Paraneoplastic pemphigus

2007 ◽  
Vol 148 (21) ◽  
pp. 979-983 ◽  
Author(s):  
Klaudia Preisz ◽  
Sarolta Kárpáti
Keyword(s):  
Graft Versus Host Disease ◽  
Bronchiolitis Obliterans ◽  
Host Disease ◽  
Graft Versus Host ◽  
Paraneoplastic Pemphigus ◽  
Anti Cd20

A paraneoplasticus pemphigus malignus vagy benignus tumorokhoz társuló autoimmun hólyagos megbetegedés diagnosztikus és immunológiai kritériumait 1990-ben fektette le Anhalt . Klinikailag súlyos, fájdalmas, mélyre terjedő bőr- és nyálkahártyatünetek jellemzik. A bőrtünetek polimorf jellegűek, általában dominál a hólyagképződés. Egyes esetekben, az ún. „graft-versus-host-disease” formákban előfordul, hogy kizárólag papulosus, lichenoid bőrtünetek észlelhetők, hólyagképződés nincs, vagy csak később jelenik meg. Elsősorban ezen alcsoport betegeiben a bőr- és nyálkahártyatünetek mellett súlyos dyspnoét okozó pulmonalis érintettség is kialakul, melynek hátterében bronchiolitis obliterans áll. A paraneoplasticus pemphigus diagnosztikájában alapvető fontosságú a bőr/nyálkahártyák direkt immunfluoreszcens, továbbá a szérum indirekt immunhisztológiai és immunoblot vizsgálata. Az eddig azonosított autoantigének döntő többsége a plakincsalád tagja: envoplakin (210 kDa), periplakin (190 kDa), plectin (~500 kDa), desmoplakin I (250 kDa), desmoplakin II (210 kDa), bullosus pemphigoid antigén 1 (230 kDa). A plakinok mellett a desmosomalis cadherinek közé sorolt desmoglein 1 és 3 a bullosus pemphigoid antigén 2 (180 kDa), a desmocollin 2 és 3, továbbá egy még nem azonosított, 170 kDa molekulatömegű transzmembrán fehérje szintén autoantigénje a kórképnek. A paraneoplasticus pemphigus nagy mortalitású kórkép, az esetek több mint 90%-ában halálos kimenetelű. A háttérben álló tumor eltávolítása mellett a bázisterápia továbbra is a nagy dózisú szisztémás szteroidkezelés, melyet citosztatikumok, immunmodulánsok adásával egészítenek ki. Szóba jön ezenkívül plasmapheresis, plazmacsere, photopheresis, nagy dózisú intravénás immunglobulin és anti-CD20 monoklonális antitest (rituximab) adása is.

Effect of Rituximab on Pulmonary Function in Bronchiolitis Obliterans Syndrome due to Graft-Versus-Host-Disease

Lung ◽  
2017 ◽  
Vol 195 (6) ◽  
pp. 781-788 ◽  
Author(s):  
Kyle R. Brownback ◽  
Laura A. Thomas ◽  
Joseph P. McGuirk ◽  
Siddhartha Ganguly ◽  
Christopher Streiler ◽  
...  
Keyword(s):  
Pulmonary Function ◽  
Graft Versus Host Disease ◽  
Bronchiolitis Obliterans ◽  
Bronchiolitis Obliterans Syndrome ◽  
Host Disease ◽  
Graft Versus Host

Ruxolitinib is an effective steroid sparing agent in bronchiolitis obliterans due to chronic graft-versus-host-disease

2019 ◽  
Vol 55 (6) ◽  
pp. 1194-1196 ◽  
Author(s):  
Christopher Streiler ◽  
Faizan Shaikh ◽  
Christian Davis ◽  
Sunil Abhyankar ◽  
Kyle R. Brownback
Keyword(s):  
Graft Versus Host Disease ◽  
Bronchiolitis Obliterans ◽  
Host Disease ◽  
Graft Versus Host ◽  
Steroid Sparing

scholarly journals Predictive Value of Bronchiolitis Obliterans Syndrome Stage 0p in Chronic Graft-versus-Host Disease of the Lung

2015 ◽  
Vol 21 (6) ◽  
pp. 1127-1131 ◽  
Author(s):  
Sameem Abedin ◽  
Gregory A. Yanik ◽  
Thomas Braun ◽  
Attaphol Pawarode ◽  
John Magenau ◽  
...  
Keyword(s):  
Graft Versus Host Disease ◽  
Predictive Value ◽  
Bronchiolitis Obliterans ◽  
Bronchiolitis Obliterans Syndrome ◽  
Host Disease ◽  
Graft Versus Host

scholarly journals C5aR1 regulates T follicular helper differentiation and chronic graft-versus-host disease bronchiolitis obliterans

JCI Insight ◽  
2018 ◽  
Vol 3 (24) ◽  
Author(s):  
Divya A. Verghese ◽  
Nicholas Chun ◽  
Katelyn Paz ◽  
Miguel Fribourg ◽  
Trent M. Woodruff ◽  
...  
Keyword(s):  
Graft Versus Host Disease ◽  
Bronchiolitis Obliterans ◽  
Host Disease ◽  
Graft Versus Host ◽  
Follicular Helper

Risk Reduction of Chronic Graft Versus Host Disease by Anti-CD20 Treatment (Rituximab).

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 5567-5567
Author(s):  
Marlies E.H.M. Van Hoef
Keyword(s):  
B Cell ◽  
Risk Reduction ◽  
Graft Versus Host Disease ◽  
Cell Depletion ◽  
B Cell Depletion ◽  
Host Disease ◽  
Medline Search ◽  
Graft Versus Host ◽  
Anti Cd20 ◽  
Study Plan

Abstract Introduction: Chronic graft versus host disease (cGVHD) occurs in 30–50% of adults transplanted by non-myeloablative (reduced intensity conditioning) allogeneic transplant, whereas it is rare in children. The syndrome complex manifests itselves as auto-immune disease. Dysregulation of B-cell function has also been reported in auto-immune diseases. This is a rationale for B-cell depletion. We considered that B-cell depletion might have a therapeutic effect in cGVHD and reduce the risks associated with cGVHD. Based on these assumptions we applied risk reduction principles and defined a plan for cure of cGVHD. Methods: To identify the risks associated with B-cell depletion in cGVHD we performed a medline search on anti-CD20 or B-cell depletion or rituximab and cGVHD. The publications were analyzed and those applicable to the topic evaluated. Clinical research methods were applied to define a plan for risk reduction of cGVHD. Results: The medline search revealed that rituximab was used for B-cell depletion in cGVHD and in auto-immune diseases. In advanced cGVHD complete and partial remissions of a variety of manifestations of cGVHD were reported. Complete remissions could also be induced early during the disease course of single manifestations of cGVHD not responsive to immune suppressive treatment or as initial treatment. The dose of rituximab varied from 1 to 4 weekly courses of rituximab 375 mg/m2, with repetitions of this schedule in advanced disease to induce complete response. During reported follow-up responding manifestations did not recur. Rituximab treatment was well tolerated with appropriate anti-allergic prophylaxis. The results support the concept that anti-CD20 treatment might cure cGVHD manifestations at first diagnosis; it is too early to define whether it also serves as prophylactic for development of new manifestations. The observations were discussed with the company marketing rituximab and a study plan was designed. This study plan is being discussed with study centers for risk reduction of cGVHD by rituximab. The risk reduction plan will be presented

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