Management of intestinal bleeding with single‐dose cyclophosphamide in Henoch–Schönlein purpura

Uluca, Ünal; Ece, Aydın; Şen, Velat; Yel, Servet; Tan, İlhan; Karabel, Duran

doi:10.1111/ped.12670

Cited by 13 publications

(8 citation statements)

References 10 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Second line treatments for GI disease described within the literature include the use of mycophenolate mofetil (MMF) (39), a single dose of intravenous cyclophosphamide [improved symptoms in 6 children with steroid resistant disease (40)], intravenous immunoglobulin IVIG [demonstrated efficacy in 6 out of 8 French children with GI pain, bleeding or enteropathy (41)], B cell depletion by monoclonal antibodies (7 out of 8 children with chronic steroid dependent disease achieved full remission) (42), methotrexate, colchicine and hydroxychloroquine (43). A cohort of 7 children with refractory GI bleeding that failed to respond to numerous second line drugs reportedly responded to plasma exchange and therefore this could be considered in severe, refractory cases (44).…”

Section: Treatmentmentioning

confidence: 99%

Childhood IgA Vasculitis (Henoch Schonlein Purpura)—Advances and Knowledge Gaps

2019

View full text Add to dashboard Cite

Immunoglobulin A vasculitis (IgAV; formerly Henoch Schonlein Purpura) is the most common form of childhood vasculitis. It can occur in any age and peaks around 4–6 years old. It demonstrates seasonal variation implicating a role for environmental triggers and geographical variation. The diagnosis is made clinically and 95% of patients will present with a rash, together with any from a triad of other systems—gastrointestinal, musculoskeletal, and renal. Most cases of IgAV in children have an excellent outcome. Treatment may be required during the acute phase for gastrointestinal involvement and renal involvement, termed IgAV nephritis (previously HSP nephritis), is the most serious long-term manifestation accounting for ~1–2% of all childhood end stage kidney disease (ESKD). It therefore requires a period of renal monitoring conducted for 6–12 months. Patients presenting with nephrotic and/or nephritic syndrome or whom develop significant persistent proteinuria should undergo a renal biopsy to evaluate the extent of renal inflammation and there are now international consensus guidelines that outline the indications for when to do this. At present there is no evidence to support the use of medications at the outset in all patients to prevent subsequent renal inflammation. Consensus management guidelines suggest using oral corticosteroids for milder disease, oral, or intravenous corticosteroids plus azathioprine or mycophenolate mofetil or intravenous cyclophosphamide for moderate disease and intravenous corticosteroids with cyclophosphamide for severe disease. Angiotensin system inhibitors act as adjunctive treatment for persisting proteinuria and frequently relapsing disease may necessitate the use of immunosuppressant agents. Renal outcomes in this disease have remained static over time and progress may be hindered due to many reasons, including the lack of reliable disease biomarkers and an absence of core outcome measures allowing for accurate comparison between studies. This review article summarizes the current evidence supporting the management of this condition highlighting recent findings and areas of unmet need. In order to improve the long term outcomes in this condition international research collaboration is urgently required.

show abstract

Section: Treatmentmentioning

confidence: 99%

Childhood IgA Vasculitis (Henoch Schonlein Purpura)—Advances and Knowledge Gaps

2019

View full text Add to dashboard Cite

show abstract

“…However, currently, it is established that steroid therapy can actually alleviate the symptoms by decreasing cytokine release and pancreatic secretions [2,9]. In HSP with severe gastrointestinal vasculitis where symptoms persist even after steroid therapy, the use of a single dose of cyclophosphamide has resulted in complete resolution of symptoms [10,11]. Our patient responded to methylprednisolone but had a recurrence of pain on converting to oral prednisolone and needed a single dose of IV cyclophosphamide for complete alleviation of the symptoms.…”

Section: Discussionmentioning

confidence: 90%

Acute Pancreatitis in Henoch-Schonlein Purpura Complicated by Large Pseudocyst Formation Requiring Cystogastrotomy

Saha¹,

Pal²,

Habeeb³

et al. 2021

IJCR

View full text Add to dashboard Cite

On investigation, blood counts, C-reactive protein (CRP), urinalysis, and liver function tests were normal but serum amylase and lipase were mildly elevated (350 IU/L and 410 IU/L respectively). Ultrasound (USG) abdomen showed hepatomegaly without any features of pancreatitis.She was diagnosed as HSP pancreatitis and because of persistent abdominal pain was initiated on oral prednisolone and discharged. After 6 days of discharge, while on oral prednisolone, she was readmitted with severe abdominal pain and recurrent multiple episodes of vomiting. This time, the pain was severe and generalized as the child presented with an acute abdomen.On examination, the child looked toxic, had tachycardia (heart rate 150 beats/minute) with decreased urine output. The blood pressure was in the normal range. Blood counts showed leukocytosis 22,700/cmm with 81% neutrophils, raised CRP (51.2 mg/L, normal <5), elevated serum amylase (2866 IU/L, normal 0-100), and lipase (2970 IU/L, normal 0-60). Contrastenhanced computed tomography (CECT) of the abdomen revealed acute pancreatitis with a peripancreatic collection without necrosis.Considering the increased clinical severity, she received three pulses of iv methylprednisolone 6 mg/kg/day but as pain abdomen recurred on restarting oral prednisolone, one dose of iv cyclophosphamide 500 mg/m 2 was given. Abdominal pain subsided and she was discharged on oral prednisolone.After 5 days, she was readmitted with a recurrence of generalized abdominal pain, vomiting, and abdominal distension.

show abstract

“…A single case report described using mycophenolate mofetil, both as a second‐line treatment for the first episode and for relapses 7,8 . Uluca et al proposed the use of a single dose of intravenous cyclophosphamide for children with severe GI bleeding who did not respond to corticosteroids 9 . A French retrospective case series reported the use of intravenous immunoglobulins as a second‐line therapy.…”

Section: Resultsmentioning

confidence: 99%

“…7,8 Uluca et al proposed the use of a single dose of intravenous cyclophosphamide for children with severe GI bleeding who did not respond to corticosteroids. 9 A French retrospective case series reported the use of intravenous immunoglobulins as a second-line therapy. It reported that six out of eight patients showed a complete response to this treatment and the other two cases relapsed with less severe GI involvement that required a second dose.…”

Section: Treatmentmentioning

confidence: 99%

Gastrointestinal involvement in childhood vasculitides

2020

View full text Add to dashboard Cite

Aim: The aim of this narrative review was to provide a comprehensive summary of the characteristics of gastrointestinal (GI) involvement in the most common paediatric primary vasculitides. Methods: We used PubMed to primarily identify papers, reviews, case series and editorials published in English from 2000 until 31 January 2020. Based on this, we report the prevalence, clinical manifestations, diagnostic approaches and management of GI involvement in each vasculitis. Results: Vasculitides are inflammatory blood vessel diseases, and the majority can affect the GI system with vascular, GI tract or solid organ involvement. GI involvement can sometimes complicate and delay the correct diagnosis. Clinical findings are usually nonspecific symptoms, such as fever, abdominal pain, nausea, vomiting and diarrhoea. Bleeding should alert paediatricians to the possibility of severe complicated vasculitis. Diagnosis relies mostly on imaging. If it goes unrecognised, GI involvement in paediatric vasculitis is a serious cause of morbidity and even mortality, related to bowel ischaemia and perforation. Treatment of GI symptoms depends on the type of vasculitis and usually involves high-dose steroids and immunosuppressants. Conclusion: GI manifestations in the most common paediatric primary vasculitides were usually nonspecific, diagnosis mostly relied on imaging, and treatment usually involved high-dose corticosteroids and immunosuppressants. K E Y W O R D S childhood, gastrointestinal involvement, immunosuppressant, steroids, vasculitis | 2227 TRAPANI eT Al. paralytic ileus, mesenteric ischaemia, bowel obstruction and perforation. Occasionally, GI involvement can be the presenting feature, and this can complicate and delay the correct diagnosis. The aim of this narrative review was to provide a comprehensive summary and update of the characteristics of GI involvement in the most common paediatric primary vasculitis. 2 | ME THODS We used PubMed to primarily identify papers, reviews, case series and editorials published in English from 2000 until 31 January 2020. The keywords included the following: IgA vasculitis, Henoch-Schonlein purpura, Kawasaki disease, Behcet disease, polyarteritis nodosa, Takayasu arteritis, deficiency of adenosine deaminase 2, antineutrophilic cytoplasmic antibodies-associated vasculitis, gastrointestinal, gut, child and paediatric or paediatric.

show abstract

Management of intestinal bleeding with single‐dose cyclophosphamide in Henoch–Schönlein purpura

Cited by 13 publications

References 10 publications

Childhood IgA Vasculitis (Henoch Schonlein Purpura)—Advances and Knowledge Gaps

Childhood IgA Vasculitis (Henoch Schonlein Purpura)—Advances and Knowledge Gaps

Acute Pancreatitis in Henoch-Schonlein Purpura Complicated by Large Pseudocyst Formation Requiring Cystogastrotomy

Gastrointestinal involvement in childhood vasculitides

Contact Info

Product

Resources

About