Tom Macleod, Charles Bridgewood, Dennis McGonagle, The Lancet Rheumatology Volume 5, Issue 1, January 2023, Pages e47-e57
Tag: Behçet's disease
Scientific corner
Role of neutrophil interleukin-23 in spondyloarthropathy spectrum disorders
https://www.sciencedirect.com/science/article/abs/pii/S2665991322003344
https://www.thelancet.com/journals/lanrhe/article/PIIS2665-9913(22)00334-4/abstract
Neutrophilic inflammation is a pervasive characteristic common to spondyloarthropathies and related disorders. This inflammation manifests as Munro’s microabscesses of the skin and osteoarticular neutrophilic inflammation in patients with psoriatic arthritis, intestinal crypt abscesses in patients with inflammatory bowel disease, ocular hypopyon in anterior uveitis, and neutrophilic macroscopic and microscopic inflammation in patients with Behçet’s disease. Strong MHC class I associations are seen in these diseases, which represent so-called MHC-I-opathies, and these associations indicate an involvement of CD8 T-cell immunopathology that is not yet well understood. In this Personal View, we highlight emerging data suggesting that the T-cell-neutrophil axis involves both a T-cell-mediated and interleukin (IL)-17-mediated (type 17) recruitment and activation of neutrophils, and also a sequestration of activated neutrophils at disease sites that might directly amplify type 17 T-cell responses. This amplification likely involves neutrophilic production of IL-23 and proteases as well as other feedback mechanisms that could be regulated by local microbiota, pathogens, or tissue damage. This crosstalk between innate and adaptive immunity offers a novel explanation for how bacterial and fungal microbes at barrier sites could innately control type 17 T-cell development, with the aim of restoring tissue homoeostasis, and could potentially explain features of clinical disease and treatment response, such as the fast-onset action of the IL-23 pathway blockade in certain patients. This axis could be crucial to understanding non-response to IL-23 inhibitors among patients with ankylosing spondylitis, as the axial skeleton is a site rich in neutrophils and a site of haematopoiesis with myelopoiesis in adults.
Scientific corner
Use of granulocyte and monocyte adsorption apheresis in dermatology (Review)
Exp Ther Med 2022 Jun 24;24(2):536. doi: 10.3892/etm.2022.11463. eCollection 2022 Aug. DOI: 10.3892/etm.2022.11463
Adsorptive granulocyte and monocyte apheresis (GMA) is an extracorporeal treatment that selectively removes activated myeloid lineage leukocytes from peripheral blood. This technique consists of a column with cellulose acetate beads as absorptive leukocytapheresis carriers, and was initially used to treat ulcerative colitis. A literature search was conducted to extract recently published studies about the clinical efficacy of GMA in patients with different skin disorders, reporting information on demographics, clinical symptoms, treatment and clinical course. Dermatological diseases, in which GMA has been performed, include generalized pustular psoriasis, pyoderma gangrenosum, palmoplantar pustular psoriasis, Behcet’s disease, Sweet’s syndrome, adult-onset Still’s disease, impetigo herpetiformis, reactive arthritis, acne and hidradenitis suppurativa syndrome, cutaneous allergic vasculitis and systemic lupus erythematosus. In most patients, GMA was started after the failure of conventional therapeutic options and it was helpful in the majority of cases. Based on the information summarized, GMA could be considered a valid non-pharmacological treatment option for patients with several dermatological conditions, which are difficult to treat with other pharmacological preparations.
PASH syndrome; cutaneous allergic vasculitis; granulocyte and monocyte apheresis; neutrophilic dermatoses; reactive arthritis; systemic lupus erythematosus.
Scientific corner
Recommendations for Therapeutic Apheresis by the Section “Preparative and Therapeutic Hemapheresis” of the German Society for Transfusion Medicine and Immunohematology
Nina Worel 1, Behrouz Mansouri Taleghani 2, Erwin Strasser 3 Transfus Med Hemother 2019 Dec;46(6):394-406. doi: 10.1159/000503937. Epub 2019 Nov 6.
The section “Preparative and Therapeutic Hemapheresis” of the German Society for Transfusion Medicine and Immunohematology (DGTI) has reviewed the actual literature and updated techniques and indications for evidence-based use of therapeutic apheresis in human disease. The recommendations are mostly in line with the “Guidelines on the Use of Therapeutic Apheresis in Clinical Practice” published by the Writing Committee of the American Society for Apheresis (ASFA) and have been conducted by experts from the DACH (Germany, Austria, Switzerland) region.
Scientific corner
P154 Clinical assessment of prognosis and the prognostic factors in intestinal Behcet’s disease
M Hachiya, T Sakurai, Y Nagata, A Hidaka, Y Akita, H Miyashita, Y Maruyama, R Miyazaki, M Noguchi, R Sawada, J Mitobe, M Mitsunaga, T Yamasaki, T Kato, M Saruta, Journal of Crohn’s and Colitis, Volume 12, Issue supplement_1, February 2018,
Approximately 30% of all cases and 50% of severe cases that had received biologics were not able to avoid surgery. In severe cases, it is important to evaluate early treatment efficacy and prognostic factors because the median time of administration of biologics was only 9 weeks. However, no independent prognostic factor was found in this study.
Scientific corner
Updated treatment strategies for intestinal Behçet’s disease
Yong Eun Park 1, Jae Hee Cheon 1 2 , Korean J Intern Med. 2018 Jan;33(1):1-19.
Recently, there has been a line of evidence suggesting that biologics such as infliximab and adalimumab are effective in treating intestinal BD. Moreover, new biologics targeting proteins other than tumor necrosis factor α are emerging and are under active investigation. Therefore, in this paper, we review the current therapeutic strategies and new clinical data for the treatment of intestinal BD.
Scientific corner
Granulocyte and monocyte adsorption apheresis for Behçet’s disease in a pregnant woman.
Yuko Higashi 1, Mitsuyoshi Shimokawa, Kazuhiro Kawai, Takuro Kanekura, J Dermatol. 2013 Dec;40(12):1042-4.
We present a 39-year-old pregnant woman with Behçet’s disease who was treated successfully with granulocyte and monocyte adsorption apheresis (GMA). There were no complications or adverse effects during her pregnancy and delivery. The neonate manifested no abnormalities.
Scientific corner
Adsorptive monocyte and granulocyte apheresis in the chronic inflammatory illness: ulcerous colitis, Crohn’s disease, rheumatoid arthritis and Behcet syndrome
J. Muñoz, M. Clavo, O. Garcia, D. Reina, A. Vidaller, R. Lafuente & L. I. Massuet
ISBT Science Series (2007) 2, 96–101
There is a strong basis to support the modulators properties of innate immunity of GCAP, although there is a lack of data that explains deeply the interactions between the mechanisms involved. GMA may represent a new therapy that offers not a single pathway effect but a global modulation of the most important pathways involved in innate immune response. Future investigations should elucidate the intimate mechanism of action.
Scientific corner
Granulocytapheresis in patients with refractory ocular Behcet’s disease
Kenichi Namba 1, Koh-Hei Sonoda, Hirokuni Kitamei, Kenji Shiratori, Akiko Ariyama, Kazuya Iwabuchi, Kazunori Onoé, Abby R Saniabadi, Shoichi Inaba, Tatsuro Ishibashi, Shigeaki Ohno, J Clin Apher. 2006 Jul;21(2):121-8
We conclude that granulocytapheresis might be effective and safe for patients with refractory ocular BD. Further studies are necessary to fully evaluate the clinical efficacy of granulocytapheresis for BD.
Scientific corner
Therapeutic neutrophil apheresis in patients with ocular Behcet disease
Koh-Hei Sonoda 1, Shoich Inaba, Akiko Ariyama, Yoh-Ich Kawano, Abby Saniabadi, Tatsuro Ishibashi, Arch Ophthalmol. 2005 Feb;123(2):267-9.
Scientific corner
Treatment of Behçet’s disease with granulocyte and monocyte adsorption apheresis
Takuro Kanekura 1, Akira Gushi, Masahiro Iwata, Seita Fukumaru, Ryoko Sakamoto, Koichi Kawahara, Ikuro Maruyama, Tamotsu Kanzaki
The painful orogenital ulcerations of Behçet’s disease are among the major symptoms of patients and are often intractable. We assessed the efficacy of granulocyte and monocyte adsorption apheresis therapy in two patients, a 21-year-old man with orogenital ulcerations and a 50-year-old woman with genital ulceration and abdominal pain. They underwent 5 and 8 granulocyte and monocyte adsorption apheresis treatments at 5-day intervals, respectively. The painful orogenital ulcerations of the man responded dramatically and the genital ulcer of the woman decreased in size and her abdominal pain was improved. Our results demonstrate that granulocyte and monocyte adsorption apheresis may be useful for treating orogenital ulcerations of Behçet’s disease.
Contact UsFor more information
Contact Us