Toshiyuki Yamamoto, Kenshi Yamasaki, Keiichi Yamanaka, Mayumi Komine, Tamihiro Kawakami, Osamu Yamamoto, Takuro Kanekura, Tetsuya Higuchi, Toshiya Takahashi, Yoshiaki Matsushima, Nobuyuki Kikuchi, Japanese Dermatological Association Pyoderma Gangrenosum Treatment Guidelines Drafting Committee Journal of Dermatology 50 (9) 2023, e253-e275 doi.org=10.1111/1346-8138.16845

Pyoderma gangrenosum (PG) is a rare, neutrophilic skin disease. For the purpose of accurate diagnosis and proper treatment of PG, the Japanese clinical practice guidance for PG developed by the Japanese Dermatological Association was published in 2022. In this guidance, clinical aspects, pathogenesis, current therapies, and clinical questions on PG are described from the viewpoints of current knowledge and evidence-based medicine. Here, the English version of the Japanese clinical practice guidelines for PG is presented and is intended to be widely referred to in the clinical examination and treatment of PG.

Allergic and Immunologic Diseases

A Practical Guide to the Evaluation, Diagnosis and Management of Allergic and Immunologic Diseases 2022, Pages 1527-1583

Apheresis is a term for a group of extracorporeal treatments in which blood is separated into its components, with some components being discarded and replaced or subsequently modified. The replacement fluids/cells or modified components, along with the remainder of the blood, are then returned to the patient. These procedures can alter the immune system, both humoral and cellular, and have been used to treat a variety of common and uncommon immunologic diseases beginning in the late 1950s. The basic background information important for understanding those apheresis procedures used to treat immunologic disorders as well as the important patient considerations are discussed. A synopsis of immunologic diseases treated with apheresis, based upon the American Society for Apheresis Guidelines for the use of apheresis in clinical practice, is provided including treatment schedules and “dosing,” patient evaluation and laboratory monitoring, and the proposed mechanism of action. Unique considerations for each treatment, such as their effects on patient management and concurrent therapies, are also discussed. Apheresis is a group of related therapies that can effectively treat several immunologic diseases with a growing but still a limited base of published evidence.

The use of therapeutic apheresis in allergic and immunological diseases – ScienceDirect

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.

https://pubmed.ncbi.nlm.nih.gov/31933569/

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6944925/

Kazuaki Inoue, Tomoki Furuya, Yoko Yokoyama

The apheresis guidelines for digestive diseases are divided into the following four fields: acute liver failure (ALF); ascites; acute pancreatitis (AP); inflammatory bowel disease (IBD).

IBD: Ulcerative colitis (UC) and Crohn’s disease (CD) are the major forms of I BD. Although their etiology is still not fully understood, activated leukocytes are significant factors in their exacerbations. In Japan, granulocyte and monocyte apheresis (GMA) and leukocytapheresis (LCAP) are approved for IBD treatment. They are recommended for remission induction in UC
patients with mild-to-moderate activity, whether steroid-resistant or -dependent. Although GMA is recommended for remission induction in colonic type CD refractory to conventional therapy, its efficacy is lower than in UC patients.

poster at ISFA 2019 pag 100-101

http://www.atalacia.com/isfa/data/abstract.pdf

Dan Turner ¹, Frank M Ruemmele ², Esther Orlanski-Meyer ¹, Anne M Griffiths ³, Javier Martin de Carpi  et al. J Pediatr Gastroenterol Nutr. 2018 Aug;67(2):257-291. doi: 10.1097/MPG.0000000000002035.

Background: The contemporary management of ambulatory ulcerative colitis (UC) continues to be challenging with ∼20% of children needing a colectomy within childhood years. We thus aimed to standardize daily treatment of pediatric UC and inflammatory bowel diseases (IBD)-unclassified through detailed recommendations and practice points. Methods: These guidelines are a joint effort of the European Crohn’s and Colitis Organization (ECCO) and the Paediatric IBD Porto group of European Society of Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN). An extensive literature search with subsequent evidence appraisal using robust methodology was performed before 2 face-to-face meetings. All 40 included recommendations and 86 practice points were endorsed by 43 experts in Paediatric IBD with at least an 88% consensus rate. Results: These guidelines discuss how to optimize the use of mesalamine (including topical), systemic and locally active steroids, thiopurines and, for more severe disease, biologics. The use of other emerging therapies and the role of surgery are also covered. Algorithms are provided to aid therapeutic decision-making based on clinical assessment and the Paediatric UC Activity Index (PUCAI). Advice on contemporary therapeutic targets incorporating the use of calprotectin and the role of therapeutic drug monitoring are presented, as well as other management considerations around pouchitis, extraintestinal manifestations, nutrition, growth, psychology, and transition. A brief section on disease classification using the PIBD-classes criteria and IBD-unclassified is also part of these guidelines. Conclusions: These guidelines provide a guide to clinicians managing children with UC and IBD-unclassified management to provide modern management strategies while maintaining vigilance around appropriate outcomes and safety issues.

https://pubmed.ncbi.nlm.nih.gov/30044357/

https://www.zora.uzh.ch/id/eprint/165387/

Hideki Fujita ¹, Tadashi Terui ¹, Koremasa Hayama ¹, Masashi Akiyama ², Shigaku Ikeda ³, Tomotaka Mabuchi ⁴, Akira Ozawa ⁴, Takuro Kanekura ⁵, Michiko Kurosawa ⁶, Mayumi Komine ⁷, Kimiko Nakajima ⁸, Shigetoshi Sano ⁸, Osamu Nemoto ⁹, Masahiko Muto ¹⁰, Yasutomo Imai ¹¹, Kiyofumi Yamanishi ¹¹, Yumi Aoyama ¹², Keiji Iwatsuki ¹³, J Dermatol . 2018 Nov;45(11):1235-1270. 

 The aim of the guidelines was to provide current information to aid in the treatment of patients with GPP in Japan. Its contents include the diagnostic and severity classification criteria for GPP, its pathogenesis, and recommendations for the treatment of GPP.

https://pubmed.ncbi.nlm.nih.gov/30230572/

Katsuyoshi Matsuoka ¹, Taku Kobayashi ¹, Fumiaki Ueno ^2 3, Toshiyuki Matsui ¹, Fumihito Hirai ¹, Nagamu Inoue ¹, Jun Kato ¹, Kenji Kobayashi ¹, Kiyonori Kobayashi ¹, Kazutaka Koganei ¹, Reiko Kunisaki ¹, Satoshi Motoya ¹, Masakazu Nagahori ¹, Hiroshi Nakase ¹, Fumio Omata ¹, Masayuki Saruta ¹, Toshiaki Watanabe ¹, Toshiaki Tanaka ¹, Takanori Kanai ¹, Yoshinori Noguchi ¹, Ken-Ichi Takahashi ¹, Kenji Watanabe ¹, Toshifumi Hibi ¹, Yasuo Suzuki ¹, Mamoru Watanabe ¹, Kentaro Sugano ¹, Tooru Shimosegawa ¹ , J Gastroenterol. 2018 Mar;53(3):305-353.

Inflammatory bowel disease (IBD) is a chronic disorder involving mainly the intestinal tract, but possibly other gastrointestinal and extraintestinal organs. Although etiology is still uncertain, recent knowledge in pathogenesis has accumulated, and novel diagnostic and therapeutic modalities have become available for clinical use. Therefore, the previous guidelines were urged to be updated. In 2016, the Japanese Society of Gastroenterology revised the previous versions of evidence-based clinical practice guidelines for ulcerative colitis (UC) and Crohn’s disease (CD) in Japanese. A total of 59 clinical questions for 9 categories (1. clinical features of IBD; 2. diagnosis; 3. general consideration in treatment; 4. therapeutic interventions for IBD; 5. treatment of UC; 6. treatment of CD; 7. extraintestinal complications; 8. cancer surveillance; 9. IBD in special situation) were selected, and a literature search was performed for the clinical questions with use of the MEDLINE, Cochrane, and Igaku Chuo Zasshi databases. The guidelines were developed with the basic concept of the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) system. Recommendations were made using Delphi rounds. This English version was produced and edited based on the existing updated guidelines in Japanese.

https://pubmed.ncbi.nlm.nih.gov/29429045/

Evidence-based clinical practice guidelines for inflammatory bowel disease – PMC (nih.gov)

A Hillary Steinhart , Ofer Ben-Bassat Frontline Gastroenterol 2013 Jul;4(3):198-204. doi: 10.1136/flgastro-2012-100171. Epub 2013 Nov 12.

Up to 30% of patients with ulcerative colitis (UC) will require surgical management. The established surgical procedure of choice is colectomy with ileal pouch-anal anastomosis (IPAA) for most patients. Patients with UC who have undergone IPAA are prone to develop inflammatory and non-inflammatory complications. Up to 50% of patients can be expected to experience at least one episode of pouchitis, and most of these patients will experience at least one additional acute episode within 2 years. In other cases, pouchitis might follow a relapsing-remitting course or a chronically active course. The specific aetiology of pouchitis is unknown and the optimal means of diagnosis and classification of pouchitis is not completely agreed upon. Diagnosis of pouchitis based on symptoms alone has been shown to be non-specific due to the fact that symptoms can originate from a myriad of aetiologies, not necessarily inflammatory in nature. As a result, the diagnosis of pouchitis should generally be based on the appropriate constellation of symptoms, combined with endoscopic and histological assessment. Due to the frequently relapsing course of pouchitis, and the fact that the aetiology and pathogenesis are not entirely clear, the long-term management can sometimes be challenging. This review outlines the features suggestive of deviation from ‘normal’ pouch function and provides an approach to the optimal use of diagnostic modalities and medical therapies to treat pouchitis in its various forms.

https://pubmed.ncbi.nlm.nih.gov/28839726/

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5369791/

Maurizio Vecchi ¹, Piero Vernia, Gabriele Riegler, Renata D’Incà, Vito Annese, Siro Bagnoli, Clin Exp Gastroenterol. 2013;6:1-7.

Granulocyte-monocyte apheresis is a relatively new therapy that has been proposed, sometimes with controversial results, for the treatment of inflammatory bowel disease, particularly ulcerative colitis. The aim of the present study was to perform a thorough review of the literature on the application of this type of treatment in ulcerative colitis and discuss the results, in order to provide an opinion on its use which is shared by the involved experts. The review of the literature was performed by searching PubMed with appropriate key words. The results obtained suggest that the major role for this treatment at this moment is for those patients with steroid dependency or with major contraindications to use of steroids. However, promising, albeit very preliminary, results have also been observed in steroid-naïve subjects, and this is of particular interest in consideration of the safety profile of this therapeutic method. As such, the Adacolumn may prove useful in specific subgroups of patients. Future phenotypic, genotypic, and molecular characterization of patients with inflammatory bowel disease might prove useful in defining better those subjects who might benefit most from this treatment modality.

https://pubmed.ncbi.nlm.nih.gov/23323022/

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3541711/pdf/ceg-6-001.pdf

Choon Jin Ooi ¹, Kwong Ming Fock, Govind K Makharia, Khean Lee Goh, Khoon Lin Ling, Ida Hilmi, Wee Chian Lim, Thia Kelvin, Peter R Gibson, Richard B Gearry, Qin Ouyang, Jose Sollano, Sathaporn Manatsathit, Rungsun Rerknimitr, Shu-Chen Wei, Wai Keung Leung, H Janaka de Silva, Rupert Wl Leong, Asia Pacific Association of Gastroenterology Working Group on Inflammatory Bowel Disease

J Gastroenterol Hepatol 2010 Mar;25(3):453-68. doi: 10.1111/j.1440-1746.2010.06241.x.

Inflammatory bowel disease (IBD) is increasing in many parts of the Asia-Pacific region. There is a need to improve the awareness of IBD and develop diagnostic and management recommendations relevant to the region. This evidence-based consensus focuses on the definition, epidemiology and management of ulcerative colitis (UC) in Asia. A multi-disciplinary group developed the consensus statements, reviewed the relevant literature, and voted on them anonymously using the Delphi method. The finalized statements were reviewed to determine the level of consensus, evidence quality and strength of recommendation. Infectious colitis must be excluded prior to diagnosing UC. Typical histology and macroscopic extent of the disease seen in the West is found in the Asia-Pacific region. Ulcerative colitis is increasing in many parts of Asia with gender distribution and age of diagnosis similar to the West. Extra-intestinal manifestations including primary sclerosing cholangitis are rarer than in the West. Clinical stratification of disease severity guides management. In Japan, leukocytapheresis is a treatment option. Access to biologic agents remains limited due to high cost and concern over opportunistic infections. The high endemic rates of hepatitis B virus infection require stringent screening before initiating immune-suppressive agents. Vaccination and prophylactic therapies should be initiated on a case-by-case basis and in accordance with local practice. Colorectal cancer complicates chronic colitis. A recent increase in UC is reported in the Asia-Pacific region. These consensus statements aim to improve the recognition of UC and assist clinicians in its management with particular relevance to the region.

https://pubmed.ncbi.nlm.nih.gov/20370724/

https://onlinelibrary.wiley.com/doi/epdf/10.1111/j.1440-1746.2010.06241.x