP]),[1] is the most common form of systemic vasculitis in children. Ninety percent of cases occur in the pediatric age group. IgAV (HSP) is self-limited in the great majority of cases. IgAV (HSP) is less common in adults.[2] Adult patients with IgAV (HSP) present with clinical manifestations similar to those that occur in children, and the two main differences between adults and children with IgAV (HSP) are intussusception is rare in adults and they are at an increased risk for developing significant renal involvement including end-stage kidney disease.[3]

It is more common in males (male:female: 1.2:1 to 1.8:1). IgAV (HSP) occurs primarily in the fall, winter, and spring and is rare in summer, possibly explained by the association of IgAV (HSP) with infections. Approximately one-half of the cases of IgAV (HSP) are preceded by an upper respiratory tract infection, especially those caused by Streptococcus.

  Case Repot Top

A 49-year-old female presented with a 10-day history of multiple erythematous, nonpruritic, pinpoint rashes initially involving both feet, which progressed upward over 5 days up to the thighs [Figure 1] and lower abdomen, and also involving the forearms. She also complained of constant colicky, diffuse abdominal pain with postprandial aggravation for 3 days without any associated hematemesis, hematochezia, and watery stools. She did not have any joint pains, pedal edema, and reduced frequency of urine. The patient also did not have recurrent sinonasal symptoms, hearing or visual impairment, breathlessness, history of asthma, paresthesia, or weakness of extremities. She also did not complain of recurrent oral ulcerations, malar rash, and dryness of mouth or eyes. She had a comorbid hypothyroidism for which she was on tablet thyroxin 125 μg/day supplementation. On physical examination, there was nonblanching, palpable, purpuric rash involving both the upper and lower extremities with truncal involvement. Abdominal and musculoskeletal examinations were normal. Laboratory test results are shown in [Table 1].
Figure 1: Palpable purpuric rashes on the legs

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Table 1: Lab parameters of the patient

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Biopsy of the skin lesions showed leukocytoclastic vasculitis with direct immunofluorescence (DIF) positive for IgA and C3 deposits. Renal biopsy was not done as the patient did not give consent for the same. Esophagogastroduodenoscopy showed severe erosive gastritis and duodenitis. Ultrasonography and contrast computed tomography of the abdomen revealed thickened bowel loops without any evidence of obstruction.

The patient was diagnosed as IgA vasculitis, and she also fit the 2005 European League Against Rheumatism (EULAR)/Paediatric Rheumatology European Society (PRES)/Paediatric Rheumatology International Trials Organisation (PRINTO) criteria. She was started on tablet mycophenolate mofetil 500 mg thrice daily with oral prednisolone starting from 0.5 mg/kg body weight tapered over 4 weeks to 5 mg/day. In view of persistence hematuria (indicating persistent of disease), although there is little evidence of B-cell-directed therapies in IgA vasculitis, because of the aggressive nature of the disease with renal and gastrointestinal (GI) involvement, she was treated with injection rituximab 600 mg once a week for 4 weeks. She had complete resolution of her symptoms, and her urine examination also normalized. Her diet was advanced to regular diet.

  Discussion Top

IgAV (HSP) is primarily a childhood disease that occurs between the ages of 3 and 15 years.[4] IgAV (HSP) is less common in adults,[2] with an estimated annual incidence of 5/100,000 adults in Slovenia.[5] In two retrospective studies from Spain and Korea, 25%–30% of patients with IgAV (HSP) were adults. Studies showed that adults had significantly worse renal outcomes compared with children.[3]

American College of Rheumatology 1990 criteria to classify for the diagnosis of IgAV (HSP)[6] are as follows:

  1. Palpable purpura
  2. Age at onset 20 years
  3. Acute abdominal pain
  4. Biopsy showing granulocytes in the walls of small arterioles and/or venules.

These criteria were based on a comparison between 85 patients with IgAV (HSP) and 722 adult patients with other forms of vasculitis. Two or more of the criteria had a sensitivity and specificity of approximately 90% in separating adult patients with IgAV (HSP) from those with other causes of vasculitis.

In 2005, pediatric consensus criteria were developed by the EULAR, the PRES, and the PRINTO.[7] These criteria are more appropriate for pediatric settings to rule out more common causes such as gastroenteritis or appendicitis. The mandatory criterion is purpura (usually palpable and in clusters) or petechiae, with lower limb predominance and without thrombocytopenia or coagulopathy along with one or more of the following:

  1. Abdominal pain (usually diffuse, with acute onset)
  2. Arthritis or arthralgia
  3. Renal involvement (proteinuria and hematuria)
  4. Leukocytoclastic vasculitis or proliferative glomerulonephritis, with predominant IgA deposition.

Diagnosis is typically based on clinical manifestations of the disease.

Treatment – Patients with severe abdominal pain who fail to respond to nonsteroidal anti-inflammatory drugs are treated with oral prednisone (1–2 mg/kg/day) for children or adults with a maximum dose of 60–80 mg/day.[8] In patient who does not tolerate oral steroids, equivalent doses of intravenous methylprednisolone (0.8–1.6 mg/kg/day, maximum dose of 64 mg/day), can be used.

Children who present with more severe renal involvement (i.e., proteinuria of >1 g/day, nephrotic syndrome, or evidence of crescentic glomerulonephritis on renal biopsy) are managed with three pulses of intravenous methylprednisolone 1 g/1.73 m2, followed by oral prednisone 30 mg/m2 once daily for 1 month, and then oral prednisone 30 mg/m2 every other day for 2 months.[9]

If renal biopsy done after 4–6 months of therapy, demonstrates evidence of persistent, active inflammation (i.e., proliferative glomerulonephritis), then some clinicians would choose to treat with either cyclophosphamide or mycophenolate. The evidence for either therapy is based on very limited data. Rituximab seems to be a safe and useful agent in inducing disease remission and reducing previous immunosuppressive treatment in IgA vasculitis. Rituximab is also useful in pediatric and adult patients resistant or refractory to glucocorticoids or other immunosuppressive drugs and in those patients in whom these agents are contraindicated. The efficacy of rituximab in adult IgA-vasculitis has been reported in few cases.[8]

  Conclusion Top

Our case highlights that IgA vasculitis commonly occurring in children can occur in adults as well, and in adults, it usually has worse outcome involving more commonly renal and GI symptoms, and in case of relapse of disease with steroids and immunosuppressive drugs, injection rituximab is given.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum 2013;65:1-11.  Back to cited text no. 1
Blanco R, Martínez-Taboada VM, Rodríguez-Valverde V, García-Fuentes M, González-Gay MA. Henoch-Schönlein purpura in adulthood and childhood: Two different expressions of the same syndrome. Arthritis Rheum 1997;40:859.  Back to cited text no. 2
Kang Y, Park JS, Ha YJ, Kang MI, Park HJ, Lee SW, et al. Differences in clinical manifestations and outcomes between adult and child patients with Henoch-Schönlein purpura. J Korean Med Sci 2014;29:198.  Back to cited text no. 3
Piram M, Mahr A. Epidemiology of immunoglobulin A vasculitis (Henoch-Schönlein): Current state of knowledge. Curr Opin Rheumatol 2013;25:171.  Back to cited text no. 4
Hočevar A, Rotar Z,Ostrovršnik J, Jurčić V, Vizjak A, Dolenc Voljč M, et al. Incidence of IgA vasculitis in the adult Slovenian population. Br J Dermatol 2014;171:524-7.  Back to cited text no. 5
Mills JA, Michel BA, Bloch DA, Calabrese LH, Hunder GG, Arend WP, et al. The American College of Rheumatology 1990 criteria for the classification of Henoch-Schönlein purpura. Arthritis Rheum 1990;33:1114.  Back to cited text no. 6
Ozen S, Pistorio A, Iusan SM, Bakkaloglu A, Herlin T, Brik R, et al. EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: Final classification criteria. Ann Rheum Dis 2010;69:798.  Back to cited text no. 7
Hernández-Rodríguez J, Carbonell C, Mirón-Canelo JA, Diez-Ruiz S, Marcos M, Chamorro AJ. Rituximab treatment for IgA vasculitis: A systematic review. Autoimmun Rev 2020;19:102490.  Back to cited text no. 8
Davin JC, Coppo R. Pitfalls in recommending evidence-based guidelines for a protean disease like Henoch-Schönlein purpura nephritis. Pediatr Nephrol 2013;28:1897.  Back to cited text no. 9


  [Figure 1]

  [Table 1]


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