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. 2023 Jun 1;183(6):615-618.
doi: 10.1001/jamainternmed.2023.0152.

Daratumumab for a Patient With Refractory Antineutrophil Cytoplasmatic Antibody-Associated Vasculitis

Torben M Rixecker  1 , Philipp M Lepper  1 , Sebastian Mang  1 , Pascal Espig  1 , Katharina Brill  2 , Lorenz Thurner  2 , Joerg Thomas Bittenbring  2
Affiliations

Daratumumab for a Patient With Refractory Antineutrophil Cytoplasmatic Antibody-Associated Vasculitis

Torben M Rixecker et al. JAMA Intern Med. .
No abstract available

Plain language summary

This case reports on a patient with antineutrophil cytoplasmatic antibody–associated vasculitis and severe pulmonary and cutaneous involvement who received conventional therapy that failed and who was rescued by daratumumab therapy.

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Conflict of interest statement

Conflict of Interest Disclosures: Dr Thurner reported receiving grants from the Wilhelm Sander Foundation, BioNanoMed, AbbVie, Janssen, and EUSSA-Pharm and participating in advisory boards for Takeda, AstraZeneca, Merck, and EUSA Pharma outside the submitted work; in addition, Dr Thurner, together with others, holds a patent for progranulin antibodies as a marker for autoimmune diseases filed by the Saarland University. Dr Bittenbring reported receiving personal fees from AstraZeneca and MSD outside the submitted work. No other disclosures were reported.

Figures

Figure 1.
Figure 1.. Evolution of Pulmonary and Cutaneous Disease Manifestations
A, Chest radiograph 3 days before daratumumab was started. B, Chest radiograph 44 days after daratumumab was started. C, Photograph of the manifestation of cutaneous disease on the right foot 4 days before daratumumab was started. D, Photograph of the manifestation of cutaneous disease on the right foot 48 days after daratumumab was started.
Figure 2.
Figure 2.. Time Course of Treatment, Respiratory Support, and Antineutrophil Cytoplasmatic Antibody (ANCA) Titer
A, Treatment course from the time of hospital admission to hospital discharge on day 120 (each vertical arrow represents 375 mg/m2 of rituximab, 750 mg/m2 of intravenous cyclophosphamide, 16 mg/kg of daratumumab, 1000 mg of intravenous methylprednisolone, exchange of and 60 mg/kg body weight albumin replacement of therapeutic plasma exchange, respectively). B, Respiratory support from the time of hospital admission to hospital discharge. C, Course of the anti–proteinase 3 (anti-PR3) immunoglobuline G (IgG) titer from the time of hospital admission to hospital discharge (the dashed line indicates normal range). ECMO indicates extracorporeal membrane oxygenation; q2d, every second day.
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