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Sjögren’s syndrome is a heterogeneous autoimmune disorder that may be associated with systemic manifestations involving multiple organs. We herein reported a rare combination of immunoglobulin A nephropathy; non-cirrhotic portal fibrosis; and polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes (POEMS) syndrome in a 15-year follow-up of a female patient initially diagnosed with Sjögren’s syndrome. The patient had excessive lymphoproliferation featured by lymphadenopathy and hyperglobulinemia. The diagnoses of immunoglobulin A nephropathy and non-cirrhotic portal fibrosis were confirmed by renal and liver biopsies. She received prolonged corticosteroids and immunosuppressive drugs, which improved immunoglobulin A nephropathy but did not hinder the progression of portal fibrosis, leading to intractable variceal bleeding. The patient died of repeated hematemesis despite endoscopic variceal ligation. Valuable pathological information of multi-organ involvement as well as detailed clinical course were presented to facilitate further understanding of this rare entity. Excessive lymphoproliferation might play an important role in the progression of systemic complications in Sjögren’s syndrome, which requires prolonged immunosuppression and organ-specific treatment.

POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes) is a rare paraneoplastic syndrome, caused by a plasma cell proliferative disorder, which is most commonly lambda restricted. The neurological hallmark, which forms one of the mandatory criteria for diagnosis, is a subacute onset demyelinating neuropathy, which can be rapidly disabling and painful. A number of multi-system features are also characteristic of this disorder, and certainly not restricted to those included in its acronym, which though limited, remains a useful and memorable name, helping distinguish POEMS syndrome from other paraproteinaemic neuropathies. The discovery of vascular endothelial growth factor (VEGF) in association with POEMS syndrome has been extremely useful in aiding clinical diagnosis, and monitoring response to treatment, as well as helping understand the underlying mechanism of disease. Interestingly, however, treatment targeting VEGF has been disappointing, suggesting other disease mechanisms or inflammatory processes are also important. Current understanding of the pathogenesis of POEMS syndrome is outlined in detail in the accompanying article by Cerri et al. Here, we review the clinical features of POEMS syndrome, differential diagnosis and available treatment options, based on current literature.

Set in a district general hospital, this case series explores two individuals who developed a rare multisystemic syndrome:; polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin abnormalities (POEMS). Diagnostic journey, trajectory of disease and outcomes are compared. Both patients presented to healthcare numerous times and saw multiple specialists for symptoms resulting predominantly from volume overload and neuropathy, prior to being admitted with atypical ischaemic stroke. During their admission, diagnosis was made after atypical intracranial arterial stenoses, sensory neuropathy not in keeping with their stroke, plasmacytoma detection and confirmatory raised vascular endothelial growth factor (VEGF) levels. POEMS is highly treatment responsive, survival rate improving with earlier diagnosis. Both patients were transferred to specialist centres for chemotherapy. Unfortunately, patient outcomes significantly differ, one having favourable recovery, while the other experiencing treatment-resistance disease requiring local repatriation for withdrawal of care. We identify challenges faced by both patients and the medical team, and discuss the importance of the general physician within the world of specialist medicine, in order to provide holistic, comprehensive patient care.

POEMS syndrome is a rare, chronic, disabling paraneoplastic disorder characterized by peripheral neuropathy, organomegaly, endocrinopathy, monoclonal plasma cells disorder and skin changes. Diagnosis relies on the fulfillment of a set of clinical criteria of which polyneuropathy and a monoclonal plasma cell dyscrasia are early and essential features. Treatment may be either local or systemic and is aimed at the monoclonal plasma cell disorder. Our knowledge of the pathogenesis underlying the POEMS syndrome has advanced greatly over the past years, favoring an important progression in the recognition and management of this disorder. Here, we discuss the recent literature that has advanced our knowledge of the pathogenesis and clinical management of the polyneuropathy in POEMS syndrome.

This article reports a rare case of a patient with POEMS syndrome who developed secondary myelodysplastic syndrome (MDS) two years after undergoing autologous stem cell transplantation (ASCT). The patient was initially misdiagnosed with chronic inflammatory demyelinating polyneuropathy (CIDP) due to symptoms of limb numbness and weakness. Two years later, the diagnosis was corrected to POEMS syndrome. After induction therapy with the lenalidomide-dexamethasone (RD) regimen, ASCT is performed and partial remission is achieved. And lenalidomide was used for maintenance therapy. Over a year later, he was infected with SARS-CoV-2 and subsequently developed pancytopenia. Bone marrow routine revealed increased myeloblasts with multilineage dysplasia, and next-generation sequencing (NGS) found a TP53 mutation, leading to the diagnosis of secondary MDS. The pathogenesis of secondary MDS in POEMS syndrome is discussed from three aspects: cytotoxic therapy, genetic predisposition, and SARS-CoV-2 infection. This case underscores the importance of prolonged surveillance for secondary myeloid neoplasms (sMN) in POEMS patients and suggests that early genomic profiling and individualized treatment may improve outcomes.

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.

Chronic diarrhea, by definition, is the passage of loose/liquid stools, with increased frequency (more than three times/day), or an output of over 200 g/day, lasting for a duration of four or more weeks. The clinical approach to identify the cause of chronic diarrhea generally depends on the local socioeconomic status. In high-income countries, systemic causes such as irritable bowel syndrome (IBS), inflammatory bowel disease, malabsorption syndromes (lactose intolerance/coeliac disease) are primarily considered. In mid- to low-income countries, infective causes like chronic bacterial, mycobacterial, fungal infections, HIV, bowel cancer are considered before systemic causes/malabsorption syndromes. Amyloidosis, more accurately, reactive amyloidosis is one of the rarer causes of chronic/persistent diarrhea. Inflammatory colitis secondary to POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) as a cause for chronic diarrhea has been reported only in a handful of cases and is often missed. We present such a case of chronic diarrhea in a middle-aged man, who was eventually diagnosed to have POEMS syndrome.

POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes) is a multisystem disorder with a good long-term prognosis. In its dozens of clinical features, those with independent prognostic value are still not well characterized. We retrospectively included 362 patients with newly diagnosed POEMS syndrome at our institute from 2000 to 2015. On the basis of a randomized sample splitting, we first identified four baseline clinical variables, including age >50 years (hazards ratio (HR) 4.07, 95% confidence interval (CI) 1.41–11.76, P =0.009), pulmonary hypertension (HR 3.99, 95% CI 1.44–11.04, P =0.008), pleural effusion (HR 3.81, 95% CI 1.23–11.79, P =0.02) and estimated glomerular filtration rate <30 ml/min/1.73 m 2 (HR 8.25, 95% CI 2.18–31.25, P =0.002), associated with inferior overall survival in the derivation cohort, with the use of multivariate Cox regression model. These factors were incorporated together to develop a prognostic nomogram. Concordance index calculation (0.727, 95% CI 0.601–0.853, P =0.018) and calibration curve plotting demonstrated its significant predictive and discriminatory capacity in the validation cohort. This nomogram could be a useful and convenient tool in clinical practice to evaluate individualized prognosis in patients with newly diagnosed POEMS syndrome.

POEMS syndrome is a rare plasma cell dyscrasia characterized by polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes. This study reviewed the clinical characteristics and long-term outcome of 99 consecutive Chinese patients with newly diagnosed POEMS syndrome in a single institute. The median age of 99 patients was 45 years, and the ratio of men/women was 1.4. The median time from onset of symptoms to diagnosis was 18 months. The typical five features of peripheral neuropathy, organomegaly, endocrinopathy, M protein, and skin change remained to be essential for patients with POEMS syndrome in China. The unusual features like pulmonary hypertension (36%) and renal impairment (37%) were not uncommon in China. Eighty-three percent patients were alive after follow-up time of 25 months, and 10% patients had survived more than 60 months. Melphalan-based therapy (OR, 0.076; 95% CI, 0.02–0.285) and normal renal function (OR, 0.246; 95% CI, 0.076–0.802) were independent prognostic factors for the survival of patients with POEMS syndrome. In conclusion, POEMS syndrome in Chinese patients was a multi-systemic disease with clinical features similar to non-Chinese ones. Active therapy can effectively improve the prognosis of patients with POEMS syndrome.