A child with immunoglobulin a vasculitis and hemorrhagic shock: An unusual presentation

Shahad Alansari; Alhanouf Alsaleem

doi:10.4103/ara.ara_3_21

CASE REPORT

Year : 2021 | Volume : 1 | Issue : 1 | Page : 23-26

A child with immunoglobulin a vasculitis and hemorrhagic shock: An unusual presentation

Shahad Alansari, Alhanouf Alsaleem
Department of Pediatrics, Section of Pediatric Rheumatology, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia

Date of Submission	21-Jun-2021
Date of Decision	26-Aug-2021
Date of Acceptance	02-Sep-2021
Date of Web Publication	13-Nov-2021

Correspondence Address:
Dr. Alhanouf Alsaleem
Department of Pediatrics, Section of Pediatric Rheumatology, King Faisal Specialist Hospital and Research Center, Riyadh
Saudi Arabia

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ara.ara_3_21

Abstract

Immunoglobulin A (IgA) vasculitis, formerly known as Henoch–Schonlein purpura, is the most common vasculitis in children affecting small-sized vessels. Although IgA vasculitis is a self-limiting disease, a small number of patients can present with severe life-threatening complications. Here, we described a pediatric patient with final diagnosis of IgA vasculitis complicated with massive gastrointestinal bleeding and hemorrhagic shock, who recovered with supportive treatment and combined immunosuppressive drugs.

Keywords: Gastrointestinal hemorrhage, Henoch–Schonlein purpura, immunoglobulin A vasculitis, leukocytoclastic vasculitis

How to cite this article:
Alansari S, Alsaleem A. A child with immunoglobulin a vasculitis and hemorrhagic shock: An unusual presentation. Ann Rheumatol Autoimmun 2021;1:23-6

How to cite this URL:
Alansari S, Alsaleem A. A child with immunoglobulin a vasculitis and hemorrhagic shock: An unusual presentation. Ann Rheumatol Autoimmun [serial online] 2021 [cited 2022 May 28];1:23-6. Available from: http://www.ara.ssr.com/text.asp?2021/1/1/23/330431

Introduction

Immunoglobulin A vasculitis (IgAV, formerly Henoch–Schonlein purpura [HSP]) is the most common vasculitis in pediatric age group with an incidence rate of 10–20/100,000 population.^[1] It typically occurs between the age group of 3 and 17 years, more common in boys than girls. The histopathological finding of leukocytoclastic vasculitis with immunoglobulin A (IgA) antibody immune complex deposition in the wall of small vessels is the hallmark of disease pathogenesis. Cutaneous nonthrombocytopenic palpable purpura, abdominal pain, and arthritis are the classical triad of IgAV.^[1] Palpable purpura is the commonest manifestation seen in nearly all patients (95%–100%). Typically involve the dependent pressure bearing areas, specifically lower-limb extremities and buttocks regions. Coexistence of gastrointestinal (GI) occurs in 50%–75% of patients.^[1],[2] GI symptoms vary from mild self-resolving rarely require treatment intervention to life-threatening complication. GI bleeding is common, with a reported incidence of 20%–30% of patients with final diagnosis of IgAV.^[1] This report represents a challenging case of IgAV complicated with massive lower GI bleeding and hemorrhagic shock who fortunately showed recovery with supportive treatment and combined immunomodulatory therapy.

Case Report

A 13-year-old boy, previously healthy, presented with a month history of diffuse colicky abdominal pain associated with lower-limb purpuric rash [Figure 1]. Basic blood work-up, inflammatory markers, urinalysis, and occult stool work-up were unremarkable. He was admitted at a local hospital; a diagnosis of IgAV was made. He was managed with high dose of prednisone for severe abdominal pain. The patient was discharged with good response and planned for steroid tapering. One week later, he presented to the emergency department with clinical picture of unilateral testicular torsion; Doppler ultrasonography showed decreased blood flow to left testes. Exploratory surgery was performed and revealed a picture of orchitis. This was followed by severe abdominal pain, non-bloody vomiting and progressive diffuse purpuric rash involved upper and lower limbs. He received a high dose of methylprednisolone (60 mg/day) for 3 days followed by tapering plan of prednisone.

Figure 1: Lower-limb purpuric rash

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By the time of presentation to our hospital, he was on stable clinical condition and maintained on high dose of prednisone (60 mg divided every 6 h). Physical examination showed a well-looking adolescent boy with stable vital signs and has multiple purpuric lesions over the upper and lower limbs extending to the buttocks. Majority of the lesions were postinflammatory nonactive with some scattered posthyperpigmentation over the resolved lesions. Abdominal examination was reassuring. Laboratory investigations revealed hemoglobin of 132 g/L, white blood cells (WBCs) of 12.2 × 10⁹/L (neutrophil predominance), and platelets of 366 × 10⁹/L, with normal coagulation profile and factor XIII assay. Erythrocyte sedimentation rate (ESR) of 6 mm/h (normal range 0–15), C-reactive protein (CRP) of 1.5 mg/L (normal range <1.0 mg/L), albumin of 39.6 g/L (normal range 40–50), normal creatinine and blood urea nitrogen, and normal complements. Coombs' test was negative. Urinalysis was negative for protein and blood. Autoimmune tests showed negative antinuclear antibody and antineutrophil cytoplasmic antibody. Skin biopsy disclosed the presence of leukocytoclastic vasculitis with IgA deposition. We proposed to discharge the patient on gradual tapering plan of steroids with close observation.

He was again admitted 2 weeks after discharge with diffuse colicky abdominal pain, history of mixed bloody stool, and worsening diffuse purpuric rash all over the body [Figure 2]. There was no history or physical findings suggestive of other organ involvement. Initial laboratory investigations showed normal cell counts, coagulation, and renal function. Inflammatory markers showed normal ESR and slightly elevated CRP (3.2 mg/L). On the 2^nd day of admission, he experienced hemodynamic instability with recurrent episodes of nonbloody vomiting associated with massive hematochezia and was transferred to critical care unit with hemorrhagic shock. Repeated cell counts showed leukocytosis WBC 25.9 × 10⁹/L (absolute neutrophil counts: 13.97 × 10⁹/L), concentrated hemoglobin up to 191 g/L, elevated CRP 123 mg/L, and normal ESR 2 mm/h. Infectious work-up was unremarkable. A computed tomography angiogram showed abnormal diffuse thickening of the small and large bowel loops with no evidence of bowel ischemia [Figure 3]. The patient received intensive supportive care and required inotropic support and multiple blood transfusions. Pulse methylprednisolone (30 mg/kg/day) was given for total of 5 days combined with one dose of intravenous immunoglobulin (IVIG 2 g/kg), followed by maintenance methylprednisolone (2 mg/kg/day) in divided doses, in addition to five cycles of plasma exchange (PLEX). Fortunately, his clinical condition improved dramatically, he was extubated and off inotropic support, and improved inflammatory markers. Rituximab (RTX) was then initiated as a second-line immunosuppressive agent. Postdischarge follow-up, the patient remained with stable clinical condition on steroid slow tapering plan.

Figure 2: Purpuric rash over the upper and lower limbs

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Figure 3: Computed tomography with contrast (coronal section) of the abdomen showing dilated thickened loops with submucosal edema

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Discussion

IgAV is the most common systemic vasculitis in pediatric age group. Cutaneous manifestations present in almost all cases followed by musculoskeletal disease and abdominal pain, generally as a benign disease with excellent prognosis. Other organ involvement accounts for the major cause of mortality and morbidity in children with IgAV. While nephritis occurs in approximately 40%–60%, mainly in the first 4–6 weeks of presentation usually as a mild disease with high recovery rate, GI involvement in IgAV is frequently seen in more than 50% of patients, usually part of the disease presentation as self-limited disease.^[1],[2] There is a small group of patients who can present either at disease onset or as a complication of disease course with severe GI manifestation including intussusceptions, bowel edema and obstruction, and intestinal bleeding.

Massive GI bleeding in IgAV is considered a rare complication with a reported incidence of <5%.^[2] Where literature is scarce; observations and lessons in how to treat such rare complication is quite variable, still mostly agreed on early use of corticosteroids as lifesaving intervention [Table 1].^{[3],[4],[5],[6],[7],[8],[9],[10],[11],[12],[13],[14],[15]}

Table 1: Summary of studies investigated treatment intervention of patients with immunoglobulin A vasculitis and gastrointestinal involvement

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Systemic corticosteroids remain the cornerstone of therapy in managing severe recurrent manifestations of IgAV. Pulse steroids (10–30 mg/kg/day) followed by maintenance dose (1–2 mg/kg per day) have been widely used in hospitalized IgAV patients. Indications include the presence of nephritis, severe GI disease, and persistent worsening purpura.^[3] With respect to steroid use in IgAV and secondary GI disease, it was found to be associated with rapid resolution of abdominal pain, reduced risk of intussusception as well as reducing the need of surgical intervention.^[4] It also showed promising outcome if used early in the treatment of massive GI hemorrhage as well as refractory intestinal involvement resembling our patient.^[5] Variable other treatment modalities have been administered with controversial outcome [Table 1].^{[3],[4],[5],[6],[7],[8],[9],[10],[11],[12],[13],[14],[15]} Some series show promising outcome with combined therapy. In a retrospective case series of seven patients with final diagnosis of IgAV and massive GI hemorrhage, all patients were successfully managed by combined treatment of pulse steroid, cyclophosphamide, IVIG, and PLEX which was similar to what we did with our patient.^[9] In our patient, the use of combined pulse steroid therapy and IVIG showed excellent life-saving outcome.

RTX is a B-cell monoclonal antibody that binds to CD20. It is expressed on human B-cells and at a low level on a small subset of T-cells.^[16] There is promising evidence on the role of RTX as a rescue therapy for IgAV with refractory disease course and corticosteroid-dependent disease.^[17] In our patient, we proposed the use of RTX as a steroid-sparing agent to maintain disease remission.

The optimal therapeutic interventions are controversial. Despite being a widely recognized condition, IgAV-associated secondary organ involvement remains challenging, specifically with regard to disease course and management.

Conclusion

IgAV (HSP) is generally considered a self-limited disease, however, severe major organ disease and refractory cases have been reported. Patients with GI bleeding have a hazardous unpredicted outcome. The literature is scarce regarding the optimal treatment of IgAV-associated severe GI disease, specifically with respect to GI hemorrhage. The available evidence from case reports and series suggests the role of aggressive combined treatment with corticosteroids, IVIG, and second-line immunosuppressive therapy. We reported here an excellent outcome with combined therapy of steroids, IVIG, and PLEX in the setting of life-threatening emergency. However, further data are needed to optimize evidence on treatment intervention and patient outcome.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Acknowledgment

We acknowledge the patient and their family for their participation.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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