TY - JOUR

T1 - How “benign” is cutaneous mastocytosis? A Danish registry-based matched cohort study

AU - Kibsgaard, Line

AU - Deleuran, Mette

AU - Flohr, Carsten

AU - Langan, Sinéad

AU - Braae Olesen, Anne

AU - Vestergaard, Christian

PY - 2020/9

Y1 - 2020/9

N2 - Background: There are limited estimates of the incidence rates (IRs) of mastocytosis, and only a few studies have addressed the long-term consequences of living with these diagnoses. Previous reports have shown that systemic mastocytosis is associated with leukemic transformations and an increased risk of death as opposed to cutaneous mastocytosis (CM) and indolent systemic mastocytosis (ISM), which have benign diagnoses with life expectancy rates similar to those of the background population. Objective: This study aimed to analyze the incidence and mortality of mastocytosis. Methods: A population-based matched cohort study of patients with mastocytosis between 1 January 1, 1977 and 31 December 31, 2014 was identified from the Danish National Health Registries. IRs of CM, ISM, and pediatric mastocytosis were highlighted. Survival estimates were compared with those of a healthy background population, using a Cox proportional hazard model. Results: A total of 1461 patients with mastocytosis were identified. The annual IR of overall mastocytosis was 1.1 per 100,000 person years (95% confidence interval [CI], 1.0–1.2). Among children, the IR was 1.8 per 100,000 person years (95% CI, 1.6–2.1). The prevalence of any comorbidity was twice as high among patients with mastocytosis compared with the population without mastocytosis (odds ratio: 2.1; 95% CI, 1.8–2.5). The Charlson Comorbidity Index–adjusted mortality among adult patients with mastocytosis was HRCutaneous Mastocytosis 1.2 (95% CI, 0.8–1.9), HRIndolent Systemic Mastocytosis 1.9 (95% CI 1.4–2.5), and HRSystemic Mastocytosis 4.2 (95%, CI 1.9–9.4), respectively. Conclusion: Based on an entire nation, with free health care at the point of access, we estimated an annual IR of mastocytosis and its subgroups. We discovered that patients with ISM had an increased risk of death compared with the general population. Our data supported the overall benign nature of CM diagnosed after age 2 years.

AB - Background: There are limited estimates of the incidence rates (IRs) of mastocytosis, and only a few studies have addressed the long-term consequences of living with these diagnoses. Previous reports have shown that systemic mastocytosis is associated with leukemic transformations and an increased risk of death as opposed to cutaneous mastocytosis (CM) and indolent systemic mastocytosis (ISM), which have benign diagnoses with life expectancy rates similar to those of the background population. Objective: This study aimed to analyze the incidence and mortality of mastocytosis. Methods: A population-based matched cohort study of patients with mastocytosis between 1 January 1, 1977 and 31 December 31, 2014 was identified from the Danish National Health Registries. IRs of CM, ISM, and pediatric mastocytosis were highlighted. Survival estimates were compared with those of a healthy background population, using a Cox proportional hazard model. Results: A total of 1461 patients with mastocytosis were identified. The annual IR of overall mastocytosis was 1.1 per 100,000 person years (95% confidence interval [CI], 1.0–1.2). Among children, the IR was 1.8 per 100,000 person years (95% CI, 1.6–2.1). The prevalence of any comorbidity was twice as high among patients with mastocytosis compared with the population without mastocytosis (odds ratio: 2.1; 95% CI, 1.8–2.5). The Charlson Comorbidity Index–adjusted mortality among adult patients with mastocytosis was HRCutaneous Mastocytosis 1.2 (95% CI, 0.8–1.9), HRIndolent Systemic Mastocytosis 1.9 (95% CI 1.4–2.5), and HRSystemic Mastocytosis 4.2 (95%, CI 1.9–9.4), respectively. Conclusion: Based on an entire nation, with free health care at the point of access, we estimated an annual IR of mastocytosis and its subgroups. We discovered that patients with ISM had an increased risk of death compared with the general population. Our data supported the overall benign nature of CM diagnosed after age 2 years.

KW - Comorbidity-adjusted analyses

KW - Danish National Patient Registry

KW - Epidemiology

KW - Mastocytosis

KW - Survival analyses

UR - http://www.scopus.com/inward/record.url?scp=85087392895&partnerID=8YFLogxK

U2 - 10.1016/j.ijwd.2020.05.013

DO - 10.1016/j.ijwd.2020.05.013

M3 - Journal article

C2 - 33015290

AN - SCOPUS:85087392895

VL - 6

SP - 294

EP - 300

JO - International Journal of Women's Dermatology

JF - International Journal of Women's Dermatology

SN - 2352-6475

IS - 4

ER -