Scientific corner

A case of severe generalized pustular psoriasis successfully treated with IL-17A monoclonal antibody and granulocyte removal therapy

Keiki ShimadaDaisuke KatagiriAika KatoNaoto NunoseMotohiko SatoYuri KatayamaKanako TerakawaTakahito NiikuraEmi SakamotoYuki YoshizakiMinami SuzukiTakashi FukayaTakeshi Tamaki & Hideki Takano Ren Replace Ther 8, 50 (2022).

Background Generalized pustular psoriasis (GPP) usually presents with fever, generalized flushing, and multiple sterile pustules on the skin, which histopathologically form subcorneal pustules characterized by Kogoj spongiform pustules. Granulocyte/monocyte adsorption apheresis (GMA) was approved in Japan in 2012. The use of biologics for psoriasis treatment is increasing. Several case reports have evaluated the combination of GMA and cyclosporine (CyA) for GPP. However, very few English reports on combining biologics and GMA in treating GPP exist. Case presentation A 79-year-old man with a history of hypertension, diabetes mellitus, chronic kidney disease, and atrial fibrillation was admitted. He had been consulting a dermatologist for psoriasis vulgaris (PV) since the age of 44. The patient was diagnosed with severe GPP and treated with 300 mg secukinumab (SEC) on day 3. SEC is a fully human monoclonal IgG1 antibody that targets IL-17A. Five doses were administered. In addition, GMA was administered once a week, three times from day 4. After the first administration of GMA, the inflammatory response and skin condition improved markedly. The patient was discharged from the hospital on day 34. Conclusions The present study is the first English-written report on the combined administration of SEC and GMA both instituted since admission for severe GPP, with immediate patient response to treatment. Notably, IL-17A plays a vital role in the pathogenesis of GPP. GMA can eliminate activated leukocytes, and the early introduction of combined IL-17 monoclonal antibody and GMA may allow disease suppression in patients with severe GPP, thus avoiding progression to multiorgan failure. Further studies may verify the effects of IL-17 monoclonal antibodies and GMA on severe GPP.

Scientific corner

Granulocyte and monocyte apheresis therapy for patients with active ulcerative colitis associated with COVID-19: a case report

Miki Koroku 1Teppei Omori 1Harutaka Kambayashi 1Shun Murasugi 1Tomoko Kuriyama 1Yuichi Ikarashi 1Maria Yonezawa 1Ken Arimura 2Kazunori Karasawa 3Norio Hanafusa 4Masatoshi Kawana 5Katsutoshi Tokushige 

Intest Res 2022 Jan;20(1):150-155. doi: 10.5217/ir.2020.00148. Epub 2021 Mar 12

Coronavirus disease 2019 (COVID-19), caused by infection with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), is now a pandemic. Although several treatment guidelines have been proposed for patients who have both inflammatory bowel disease and COVID-19, immunosuppressive therapy is essentially not recommended, and the treatment options are limited. Even in the COVID-19 pandemic, adjuvant adsorptive granulocyte and monocyte apheresis may safely bring ulcerative colitis (UC) into remission by removing activated myeloid cells without the use of immunosuppressive therapy. Our patient was a 25-year-old Japanese male with UC and COVID-19. This is the first case report of the induction of UC remission with granulocyte and monocyte apheresis treatment for active UC associated with COVID-19.

Scientific corner

Rapidly Progressing Aseptic Abscesses in a Patient with Ulcerative Colitis

Yoshiharu Yamaguchi 1Marie Nakagawa 1Shoko Nakagawa 1Kazuhiro Nagao 1Satoshi Inoue 1Tomoya Sugiyama 1Shinya Izawa 1Yasutaka Hijikata 1Masahide Ebi 1Yasushi Funaki 1Naotaka Ogasawara 1Makoto Sasaki 1Kunio Kasugai 1 Intern Med  2021 Mar 1;60(5):725-730. doi: 10.2169/internalmedicine.5733-20. Epub 2020 Sep 30.

Aseptic abscesses (AAs) are extraintestinal manifestations of inflammatory bowel disease (IBD). IBD-associated AAs are rare in Japan. We treated a 45-year-old man with ulcerative colitis (UC)-associated AAs. During remission, multiple progressive abscesses were detected in the spleen; he underwent splenectomy because an infectious disease was suspected. Although his condition improved temporarily after splenectomy, a large liver abscess was noted, and a diagnosis of UC-associated AAs was made. Granulocytapheresis (GCAP) and infliximab (IFX) administration resolved the abscess. This is the first reported case of UC-associated AAs in a Japanese patient treated by splenectomy, GCAP, and IFX.

Scientific corner

Granulocyte and monocyte adsorption apheresis as an effective treatment for Reiter disease

A Yoshifuku 1K OyamaA IbusukiM KawasakiM SakanoueS MatsushitaK KawaiK KawaharaI MaruyamaT Kanekura

Clin Exp Dermatol 2012 Apr;37(3):241-4. doi: 10.1111/j.1365-2230.2011.04181.x. Epub 2011 Oct 18.

Reiter disease (RD) is characterized by a triad of sterile arthritis, urethritis and conjunctivitis. The conditions occur concomitantly or sequentially, and are associated with mucocutaneous features such as circinate balanitis and stomatitis. Arthritis usually occurs in attacks followed by recovery, but it sometimes progresses to permanent damage of the affected joints. Because the symptoms of this disorder are attributable to activated neutrophils, we assessed the efficacy of granulocyte and monocyte adsorption apheresis (GCAP) in a 73-year-old man with RD who had skin rashes on his penis, scrotum and right hand, with severe arthralgia. The patient’s skin rash and joint pain responded dramatically to five sessions of GCAP delivered at intervals of 5 days. We present a detailed description of the patient and discuss the mechanisms of GCAP, and suggest that GCAP may be useful for treating RD.

Scientific corner

Treatment of Pyoderma Gangrenosum With Granulocyte and Monocyte Adsorption Apheresis

Takuro Kanekura,Koichi Kawahara,Ikuro Maruyama,Tamotsu Kanzaki Ther Apher Dial 2005 Aug;9(4):292-6. doi: 10.1111/j.1744-9987.2005.00284.x.

Pyoderma gangrenosum is an intractable skin disorder characterized by the development of erythematous pustules or nodules that rapidly progress to destructive, necrotizing, non-infective ulcers. We assessed the efficacy of granulocyte and monocyte adsorption apheresis (GCAP) therapy in two new patients, a 67-year-old man with ulcerations on his lower leg, and a 44-year-old man with turgid erythematous lesions with burrowing abscesses and sinus formation on his hip, groin, and thighs. Both patients received 10 GCAP treatments at 5-day intervals. Their skin lesions responded well. The 9 cmx6 cm ulcer on the lower right leg of the 67-year-old patient was completely covered by regenerated skin at the completion of therapy. The turgid skin lesions containing pustules and ulcers of the other patient showed amelioration and a marked decrease in the volume of exudate. Our results suggest that GCAP is a useful treatment modality for pyoderma gangrenosum.

Scientific corner

Improvement of adult Still’s disease with granulocyte and monocyte adsorption apheresis

T Kanekura 1K TerasakiY HigashiN YoshiiK KawaharaI MaruyamaT Kanzaki

Clin Exp Dermatol 2004 Jul;29(4):410-2. doi: 10.1111/j.1365-2230.2004.01502.x.

Adult Still’s disease is characterized by a high spiking fever, transient skin rash, and polyarthralgia. Joint pain is one of the major complaints and is often intractable. We assessed the efficacy of granulocyte and monocyte adsorption apheresis (GCAP) therapy for treating arthralgia in adult Still’s disease. A 33-year-old woman with adult Still’s disease who suffered from recalcitrant arthralgia resistant to systemic corticosteroids was treated with GCAP therapy. She underwent five GCAP treatments at 5-day intervals. Her joint pain responded dramatically to the GCAP therapy, suggesting that GCAP may be useful for treating adult Still’s disease. We present a detailed description of the patient and this novel therapy.

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