Anti-BP180 NC16A IgG Titres as an Indicator of Disease Activity and Outcome in Asian Patients with Bullous Pemphigoid

Abstract

Introduction: Anti-BP180 IgG titres were observed to parallel disease activity in case series of bullous pemphigoid (BP). This study aimed to examine whether anti-BP180 titres are an indicator of disease severity, clinical course and outcome in Asian patients with BP. Materials and Methods: This was a prospective observational study conducted between March 2005 and March 2008 in the Immunodermatology Clinic at the National Skin Centre, Singapore. Disease activity and anti-BP180 IgG titres were measured 4-weekly for 12 weeks and during disease fl ares and clinical remission. Associations between anti-BP180 titres and disease activity, disease fl are, clinical remission and cumulative prednisolone dose were examined. Results: Thirty-four patients with newly diagnosed BP were recruited. Median follow-up duration was 3 years. Notable correlations between disease activity and anti-BP180 titres were at baseline (r = 0.51, P = 0.002), and disease fl are (r = 0.85, P <0.001). Lower titres at Week 12 were associated with greater likelihood of clinical remission (P = 0.036). Post hoc, patients with anti-BP180 titres above 87.5 U/mL at time of diagnosis who reached remission within 2 years of diagnosis received signifi cantly higher cumulative doses (mg/kg) of prednisolone (median, 72.8; range, 56.5 to 127.1) than those with titres <87.5 U/mL (median, 44.6; range, 32.5 to 80.8); P = 0.025). Conclusion: Anti-BP180 titres may be a useful indicator of disease activity at time of diagnosis and at disease fl are. Lower titres at Week 12 may predict greater likelihood of clinical remission. Titres above 87.5 U/mL at time of diagnosis may suggest the need for higher cumulative doses of prednisolone to achieve remission within 2 years. Key words: Immunobullous disease, Subepidermal skin blistering, Tense skin blisters

Bullous pemphigoid: The role of type 2 inflammation in its pathogenesis and the prospect of targeted therapy

“…Among these autoantibodies, anti-BP180 antibodies are mainly targeted to the immunodominant region termed NC16A in the extracellular regions ( 53 ). These anti-BP180 NC16A IgG are positively correlated with the Autoimmune Bullous Skin Disorder Intensity Score and Bullous Pemphigoid Disease Area Index (BPDAI) in its subcomponents of erosion/blister, urticaria/erythema and pruritus scores, while the level of anti-BP230 IgG is not correlated with these scores, suggesting that anti-BP180 NC16A IgG can be a useful indicator of BP activity ( 54 , 55 ). The above mechanism can partially explain the formation of blisters but fail to explain the manifestation of itching, erythema, and eosinophilia in BP.…”

Neutrophil-to-lymphocyte ratio and platelet-to-lymphocyte ratio are positively correlated with disease activity of bullous pemphigoid

“…Bullous pemphigoid (BP) is a common autoimmune subepidermal bullous disease characterized by immunoglobulin G (IgG) autoantibodies mainly against the NC16A domain of BP180 (type XVII collagen, 180 kDa), causing tense bullae [1,2]. IgG anti-BP180 NC16A antibodies are related to the disease severity of BP [3][4][5][6][7][8][9][10][11][12][13][14][15][16].…”