Fibrosis is a consequence of the inflammatory response. When fibrotic tissue becomes excessive, it can have diverse pathophysiological effects on a number of organ systems. The mechanisms underlying fibrosis and approaches to therapy are reviewed.

https://www.nejm.org/doi/suppl/10.1056/NEJMra1300575/suppl_file/nejmra1300575_appendix.pdf

Fibrosis — A Common Pathway to Organ Injury and Failure

A. Khosroshahi, Z. S. Wallace, J. L. Crowe, T. Akamizu, A. Azumi, M. N. Carruthers, S. T. Chari, E. Della-Torre, L. Frulloni, H. Goto, P. A. Hart, T. Kamisawa, S. Kawa, M. Kawano, M. H. Kim, Y. Kodama, K. Kubota, M. M. Lerch, M. L€ ohr, Y. Masaki, S. Matsui, T. Mimori, S. Nakamura, T. Nakazawa, H. Ohara, K. Okazaki, J. H. Ryu, T. Saeki, N. Schleinitz, A. Shimatsu, T. Shimosegawa, H. Takahashi, M. Takahira, A. Tanaka, M. Topazian, H. Umehara, G. J. Webster, T. E. Witzig, M. Yamamoto, W. Zhang, T. Chiba, and J. H. Stone

https://onlinelibrary.wiley.com/doi/pdf/10.1002/art.39132

International Consensus Guidance Statement on the Management and Treatment of IgG4-Related Disease

https://www.nejm.org/doi/pdf/10.1056/NEJMra1104650

IgG4-related disease

https://gut.bmj.com/content/gutjnl/58/12/1680.full.pdf

Recent advances in autoimmune pancreatitis

https://vbn.aau.dk/ws/files/268549086/OpenAccessArticle.pdf

Guidelines for the understanding and management of pain in chronic pancreatitis

Objectives To evaluate the efficacy of rituximab (RTX) in IgG4-related disease (IgG4-RD) in an open-label pilot trial. Methods We treated 30 IgG4-RD patients with two doses of RTX (1000 mg each). The participants were either treated with RTX alone (n = 26; 87%) or required to discontinue baseline glucocorticoids (GC) within 2 months (n = 4; 13%). Disease activity was measured by the IgG4-RD Responder Index (IgG4-RD RI) and physician's global assessment (PGA). Disease response was defined as the improvement of the IgG4-RD RI by two points. The primary outcome, measured at 6 months, was defined as: (1) decline of the IgG4-RD RI ≥2 points compared with baseline; (2) no disease flares before month 6; and (3) no GC use between months 2 and 6. Complete remission was defined as an IgG4-RD RI score of 0 with no GC use. Results Disease responses occurred in 97% of participants. The baseline IgG4-RD RI and PGA values, 11±7 and 63±22 mm, respectively, declined to 1±2 and 11±16 mm at 6 months (both p Conclusions RTX appears to be an effective treatment for IgG4-RD, even without concomitant GC therapy. Trial registration number ClinicalTrials.gov identifier: NCT01584388.

https://ard.bmj.com/content/annrheumdis/74/6/1171.full.pdf

Rituximab for IgG4-related disease: a prospective, open-label trial

Guarantor of the article: Mark Topazian, MD.

Specific author contributions: BK Abu Dayyeh: conception and design, data analysis, drafting of the manuscript. Darwin Conwell: data collection and analysis, critical revision of the manuscript. NS Buttar: data collection and analysis, critical revision of the manuscript. PA Hart, BL Bick, ST Chari, JE Clain, FC Gleeson, MJ Levy, RK Pearson, BT Petersen, S Suleiman, PA Banks, E Rajan, SS Vege: critical revision of the manuscript. V Kadilaya: data collection. LS Lee: data collection. S Chowdhary: data collection. M Topazian: conception and design, data collection, data analysis, critical revision of the manuscript, overall study supervision. All authors: approved the final version of the manuscript.

Financial support: Division of Gastroenterology and Hepatology, Department of Internal Medicine, Mayo Clinic, Rochester, MN; Harvard Digestive Diseases Center (DC, NIH 5 P30 DK034854-24); and NIH NIDDK (DC, 1R21 DK081703-01A2), Burrill Family Research Grant. These entities did not participate in the conception or design of the study, the analysis or interpretation of data, the writing of the manuscript, or approval of the final manuscript.

Potential competing interests: None.

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Pancreatic Juice Prostaglandin E2 Concentrations Are Elevated in Chronic Pancreatitis and Improve Detection of Early Disease

BACKGROUND Soluble signaling molecules may play an important role in malignant pathogenesis. We hypothesize that perioperative cytokine levels are associated with outcomes in patients with pancreatic adenocarcinoma (PDAC) undergoing surgical resection. METHODS One hundered and eighteen patients with benign or malignant pancreatic disease were enrolled in a prospective study through a protocol for banking biologic samples. Peripheral blood was drawn at time of operation, and a multiplex cytokine assay was performed. Statistical analysis was via χ2 and Kaplan Meier methods. RESULTS Of 118 patients enrolled, 85 (72%) had a diagnosis of PDAC, and 60 (70%) ultimately underwent partial pancreatectomy. Cytokine levels were not associated with postoperative complications in this initial cohort. A plasma level of monocyte chemoattractant protein-1 (MCP-1) pg/mL ≤118 was associated with better overall survival (OS) (median survival 21 months vs 12.8 months, P = 0.023), as was non-detectable interleukin-8 (IL-8) (19 months) versus detectable IL-8 (12.8 months, P = 0.05). Patients with both MCP-1 >118 pg/mL and detectable IL-8 had a median survival of 10.6 months (P = 0.028). CONCLUSIONS MCP-1 and IL-8 cytokine levels are associated with decreased survival following pancreatectomy for PDAC, and may be useful biomarkers. Measurement of these cytokine levels at different time points in future investigations will be important to validate these findings.

Perioperative cytokine levels portend early death after pancreatectomy for ductal adenocarcinoma.

Background
Autoimmune pancreatitis is the prototypical manifestation of immunoglobulin G4-related disease, a fibroinflammatory syndrome that can affect virtually any organ. Rarely, skin involvement has been reported in immunoglobulin G4-related disease. Isolated case reports have described other distinct associated dermatoses.

Philip A. Hart

Papers

Rituximab for IgG4-related disease: a prospective, open-label trial

Pancreatic Juice Prostaglandin E2 Concentrations Are Elevated in Chronic Pancreatitis and Improve Detection of Early Disease

Perioperative cytokine levels portend early death after pancreatectomy for ductal adenocarcinoma.

Dermatologic Disorders in 118 Patients with Autoimmune (Immunoglobulin G4-Related) Pancreatitis: A Retrospective Cohort Analysis

Unlikely Organisms During an Evaluation for Abdominal Pain in an Immunosuppressed Patient: 2410