Therapy of paraneoplastic pemphigus with Rituximab: a case report and review of literature

Barnadas, M.A.; Roe, Elizabeth; Brunet, Salut; Garcia, Pablo; Bergua, Paula; Pimentel, Lustosa; Puig, L.; A, Francia; Garcia, Roy; Gelpí, Carmen; Sierra, Jorge; Coll, Pere; Alomar, A.

doi:10.1111/j.1468-3083.2005.01345.x

Cited by 63 publications

(51 citation statements)

References 31 publications

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“…29,31 Paraneoplastic pemphigus carries a poor prognosis, with a 2 year 90% mortality rate. 29 Bullous pemphigoid is characterized by tense, subepidermal, blisters of the skin due to antibodies against basement membrane antigens, bullous pemphigoid antigens 1 and 2.…”

Section: Discussionmentioning

confidence: 99%

“…14 In the previous studies of IVIg treatment for patients with pemphigus, it was reported that the autoantibody titers did reduce during the treatment. 16 Rituximab was originally developed to treat refractory, low-grade, follicular, B cell non-Hodgkins lymphoma 2 , but has been used experimentally in various autoimmune diseases including autoimmune blistering disorders (pemphigus vulgaris, 5-7,9-10,12,14,17-27 pemphigus vegetans, 12 pemphigus foliaceus, 5,8,11,12,28 paraneoplastic pemphigus, [29][30][31][32][33] bullous pemphigoid, 34 and epidermolysis bullosa acquisita 13,35,36 …”

Section: Discussionmentioning

confidence: 99%

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Effectiveness and side effects of anti-CD20 therapy for autoantibody-mediated blistering skin diseases: A comprehensive survey of 71 consecutive patients from the Initial use to 2007

Peterson

Chan

2008

TCRM

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Abstract:In order to examine the effi cacy and side effects of the monoclonal antibody anti-CD20 (rituximab) on autoimmune blistering skin diseases, we performed a comprehensive survey of 71 consecutive patients from initial use up to 2007, using the PubMed database. A heterogeneous group of patients, including 51 patients with pemphigus vulgaris, one with pemphigus vegetans, nine with pemphigus foliaceus, fi ve with paraneoplastic pemphigus, four with epidermolysis bullosa acquisita, and one with both bullous pemphigoid and graft vs host disease was included in this survey. Overall the monoclonal antibody seems to be effective in that 69% of patients showed complete response, 25% of patients showed partial response, whereas 6% of patients showed progressive disease. Six deaths occurred in association with the treatment, with four of these deaths in patients with paraneoplastic pemphigus, a disease characteristically resistant to conventional medication and with a high mortality rate. Of note, 11 patients who received combined rituximab and intravenous immune globulin treatments had the best outcome: complete response without any serious side effects. Therefore further investigation on rituximab with controlled clinical trial is a worthy pursuit.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Effectiveness and side effects of anti-CD20 therapy for autoantibody-mediated blistering skin diseases: A comprehensive survey of 71 consecutive patients from the Initial use to 2007

Peterson

Chan

2008

TCRM

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“…[4][5][6] Because our patient had follicular lymphoma, we performed eight cycles of rituximab treatment. 13 The rituximab treatment reduced B cells that produced anti-Before BD After BD desmogleins-1 and -3, and BP180 antibodies, leading to the remission of PNP.…”

Section: Discussionmentioning

confidence: 99%

“…However, it was previously reported that an anti-CD20 antibody, rituximab, successfully induced remission in many patients with pemphigus accompanying follicular lymphoma. [4][5][6] Bronchiolitis obliterans (BO) is an irreversible and lethal lung disease that occurs in chronic graft-versus-host disease patients after allogeneic hematopoietic stem cell transplantation. It was also reported that 30-40% of patients with PNP progress to BO, which represents the major cause of death.…”

Section: Introductionmentioning

confidence: 99%

Rituximab Monotherapy and Rituximab-Containing Chemotherapy Were Effective for Paraneoplastic Pemphigus Accompanying Follicular Lymphoma, but not for Subsequent Bronchiolitis Obliterans

Hirano

Higuchi

Yuki

et al. 2015

J Clin Exp Hematopathol

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A 60-year-old male patient suffered from mild exertional dyspnea, wheezing, and systemic blisters. He was diagnosed with paraneoplastic pemphigus (PNP) with follicular lymphoma in the pancreas head and pelvic cavity. He was first treated with eight cycles of rituximab; his blisters and erosions gradually improved and highly elevated levels of auto-antibodies related to PNP gradually decreased to normal levels. However, obstructive and restrictive respiratory failure still progressed. Computed tomography of the inspiratory and expiratory phases revealed obstructive pulmonary disorder, leading to a diagnosis of bronchiolitis obliterans (BO). The patient underwent plasma exchange and was repeatedly treated with rituximab monotherapy and rituximab-containing chemotherapies, but died 7 months after the diagnosis of BO. Early introduction of rituximabcontaining regimens may be necessary to prevent the development of BO accompanying PNP. However, when a diagnosis of PNP-related BO is made, lung transplantation may also be considered for patients in whom rituximab-containing regimens are effective for PNP. 〔J Clin Exp Hematop 55(2) : 83-88, 2015〕

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“…[22][23][24] B-lymphocytes express the CD20 molecule and can be selectively removed by the use of anti-CD20 therapeutic antibodies such as rituximab. Several recent studies have demonstrated the efficacy of this approach; some patients have long lasting remission after a single course of treatment.…”

Section: Monoclonal Antibody Therapymentioning

confidence: 99%

Pemphigus: a brief review

Groves

2009

Clinical Medicine

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Pemphigus is an uncommon autoimmune blistering disease, mediated by antibodies directed against desmosomal adhesion proteins (most particularly desmogleins 1 and 3) that are responsible for maintaining integrity of the epidermis. This article will discuss its epidemiology, pathogenesis, clinical variants and approaches to treatment. EpidemiologyRobust epidemiologic data on pemphigus incidence are scarce but a recent study from the UK suggests an incidence of 0.68 cases per 100,000 person years. 1 Incidence varies in different parts of the world, being more common in the Near and Middle East than in Western Europe and North America. Those with Jewish ancestry also seem to have a particularly high incidence. 2 All ages can be affected by pemphigus, though it is most common in middle age, and both sexes are affected equally. Several studies on the effect of smoking on pemphigus seem to suggest that, at least in some populations, it has a protective effect. 3 A rare endemic form of pemphigus foliaceus (fogo selvagem) has been identified in parts of Brazil and in North Africa. 4 The cause remains to be identified, but cases seem to cluster in rural villages around rivers and resolve when patients move away from the endemic areas. It has been suggested that the disease may be transmitted by insects living in the area.Before the advent of systemic steroid therapy pemphigus was often a lifethreatening disease. Morbidity is now more often a result of therapy than related to the underlying disorder. Disease duration varies widely, though remission rarely occurs in less than three years. Some patients require ongoing therapy for over 30 years. GeneticsThe genetics of pemphigus are complex. 5,6 Familial cases are uncommon but there are strong associations with certain genes in the MHC complex on chromosome 6, some of which vary according to the population studied. Thus, in Jewish populations DRB1*0402 is highly overexpressed whereas this is less common in Western Europeans. Conversely, the DRB1*1401 allele is highly overexpressed in European and Japanese groups. 6 Interestingly, genetic polymorphisms have recently been identified in the desmoglein 3 antibody target gene that associate with the disease and are in epistasis with the MHC associations. 7 PathogenesisThe primary pathogenic event in all forms of pemphigus is acantholysis, the separation of epidermal keratinocytes from each other. This leads to blister formation within the epidermis and is a key histological diagnostic feature of the disease (Fig 1).The immunopathology of pemphigus is increasingly well understood. 8,9 Antibodies develop against various elements of the desmosomes, particularly desmogleins 1 and 3. Desmoglein 1 is found particularly in the superficial layers of the epidermis; antibodies against this protein alone result in pemphigus foliaceus. Desmoglein 3 is more widely distributed in the lower parts of the epidermis and in mucosal epithelia. Thus, antibodies against this protein are associated with pemphigus vulgaris, typically presenting with muc...

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Therapy of paraneoplastic pemphigus with Rituximab: a case report and review of literature

Cited by 63 publications

References 31 publications

Effectiveness and side effects of anti-CD20 therapy for autoantibody-mediated blistering skin diseases: A comprehensive survey of 71 consecutive patients from the Initial use to 2007

Effectiveness and side effects of anti-CD20 therapy for autoantibody-mediated blistering skin diseases: A comprehensive survey of 71 consecutive patients from the Initial use to 2007

Rituximab Monotherapy and Rituximab-Containing Chemotherapy Were Effective for Paraneoplastic Pemphigus Accompanying Follicular Lymphoma, but not for Subsequent Bronchiolitis Obliterans

Pemphigus: a brief review

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