Autoantibodies against the plakin family proteins as a novel marker for chronic graft-versus-host disease of the lung

Recently published research in Bone Marrow Transplantation explores the aberrant B-cell immunity found in patients with bronchiolitis obliterans syndrome (BOS), illustrating the relationship between BOS, chronic Graft Versus Host Disease (GVHD) and the rare autoimmune skin disease paraneoplastic pemphigus (PNP).

At present, a BOS diagnosis is determined via pulmonary function test (PFT), which is challenging to perform in younger patients, and since BOS patients require lung transplantation at the end stage of the disease, it is critical that diagnosis and treatment of BOS is enhanced to improve prognosis. Thus, the researchers focused their attention toward the shared underlying pathophysiology of both GVHD and PNP - autoantibodies primarily targeted against desmogleins and plakin family proteins.

21 patients who developed chronic GVHD following allogenic hematopoietic stem cell transplantation (HSCT) were included in their study, with nearly half (48%) going on to develop BOS. Immunoprecipitation-immunoblotting (IP-IB) analysis showcased that anti-Epiplakin 1 (EPPK1) antibodies were not found in the blood sera taken from patients pre-transplantation. Additionally, the immunosuppressant antibody-based treatment Rituximab has been given to a group of post-HSCT BOS patients with a response rate of upwards of 62%, further justifying the researcher's focus toward B-cell involvement in BOS patients. These findings warrant further research to validate the use of autoantibodies to plakin family proteins as a biomarker for BOS after allogeneic HSCT.


Kawashima, N., Nishikawa, E., Tsuchisaka, A. et al. Autoantibodies against the plakin family proteins as a novel marker for chronic graft-versus-host disease of the lung. Bone Marrow Transplant (2021).


Tags: GVHD, abstract, antibodies, autoantibodies, plakin, family, proteins, novel marker, chronic, lung