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Abstract Number: 1175

Rituximab for Idiopathic and IgG4-Related Retroperitoneal Fibrosis

Rachel Wallwork1, Zachary S. Wallace2, Cory A. Perugino3, Amita Sharma4 and John H. Stone5, 1Department of Medicine, Massachusetts General Hospital, Boston, MA, 2Division of Rheumatology, Allergy and Immunology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, 3Ragon Institute of MGH, MIT and Harvard, Cambridge, MA, 4Department of Radiology, Massachusetts General Hospital, Boston, MA, 5Massachusetts General Hospital Rheumatology Unit, Harvard Medical School, Boston, MA

Meeting: 2017 ACR/ARHP Annual Meeting

Date of first publication: September 18, 2017

Keywords: IgG4 Related Disease, Retroperitoneal Fibrosing and rituximab

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Session Information

Date: Monday, November 6, 2017

Title: Miscellaneous Rheumatic and Inflammatory Diseases Poster I

Session Type: ACR Poster Session B

Session Time: 9:00AM-11:00AM

Rituximab for Idiopathic and IgG4-Related Retroperitoneal Fibrosis

Background/Purpose: Untreated retroperitoneal fibrosis (RPF) can lead to chronic back and flank pain and/or renal failure. The mainstay of treatment for non-malignant (IgG4-related disease [RD] or idiopathic) RPF is glucocorticoid (GC) therapy which often has insufficient efficacy or is complicated by toxicity.  We performed a retrospective review of patients with RPF treated with rituximab (RTX).

Methods: We reviewed the database of the Massachusetts General Hospital’s Center for IgG4-RD and identified all patients with RPF treated with RTX who had follow-up (2010-2016). RPF was diagnosed by CT or MRI following commonly accepted radiographic criteria (Lancet 2011;378:338). Demographics, date of diagnosis, symptoms, co-morbidities, laboratory values, use of stents or percutaneous nephrostomy (PCN) tubes, biopsy results, and symptomatic response to treatment were collected from the database. A board-certified radiologist (AS) reviewed baseline and follow-up imaging. Response to treatment was defined as symptom improvement and/or radiographic improvement defined as reduction in lesion size in at least two radiographic planes (Lancet 2011;378:338).

Results: Twenty-six RPF patients received RTX (Table 1). The median age was 55 years (IQR 43, 58) and a majority was male (85%) and former or current smokers (85%). Pathology was available in 20 (77%) patients. In 17/20 (85%) patients, diagnostic features of IgG4-RD were present; in the remaining 3/20 (15%), malignancy and infection were ruled out.  Of the six patients not biopsied, 2 (33%) met clinical diagnostic criteria for IgG4-RD and four had typical radiographic idiopathic RPF with no clinical suspicion of infection or malignancy.  Ten of 26 (39%) patients had previously failed GC therapy. RTX was used as monotherapy in 20 (77%) patients and in combination with GC in 6 patients (23%); ten (39%) patients required ureteral stents or PCN tubes. Of the 21 patients with baseline symptoms (e.g., pain), all (100%) had improvement.  Twenty-two (88%) of the 25 patients with follow-up imaging had radiographic improvement; in the remaining 3 patients, the RPF remained stable. Of the 10 patients with a stent or PCN, 4 (40%) had it successfully removed.  Four of the six patients whose stents or PCN were not removed had disease duration for greater than 1 year prior to RTX.  

Conclusion: In this retrospective study of patients with idiopathic or IgG4-related RPF, peripheral B cell depletion with RTX was highly effective, used as monotherapy in the majority of patients. A striking proportion (85%) of patients were current or former smokers. Future research could explore whether patients with disease for greater than 1 year prior to RTX respond less well.

 

Table 1: Baseline Demographics and Features with Treatment Response

Sample Size

26

Age at Diagnosis (Median, IQR)

55 (43, 58)

Male (%)

22 (85%)

Non-Hispanic White

19 (73%)

Smoking History

22 (85%)

 Former

    16 (62%)

       Current

    6 (23%)

Serum IgG4 Concentration Elevated

11 (42%)

Other Organ Involvement

6 (23%)

Diagnostics

      Typical Radiographic Appearance

26 (100%)

          RPF Biopsied

  20 (77%)

            Diagnostic of IgG4-RD

      17 (65%)

            Suggestive of IgG4-RD, ruled out malignancy/infection

      3 (12%)

          Met Clinical Criteria for IgG4-RD (of those not biopsied)

   2 (8%)

Presentation

Pain (back or flank)

19 (73%)

Renal Failure

16 (62%)

Treatment Approach

Prior Treatment with Steroids

10 (39%)

Rituximab with Steroids

6 (23%)

Rituximab Only

20 (76%)

Ureteral Stents or Percutaneous Nephrostomy (PCN) Tube

10 (39%)

Treatment Response

Symptomatic Response (N=26)

21 (100%)*

Radiographic Response** (N=25)

22 (88%)†

      Stable (if no reduction in size, N=3)

3 (100%)

Stent or PCN Removed (N=10)

4 (40%)††

*of those with baseline symptoms; **Response defined as improvement in size in at least 2 planes (e.g., anterior-posterior, cranio-caudal); †of 25 patients with f/u radiology; ††of 10 patients with stents or PCN

 


Disclosure: R. Wallwork, None; Z. S. Wallace, None; C. A. Perugino, None; A. Sharma, None; J. H. Stone, Xencor, 2.

To cite this abstract in AMA style:

Wallwork R, Wallace ZS, Perugino CA, Sharma A, Stone JH. Rituximab for Idiopathic and IgG4-Related Retroperitoneal Fibrosis [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/rituximab-for-idiopathic-and-igg4-related-retroperitoneal-fibrosis/. Accessed .
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