Therapeutic Apheresis Treatment in a Patient with Calciphylaxis Secondary to Lupus Nephropathy on Dialysis – A Case Report

Introduction

Advances in understanding disease pathogenesis have led to increasingly selective blood purification methods for removing pathogenic factors with high specificity. Therapeutic apheresis enables the elimination of acute toxic substances and high–molecular-weight molecules such as lipoproteins, immunoglobulins, and fibrinogen, which standard dialysis cannot clear.¹

“Apheresis” (Latin aphaeresis, Greek “ἀφαίρεσις” = “separation”) refers to extracorporeal therapy that selectively collects blood components (cytapheresis) or removes high-molecular-weight elements (>150 kDa) from plasma (plasmapheresis), including immunoglobulins, immune complexes, and inflammatory mediators. According to the International Standards for Therapeutic Apheresis Units (2014), therapeutic plasmapheresis (TP) should be considered when the disease involves a pathogenic factor whose rapid removal may halt progression, no effective alternative exists, or when disease severity precludes waiting for a pharmacological response.²

Double filtration plasmapheresis (DFPP) is a selective variant in which separated plasma passes through a second filter, retaining pathogenic molecules and returning essential components, minimizing albumin loss and infection risk.³

Calciphylaxis is a rare ESKD complication with high mortality, associated with necrotic skin lesions, mineral metabolism disorders, and inflammation. We describe a case of dialysis-associated calciphylaxis in lupus nephropathy successfully treated with DFPP.

Case Report

A 42-year-old female, diagnosed at 18 with lupus nephritis (ANA and lupus anticoagulant positive) and on thrice-weekly hemodialysis (HD) since diagnosis due to ESKD, underwent deceased-donor kidney transplantation in 2019. The postoperative course was complicated by acute rejection, left upper-arm AVF thrombosis, and surgical site infection. Wound dehiscence with liponecrosis required VAC therapy and necrosectomy. Cultures grew Streptococcus salivarius and Enterococcus faecium, prompting targeted antibiotics.

Thrombophilia screening revealed a heterozygous MTHFR C677T mutation (normal homocysteine). In 2022, despite normal C3/C4 and no active lupus, she developed necrotic skin lesions diagnosed as dialysis-associated calciphylaxis. Labs showed ESR at 96 mm/h and CRP at 7–10 mg/dL.

Due to persistent inflammation and lack of response to conventional measures, DFPP was started. Plasma separation (Plasmaflo OP filter) was followed by fractionation (Cascadeflo EC-50W filter) to remove high–molecular-weight pathogenic substances (IgG, LDL-C) while preserving albumin and low–molecular-weight components.⁴

Eight DFPP sessions were performed: weekly ×1 month, then biweekly ×2 months. Mean treated blood volume was 21 L, plasma volume 3.7 L, with a treated plasma/total plasma volume ratio of 1.28. An average of CRP reduction of 49% and fibrinogen reduction of 69% per session was noted.

Photographic follow-up showed progressive healing [Figure 1]. At cycle end, the patient was clinically stable, with no new calciphylaxis episodes while on maintenance HD.

Close

Figure 1:

Photographic documentation of lesion evolution during the apheresis cycle.

Export to PPT

Discussion

This case supports DFPP as a potential adjunct for dialysis-associated calciphylaxis with persistent inflammation refractory to standard therapy. The approach targets high-molecular-weight molecules implicated in vascular calcification, endothelial dysfunction, and microvascular thrombosis.

In this patient, DFPP produced sustained reductions in inflammatory markers, visible lesion healing, and absence of relapse during follow-up. Post-procedural leukocytosis, also described in rheopheresis and other extracorporeal techniques, may reflect improved tissue perfusion.⁵

Calciphylaxis remains a management challenge, with standard care encompassing wound management, correction of mineral metabolism, and optimization of dialysis. While evidence for DFPP is limited, this case suggests it may benefit selected patients. Larger studies are needed to confirm efficacy, refine protocols, and assess long-term outcomes.