Department of Otolaryngology, Head and Neck Surgery and Maxillofacial Surgery, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel; Sackler School of Medicine, Tel-Aviv University, Tel Aviv, Israel. Electronic address:
Published: May 2023
AI Article Synopsis
Article Abstract
Objective: This study aimed to present our experience in treating laryngeal pemphigoid (LP) patients, including disease course, treatment and treatment response, and to search for predictors of response to treatment.
Study Design: A retrospective cohort study.
Methods: The medical records of all patients with LP from March 2013 to August 2020 were reviewed. Potential relationships between disease severity and response to treatment and demographics, diagnosis, extent of laryngeal and extra-laryngeal involvement, comorbidities, immunostaining, and serology profile were explored.
Results: Eight patients were included in the study (seven females, one male, mean age 79 years, mean follow-up 22 months). Diagnoses included mucous membrane pemphigoid (MMP, n = 5), bullous pemphigoid (BP, n = 3). Two patients achieved complete laryngeal remission, four achieved partial remission, and two had no remission. The time to achieve laryngeal disease control was longer than for extra-laryngeal disease (P = 0.02). Potential associations were found between the absence of immunoglobulin G (IgG)-type auto-antibodies deposits in the basement membrane zone and a laryngeal disease that responded to topical corticosteroids and between the presence of BP180-C-terminal IgG auto-antibodies and a resistant rapidly progressive laryngeal disease.
Conclusions: LP has a spectrum of severity, variable response and is more resistant to treatment. The absence of IgG-type auto-antibodies may indicate a response to topical corticosteroids. Based on our limited observation, the presence of IgG-type auto-antibodies that target BP180-C-terminal domain may indicate a more severe scarring disease. Early recognition of these "high-risk patients" will allow early initiation of advanced systemic treatment that may prevent the irreversible effects of scarring.
Department of Otolaryngology, Head and Neck Surgery and Maxillofacial Surgery, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel; Sackler School of Medicine, Tel-Aviv University, Tel Aviv, Israel. Electronic address:
Objective: This study aimed to present our experience in treating laryngeal pemphigoid (LP) patients, including disease course, treatment and treatment response, and to search for predictors of response to treatment.
Study Design: A retrospective cohort study.
Methods: The medical records of all patients with LP from March 2013 to August 2020 were reviewed.
Anticardiolipin antibodies (ACA) have been described in association with thrombosis and several vascular diseases and may be involved in retinal vasculitis. To investigate this question we tested sera from 86 patients with posterior uveitis and 100 healthy controls for the presence of anticardiolipin antibodies. The antibody was present in 15 patients: in 7 of the patients with acute retinal necrosis (n = 24), in 4 with idiopathic retinal vasculitis (n = 10), 3 with syphilis (n = 6) and in one SLE patient with retinal vasculitis.
Functional in vitro studies of B cells from 3 murine strains which develop severe early onset SLE-like disease with marked polyclonal B cell hyperactivity lead to the following conclusions: 1.) B cell proliferation and differentiation in lupus mice remains dependent on accessory signals of either macrophage or T cell origin; 2.) B cells from BXSB, NZB/W and MRL/1 mice appear to require the same number and type of signals as normal B cells to undergo polyclonal or antigen-directed responses.
Anti-red auto-antibodies were found in 69 out of 892 000 blood donors aged between 20 and 60 years, 63 of whom were followed for up to five years. This would suggest an overall incidence of 1 in 13 000 members of a "normal" population. Uncontrolled methyl-dopa treatment could be incriminated in 25% of cases.
288 sera with red cell auto-antibodies and 141 control sera were investigated for cold lymphocytotoxins (CLCT). An incidence of 72% and 40% respectively was found in patients with cold haemagglutinin disease and in those with warm IgG type AHA. The absence of increased frequency in idiopathic or methyl-dopa induced asymptomatic red cell auto-immunization would suggest that CLCT might reflect an advanced degree of auto-immune disorder.
