Bullous pemphigoid (BP) is a chronic debilitating autoimmune blistering disease that frequently occurs in the elderly population. Previous studies have suggested a high morbidity and mortality associated with BP. However, relatively few studies have investigated prognostic factors of BP mortality, and they showed considerably various results. This meta-analysis aimed to quantitatively assess the association between several potential prognostic factors and risk of mortality in bullous pemphigoid. A comprehensive search was performed using Pubmed, Embase, and Cochrane Library. Cohort studies that assessed prognostic factors of BP mortality were included. Random-effects model was utilized to calculate the pooled hazard ratio (HR). Publication bias was evaluated qualitatively by constructing a funnel plot and quantitatively by conducting Egger’s test. 14 studies were included comprising 2499 patients. Combined HRs suggested that advanced age (HR 1.63, 95% CI 1.34–1.97), presence of circulating antibodies (HR 1.77, 95% CI 1.20–2.62), concomitant dementia (HR 2.01, 95% CI 1.22–3.33), and concomitant stroke (HR 1.86, 95% CI 1.29–2.67) have an unfavorable impact on patient survival. Gender, disease extent, mucosal involvement, and indirect immunofluorescence result were not shown to be linked to mortality by our analysis. This study indicated that BP patients with older age, circulating antibodies, dementia, and stroke are at greater risk of mortality. Clinicians should be aware of this association and utilize this information in patient education and treatment process.Electronic supplementary materialThe online version of this article (doi:10.1007/s00403-017-1736-1) contains supplementary material, which is available to authorized users.

Bullous pemphigoid (BP) is a chronic autoimmune blistering disorder that has a predilection for the elderly. It is characterized by a poor prognosis due to its high mortality rate and the tendency to relapse. The relapse rate of BP ranges from 27.87% to 53% after disease remission, while the majority of relapses occur early (within 6 months) during remission. Clinical interventions aimed to prevent early relapses could reduce potential complications from first-line treatment and make follow-up care easier for clinicians in practice, thus improving the prognosis of BP and the quality of patients' lives. In this article, we attempt to review previous studies from the medical literature concerning relapse and risk factors related to relapse in BP patients. Treatments found to be able to reduce or increase relapse rate were also discussed. We propose that clinicians be aware of these risk factors and manage proper treatment accordingly. Key Messages   • The relapse rate of bullous pemphigoid ranges from 27.87% to 53% after disease remission, while the majority of relapses occur early (within 6 months) during remission.   • Ageing, disease severity, neurological disorder, serum ECP, BAFF, IL-17, IL-23, CXCL10 and anti-BP180 antibody levels have been shown to be related to relapse, while confirmation studies are needed before using them for guidance for relapse prevention.   • Longer usage of corticosteroid with a low dose, combination of immunosuppressants and intravenous immunoglobulin are helpful in lowering relapse rate.

Peripheral primitive neuroectodermal tumors (PNETs) constitute very rare and aggressive malignancies. To date, there are no standard guidelines for management of peripheral PNETs due to the paucity of cases arising in various body sites. Therapeutic approach is derived from Ewing sarcoma family, which currently remains multimodal. Our study retrospectively analyzed 86 PNET patients from February 1, 1998 to February 1, 2018 at Peking Union Medical College Hospital with an additional 75 patients from review of literature. The clinicopathologic and treatment plans associated with survival was investigated. Surgery, chemotherapy, female sex, small tumor size, no lymph node metastasis, R0 surgical resection, (vincristine + doxorubicin + cyclophosphamide)/(isophosphamide + etoposide) regimen, and more than 10 cycles of chemotherapy were associated with improved overall survival in univariate analysis. Surgery, more than 10 cycles of chemotherapy, and small tumor size were independent prognostic factors for higher overall survival. Our data indicates that multimodal therapy is the mainstay therapeutic approach for peripheral PNET.