A good response to glucocorticoid for Henoch–Schönlein purpura with abdominal pain and gastrointestinal bleeding in an adult

Abstract Rationale: Henoch–Schönlein purpura (HSP) is a small-vessel vasculitis that has been extensively studied in children, but little is known about its natural history in adults. There is no consensus regarding the treatment of glucocorticosteroids use for HSP. The efficacy of glucocorticoid for preventing from severe complications or relapse is also controversial in HSP. Patient concerns: A 21-year-old male was admitted to the hospital due to abdominal pain for more than 20 days, hematochezia for more than 10 days, and rash for 2 days. Diagnoses: The diagnosis of HSP is based on the European League against Rheumatism and the Paediatric Rheumatology European Society in 2006. Interventions: The patient received glucocorticosteroids treatment for 17 days at the time of first hospitalization. Outcomes: The abdominal pain and hematochezia completely disappeared on the 6th day after the use of glucocorticosteroids, and purpura completely disappeared on the 8th day. Lessons: Our patient has a good response to glucocorticoid. Glucocorticosteroids may be effective for the treatment of HSP.


Introduction
Henoch-Schönlein purpura (HSP) is the most common systemic vasculitis in children. [1] Annual incidence of HSP in children is estimated to be 15/100,000 cases. [2] The proportion of males and females in children is close to 2:1. [3] By comparison, it is more rare in adults with an estimated annual incidence of 1.3/100,000 cases. [2] Compared with HSP in children, HSP in adults have more serious clinical manifestations and worse outcomes. [4] Clinical characteristics of HSP include abdominal pain, gastrointestinal bleeding, nonthrombocytopenic palpable purpura, arthritis, and renal involvement. [5] Most of them occur in autumn and winter, but its pathogenesis and causes are still unclear. [6] In 2006, the diagnostic criteria published by the European League against Rheumatism and the Paediatric Rheumatology European Society which include palpable purpura in combination with at least one of other manifestations (ie, abdominal pain, immunoglobulin A deposition, arthritis or arthralgia, and renal involvement). [7] There is no consensus on treatment of glucocorticoid for HSP in adults. The efficacy of glucocorticoid in preventing from severe complications or relapse is also controversial in HSP. [8,9] In this case report, we showed that an adult patient with HSP presenting with abdominal pain and gastrointestinal bleeding had a good response by glucocorticoid.

Case presentation
On February 12, 2019, a 20-year-old male was admitted to our department due to abdominal pain for more than 20 days, hematochezia for more than 10 days, and rash for 2 days.
On February 15, 2019, his abdominal pain was improved obviously and heart rate was dropped to normal with an average of 80 beats per minute. He intermittently had hematochezia with the total amount of about 400 mL. Laboratory tests showed WBC, 32.2 Â 10 9 /L; GR%, 90.1%; RBC, 3.75 Â 10 12  On February 21, 2019, his condition was relatively stable. Gastroscopy showed chronic superficial gastritis with bile reflux (Fig. 2). The patient's rash disappeared after glucocorticoid treatment. The dosage of methylprednisolone was gradually decreased.

Effectiveness of glucocorticoid
Currently, the evidence about the application of glucocorticoid for HSP is insufficient. Some researchers reported that early use of prednisone in HSP did not reduce either the risk of renal involvement within one year or the risk of acute gastrointestinal complications. [8] In contrast, Ronkainen et al found that the mean duration of total days abdominal pain was shortened by 1.2 days in the prednisone group. [10] Similarly, Dr Saulsbury also reported that glucocorticoids could accelerate the relief of abdominal pain. [11] Some authors suggested that the dose of glucocorticoid was 1 to 2 mg/kg/d and gradually reduced in the treatment of HSP. [10,11] Our patient's weight is 60 kg, and intravenous glucocorticoid was given at a dose of 80 mg twice a day for the first 3 days, followed by a dose of 40 mg twice a day for the second 3 days. Then, the glucocorticoid was taken orally and gradually reduced until he was discharged.
In our patient, the abdominal pain relieved since the first use of glucocorticoids. Then, the rash completely disappeared on the 8th day after the first use of glucocorticoids. The laboratory parameters including WBC, GR%, and ESR gradually decreased to normal (Fig. 3). Abdominal pain is a common clinical Medicine manifestation in patients with HSP. Up to 75% of HSP patients have gastrointestinal tract involvement, which is often characterized by diffuse colic pain. [3] In addition, HSP is considered a self-limiting disease with resolution of symptoms within 4 to 6 weeks of onset, but can lead to some poor outcomes, such as gastrointestinal bleeding, intussusception, and end-stage renal disease. [9] Occult or symptomatic gastrointestinal bleeding, such as hematemesis, melena, and rectal bleeding, is observed in 50% of children with HSP. [12] By comparison, gastrointestinal bleeding associated with HSP is rare in adults, accounting for only 4%. [13] The outcomes of gastrointestinal bleeding are poor, and some patients require blood transfusion or surgery, and even die. [14] A case report suggested that methylprednisolone pulse treatment might be effective for gastrointestinal bleeding in HSP. [15] In our case, the use of glucocorticoid therapy in the patient with gastrointestinal bleeding is effective.

Relapse and its risk factors
Our case developed abdominal pain again accompanied by hematuria and proteinuria without hematochezia or rash about 1 week after discharge. Abdominal pain is the most common presentation for disease recurrence (about 63%). [12] Hematuria and proteinuria indicating renal involvement are more common in adults than in children [2] ; and 67% of patients develop hematuria at the 5th week of onset in adults. [16] The precipitating factor for relapse in our case might be a recent history of infection, which presented with cough, sputum, and runny nose with an increased ESR level on laboratory tests (Fig. 3). The upper respiratory tract infection is a potential trigger in HSP. [11] Researchers also suggested that the relapse was strongly associated with a high ESR value. [17] In conclusion, our patient with HSP had a good response to glucocorticoid therapy. However, current evidence in regard to the efficacy of glucocorticoid or relapse is lacking. Further researches are necessary to explore the relationship between the use of glucocorticoid and prognosis of HSP.