Proposal of new clinical diagnostic criteria for POEMS syndrome

Abstract

ObjectiveTo propose the optimal diagnostic criteria for polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes (POEMS) syndrome using appropriate statistical methods and disease controls.MethodsThis retrospective cohort study included 104 consecutive patients with suspected POEMS syndrome, among whom a gold standard group of 60 patients with definitive POEMS syndrome diagnosis were followed for at least 12 months to strictly exclude other disorders mimicking POEMS syndrome and to confirm response to POEMS syndrome-specific treatment. Thirty patients with chronic inflammatory demyelinating polyradiculoneuropathy (demyelinating polyradiculoneuropathy controls) and 30 with multiple myeloma or immunoglobulin light chain amyloidosis (monoclonal plasma cell proliferation controls) were also included. Logistic regression analyses were performed to determine optimal combination of clinical and laboratory abnormalities, characteristic of POEMS syndrome.ResultsThe diagnostic criteria were statistically defined as the presence of the three major criteria (polyneuropathy (typically demyelinating), monoclonal plasma cell proliferative disorder and elevated vascular endothelial growth factor) and at least two of the four minor criteria (oedema/effusion, skin changes, organomegaly and sclerotic bone lesions), based on best performance by area under the receiver operating characteristic curve analyses. The sensitivity and specificity were 100% and 100%, respectively; the diagnostic accuracy of the proposed criteria was equivalent to somewhat complicated previous criteria.ConclusionsThe statistically defined, simple diagnostic criteria for POEMS syndrome could accelerate early diagnosis and treatment, thereby contribute to better outcome in patients with this serious disease. Prospective larger studies are required to confirm the validity.

Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes syndrome: Diagnosis, treatment and the current status in Japan

“…New diagnostic criteria are simpler and easier to manage, which would be favorable, because they might be used by clinical practitioners from different departments. Additionally, the sensitivity and specificity of both new and current diagnostic criteria are 100%, respectively . These results suggest that we succeeded in simplifying diagnostic criteria without losing diagnostic accuracy.…”

“…Dispenzieri’s criteria have been used internationally; in contrast, in Japan, modified criteria proposed by Misawa and Kuwabara have been used . As the symptoms of POEMS syndrome are diverse, every clinical and laboratory finding should be checked based on the criteria; however, making the diagnosis of POEMS syndrome includes several issues …”

“…To solve the aforementioned issues, and facilitate early and precise diagnosis, new diagnostic criteria were proposed for POEMS syndrome by the following procedure …”

“…Next, four minor criteria (extravascular volume overload, skin changes, organomegaly and sclerotic bone lesions) were identified using logistic regression analysis. All three major criteria and at least two of the four minor criteria are necessary for the diagnosis of POEMS (Table ) …”

“…The optimal diagnostic criteria are required for facilitating early diagnosis and treatment; furthermore, a thorough understanding of the characteristics of the disease is also essential. This review focuses on diagnosis, treatment and the current status of the disease in Japan based on a national survey for POEMS syndrome …”