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Rituximab addition to cyclophosphamide and glucocorticoids may treat refractory ANCA-positive retroperitoneal fibrosisAdding rituximab helps treat a rare and difficult condition

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Key Takeaway
Note that rituximab addition may improve outcomes in refractory ANCA-positive retroperitoneal fibrosis.

This case report describes the management of a single patient with MPO-ANCA-positive retroperitoneal fibrosis. The patient initially received a combination of glucocorticoids and cyclophosphamide, which resulted in limited efficacy. Following the addition of rituximab, the patient experienced significant regression of retroperitoneal fibrosis lesions.

While the addition of rituximab appeared to improve clinical outcomes and potentially reduce reliance on glucocorticoids, the report notes a serious adverse event: a vertebral compression fracture, which was likely glucocorticoid-related. The evidence is limited by the nature of the report as a single case study, which does not allow for the determination of broader clinical patterns or definitive treatment protocols.

Clinical utility is currently limited. While the combination of cyclophosphamide and rituximab may be a potential therapeutic option for refractory ANCA-positive retroperitoneal fibrosis, the results from this single case cannot be generalized to the broader patient population.

How this fits prior evidence

This case report addresses a gap in the management of ANCA-positive retroperitoneal fibrosis. It does not relate to the previously covered findings regarding rituximab monotherapy in follicular lymphoma, metabolic syndrome in lupus, HPV infection in SLE patients, anti-HBc positivity in DLBCL, or TAFRO syndrome treatment.

Imagine dealing with a rare condition that affects the area behind your abdominal organs. For some patients with a specific type of this condition, standard treatments like steroids and cyclophosphamide do not work well enough. This makes it very difficult to manage the disease effectively.

A single patient with this rare condition was treated with a combination of steroids and cyclophosphamide. This initial treatment showed limited success. However, when doctors added a medication called rituximab, the patient saw a significant shrinking of the disease lesions. This change also helped the patient rely less on steroids.

While these results are promising, it is important to remember that this was a report on just one person. Because of this small sample size, the results are not yet certain for everyone. Additionally, the patient did experience a serious side effect, a vertebral compression fracture, which was likely caused by the steroids. Talk to your doctor about how these treatments might apply to your specific health needs.

What this means for you:
Adding rituximab to standard treatment may shrink lesions and reduce steroid use in some rare cases.

Common questions

What is retroperitoneal fibrosis?

Retroperitoneal fibrosis is a rare condition where fibrous tissue builds up in the area behind the abdominal organs. This specific case involved a patient with a version of the condition linked to MPO-ANCA, which is a type of marker found in some autoimmune conditions.

How did the treatment change for the patient?

The patient first received a combination of glucocorticoids and cyclophosphamide, but this had limited success. When the doctors added rituximab to the treatment plan, the patient experienced a significant regression of the disease lesions and a reduced need for glucocorticoids.

Are there any risks with this treatment?

The patient in this report experienced a serious side effect called a vertebral compression fracture. Doctors believe this specific injury was likely caused by the use of glucocorticoids. You should discuss all potential risks and side effects with your doctor.

Study Details

Study typeGuideline
EvidenceLevel 5
PublishedNov 2026
View Original Abstract ↓
BackgroundMyeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA)-positive retroperitoneal fibrosis (RPF) is an extremely rare condition. Currently, there is no consensus on diagnostic criteria or optimal treatment strategies for autoimmune disease-associated RPF. Herein, we report a case of refractory MPO-ANCA-positive RPF.Case presentationA patient presented to the Department of Rheumatology and Immunology with abdominal pain and was diagnosed with ANCA-positive RPF following comprehensive evaluation. Initial treatment with glucocorticoids combined with cyclophosphamide (CTX) demonstrated limited efficacy, and the patient developed a likely glucocorticoid-related adverse event, namely, vertebral compression fracture. Rituximab (RTX) was subsequently added to the regimen. This treatment adjustment resulted in a significant regression of RPF lesions.ConclusionsFor refractory ANCA-positive RPF, combination therapy with CTX and RTX represents a potential therapeutic option and may reduce reliance on glucocorticoids.
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