Acquired Haemophilia A in DPP4 Inhibitor-induced Bullous Pemphigoid as Immune Reconstitution Syndrome

Sugiyama, Seiko; Tanaka, Ryota; Hayashi, Hiroaki; Izumi, Kiyotaka; Nishie, Wataru; Aoyama, Yumi

doi:10.2340/00015555-3539

Cited by 5 publications

(2 citation statements)

References 14 publications

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“…It is also important to determine whether inflammatory responses occurring after DPP-4i cessation could result in changes in the proportion of blood cells and the NLR. The rationale for analyzing the change in the NLR after DPP-4i cessation is based on our findings that acquired hemophilia occurred as a manifestation of IRIS in a patient with DPP-4i-BP in association with the cessation of DPP-4i and prednisone [ 20 ] and that sudden exacerbations of DPP-4i-BP associated with CMV esophagitis (Case 1) occurred shortly after the cessation of pulse corticosteroids. Because the rapid recovery of lymphocytes observed in IRIS occurring after DPP-4i cessation could be reflected by a decrease of the NLR during the post-cessation stage, we could interpret a decrease in the NLR at short-term follow-up intervals as indicating that robust immune recovery as a manifestation of IRIS would occur late after the cessation of DPP-4is.…”

Section: Discussionmentioning

confidence: 99%

Anti-Cytomegalovirus Therapy: Whether and When to Initiate, Those Are the Questions

2022

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Cytomegalovirus (CMV) reactivation in patients with autoimmune bullous disease (AIBD) or severe drug eruption treated with immunosuppressive therapy was traditionally thought to be merely an epiphenomenon of the underlying immunosuppression. However, a detailed review of the clinical course of these patients revealed that CMV reactivation occurs upon rapid immune recovery, which is termed immune reconstitution inflammatory syndrome (IRIS), and that the timely initiation of anti-CMV therapy, when combined with maintenance doses of immunosuppressive agents, contributes to a rapid resolution of severe infectious complications thought to be refractory to conventional immunosuppressive therapies and unrelated to CMV reactivation. Thus, CMV reactivation resulting in fatal outcomes (CMV-IRIS) can be prevented by the timely detection of CMV DNA or antigens in the blood and by rapidly starting anti-CMV therapy while maintaining immunosuppressive therapy. Anti-CMV therapy is highly recommended for patients with CMV-IRIS or severe drug eruption who have risk factors for CMV reactivation resulting in fatal outcomes.

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Section: Discussionmentioning

confidence: 99%

Anti-Cytomegalovirus Therapy: Whether and When to Initiate, Those Are the Questions

2022

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“…Other treatments include cyclophosphamide, high-dose immunoglobulins and immunoabsorption ( 13 , 23 , 55 ). Tapering of immunosuppressive agents is more prudent because immune reconstitution inflammatory syndrome presenting as AHA has been reported in BP patients ( 56 ). Immune recovery, which occurs rapidly after inadequate withdrawal of the drug, may trigger subsequent AHA recurrence.…”

Section: Acquired Hemophilia Amentioning

confidence: 99%

Associations between bullous pemphigoid and hematological diseases: Literature review on mechanistic connections and possible treatments

Yang

Zhao²,

Yang³

et al. 2023

Front. Immunol.

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Bullous pemphigoid is an autoimmune blistering disorder that primarily occurs in elderly patients. Reports indicate that BP coexists with various hematological diseases, including acquired hemophilia A, hypereosinophilic syndrome, aplastic anemia, autoimmune thrombocytopenia, and hematological malignancies. Early identification of these comorbidities contributes to a better control and reduced mortality. This article details the atypical clinical manifestations of BP when associated with hematological diseases, specific diagnostic strategies, underlying mechanistic connections, and possible treatments. Cross-reactivity between autoantibodies and exposed abnormal epitopes, shared cytokines and immune cells, together with genetic susceptibility are the most common connections between BP and hematological diseases. Patients were most often successfully treated with oral steroids combined with medications specifically targeting the hematological disorders. However, the individual comorbidities require specific considerations.

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Expanding Concept of Immune Reconstitution Inflammatory Syndrome: A New View Regarding How the Immune System Fights Exogenous Pathogens

Aoyama

Shiohara

2020

Current Topics in Environmental Health and Preventive Medicine

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Acquired Haemophilia A in DPP4 Inhibitor-induced Bullous Pemphigoid as Immune Reconstitution Syndrome

Cited by 5 publications

References 14 publications

Anti-Cytomegalovirus Therapy: Whether and When to Initiate, Those Are the Questions

Anti-Cytomegalovirus Therapy: Whether and When to Initiate, Those Are the Questions

Associations between bullous pemphigoid and hematological diseases: Literature review on mechanistic connections and possible treatments

Expanding Concept of Immune Reconstitution Inflammatory Syndrome: A New View Regarding How the Immune System Fights Exogenous Pathogens

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