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Original Research: POEMS SYNDROME |

Pulmonary Manifestations in Patients With POEMS Syndrome*: A Retrospective Review of 137 Patients FREE TO VIEW

Joanne Shirine Allam, MD; Cassie C. Kennedy, MD; Timothy R. Aksamit, MD, FCCP; Angela Dispenzieri, MD
Author and Funding Information

Affiliations: *From the Divisions of Pulmonary and Critical Care Medicine (Drs. Allam, Kennedy, and Aksamit), and Hematology (Dr. Dispenzieri), Mayo Clinic College of Medicine, Rochester, MN.,  These authors made equal contributions as first author.

Correspondence to: Angela Dispenzieri, MD, Division of Hematology, Mayo Clinic College of Medicine, 200 First St SW, Rochester, MN 55905; e-mail: dispenzieri.angela@mayo.edu



Chest. 2008;133(4):969-974. doi:10.1378/chest.07-1800
Text Size: A A A
Published online

Background: POEMS syndrome is a monoclonal plasma cell disorder characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. Rare reports of pulmonary manifestations of POEMS syndrome such as pulmonary hypertension exist; however, a comprehensive review of the pulmonary manifestations of POEMS syndrome is lacking.

Methods: Retrospective review of patients with a diagnosis of POEMS syndrome at our institution between 1975 and 2003. Demographics, signs and symptoms, test findings (ie, radiographs, pulmonary function tests, and echocardiography), and survival data were extracted. Kaplan-Meier survival analysis was performed. In addition, categoric variables were compared using the Pearson χ2 test or Fisher exact test, where appropriate.

Results: The study comprised 137 patients (66% male) with a mean age of 51.6 years. Respiratory symptoms were common within 2 years of diagnosis (28%). The median overall survival time was 147 months. Pulmonary manifestations of POEMS syndrome included pulmonary hypertension, restrictive lung disease, respiratory muscle weakness, and an isolated diminished diffusing capacity. Significant radiographic findings such as pleural effusions, diaphragm elevation, and increased cardiac silhouette were seen in 23% of patients. When separated by the presence or absence of respiratory muscle weakness, the median survival time was 87 vs 139 months, respectively (p < 0.05). The presence of cough was associated with reduced survival time.

Conclusions: Pulmonary manifestations of POEMS are common, and both symptomatic and asymptomatic respiratory involvements are frequent on presentation in patients with POEMS syndrome. Respiratory muscle weakness and cough portend a poorer prognosis. These results suggest the need for increased awareness of the association between POEMS syndrome and pulmonary disease to guide appropriate screening and supportive therapy.

Figures in this Article

The syndrome consisting of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin manifestations (POEMS) is a monoclonal plasma cell disorder that is seen across medical disciplines. Other commonly reported14 manifestations of POEMS syndrome include edema, papilledema, sclerotic bone lesions, lymphadenopathy, thromboses, and Castleman disease. Common features such as polyneuropathy and edema may be associated with respiratory muscle weakness or pleural effusions. In addition, case reports515 of pulmonary hypertension associated with POEMS syndrome further widen the spectrum of associated respiratory disease. A comprehensive review of the spectrum of pulmonary manifestations of POEMS syndrome and the impact that these manifestations have on the course of disease is lacking. We sought to define the full range of pulmonary manifestations of POEMS syndrome patients at our institution, including symptoms, notable examination findings, radiographic abnormalities, pulmonary function test (PFT) and echocardiographic findings, and survival characteristics.

Patient Identification

This investigation was reviewed and approved by the Mayo Clinic Institutional Review Board. A comprehensive computerized database search of medical records was performed to identify all patient encounters with a diagnosis of “POEMS syndrome” from 1987 to December 31, 2003. Some of the patients were identified under the POEMS synonyms of immunocytoma, osteosclerotic myeloma, or Takatsuki syndrome. Prior to 1987, cases were not systematically retrievable under a “POEMS syndrome” diagnosis; however, we had access to a preexisting comprehensive collection of POEMS syndrome cases consenting to research from 1975 to 1987.3 The diagnosis was made according to previously reported criteria.3 Cases were included if patients had consented to be included in research and were subsequently verified on review of the record to have POEMS syndrome. Ninety-nine percent of those patients whose cases were retrieved via computer consented to participation.

Clinical Data Collection

Three of the authors (C.C.K., J.S.A., and A.D.) performed data abstraction based on defined criteria. Charts were reviewed for demographic information including race, gender, age at diagnosis, smoking history (recorded as the number of years of smoking multiplied by the estimated number of packs smoked per day), and pulmonary or cardiovascular comorbidities. Signs and symptoms of cough, dyspnea, chest pain, orthopnea, and clubbing were abstracted as recorded at initial presentation. Chest radiograph reports were reviewed for effusions, atelectasis, infiltrates, nodules/masses, diaphragm elevation, or adenopathy. Chest radiograph data were abstracted as initially recorded by a Mayo Clinic radiologist. Data from PFTs were abstracted to identify inspiratory and expiratory pressures, maximum voluntary ventilation, body mass index, FEV1, FVC, total lung capacity, and diffusing capacity of the lung for carbon monoxide (Dlco). Predicted normal values were calculated using a standardized database by the pulmonary function laboratory at the time of testing.1617 If multiple PFTs were present in the record, subsequent tests were also recorded. Maximal inspiratory and expiratory pressures were defined as abnormal if both were < 70% predicted. FEV1, FVC, and Dlco were considered abnormal if they were < 80% predicted. Total lung capacity was considered abnormal if it was < 80% or > 120% predicted. Echocardiogram reports were reviewed from the time of diagnosis for right ventricular systolic pressure (RVSP), ejection fraction, estimated pulmonary artery pressure, right ventricular enlargement, and ECG (whether normal or arrhythmic, or showing the presence of a heart block). The ejection fraction was considered abnormal if it was < 55%. RVSP was calculated using the tricuspid regurgitant jet. Pulmonary hypertension was considered to be present if the RVSP was > 35 mm Hg in the presence of a normal ejection fraction. Outcomes of death and transplant were ascertained from the clinical record, and death was verified with the Social Security Death Index for US citizens. If the date of death had not been recorded in either, the patients’ survival was censored at the date of the last clinical encounter.

Statistical Analysis

Data analysis was performed using a statistical software package (JMP, version 6.0; SAS Institute Inc; Cary, NC). Survival was calculated from the time of diagnosis, and survival curves were constructed according to the Kaplan-Meier method. The groups were compared using log-rank tests; categoric variables were compared across groups using the Pearson χ2 test or Fisher exact test, where appropriate.

Demographics

One hundred thirty-seven patients were identified as having POEMS syndrome and consented to being included in a research study. Sixty-six percent of patients were men, and 89% were white (Table 1 ). The median age at diagnosis was 51 years (age range, 20 to 83 years). Forty-one percent of patients were smokers; the average number of pack-years smoked was 19.5. Six percent of patients had preexisting pulmonary morbidities, and 7% of patients had a preexisting diagnosis of congestive heart failure (Table 1).

Pulmonary Symptoms

Respiratory symptoms were found to be present in 38 patients (28%). Only one of those patients had a hemoglobin value of < 10 g/dL. The clinical symptoms reported were dyspnea (20%), chest pain (10%), cough (8%), and orthopnea (7%) [Table 2 ]. Clubbing was described in the physical examination in only 4% of individuals. Of the 38 patients with respiratory symptoms, 34 patients had at least one diagnostic test ordered (eg, PFT, echocardiogram, or chest roentgenogram), and 25 patients had at least one abnormal test result that might explain their symptoms. More patients with respiratory symptoms had further testing (eg, echocardiogram, PFT, or chest radiograph) than those who did not (Table 3 ).

PFT Findings

Thirty-two of the 137 patients in our sample underwent PFTs within the first 2 years after the POEMS diagnosis was made; 75% of those PFT findings were abnormal (Table 3). The most common pattern was a restrictive one, which was associated with a diminished Dlco, followed by the finding of an isolated drop in Dlco. Fifty-nine percent of patients tested for maximal respiratory pressures were found to have significant weakness, which was defined as a drop in both inspiratory and expiratory pressures below 70% predicted. These findings were significantly more frequent in the subgroup with respiratory symptoms (Table 3). Restrictive disease developed in an additional four patients 5, 5, 15, and 16 years later. An isolated drop in Dlco developed in one other patient 7 years after the diagnosis of POEMS syndrome.

Radiographic Features

Standard chest roentgenograms were performed in 114 patients within 2 years of the diagnosis. Sixty-one percent of those were read as abnormal; 28% had significant abnormalities (pleural effusions, 13 patients; cardiomegaly, 8 patients; enlarged pulmonary vessels, 7 patients; mediastinal adenopathy, 5 patients; diaphragm elevation, 12 patients). The remainder of the abnormalities (33%) consisted of atelectasis or bibasilar fibrosis. Effusions developed in five other patients at a later date.

CT scans of the chest or abdomen were performed in 50 patients. There was no report of any parenchymal lung abnormalities or signs of interstitial lung disease in any of the visualized lung portions.

Pulmonary Hypertension

Of the 137 patients included in this study, 25 had an echocardiogram performed within the first 2 years after receiving a diagnosis. Pulmonary hypertension was demonstrated on echocardiogram in 12 patients (or 48% of the patients who were tested) [Table 3]. Similar rates of pulmonary hypertension were observed in patients with and without respiratory symptoms, but the number of those studied in each group was small.

The patients with pulmonary hypertension did not differ from the rest of the group with regard to age, gender, and race. Symptoms of clubbing, cough, and chest pain, and the results of PFTs, including respiratory muscle testing, were similar in the two groups. Patients with pulmonary hypertension did report significantly more orthopnea and dyspnea than those without (p = 0.01 and 0.02, respectively). No patients had documented pulmonary thrombosis as the cause for the pulmonary hypertension. Very few patients had follow-up echocardiograms available, and, therefore, the progression of the pulmonary hypertension could not be followed. Three more patients had evidence of pulmonary hypertension on echocardiograms that had been performed 3, 11, and 22 years after the diagnosis. Pulmonary hypertension was diagnosed in seven additional patients, who had not undergone echocardiograms at the Mayo Clinic, later in the course of their disease based on studies performed at a different institution.

The mean RVSP was 47 mm Hg (range, 36 to 81 mm Hg). The median survival time for patients with pulmonary hypertension was 63.3 months. There was no significant correlation between the RVSP value and survival. As expected, patients with pulmonary hypertension were more likely to have an enlarged pulmonary vasculature seen on chest roentgenograms than those without pulmonary hypertension (p = 0.01). The presence of pulmonary hypertension did not correlate with any of the presenting symptoms of POEMS syndrome.

Survival Characteristics

The overall median survival time was 147 months (Fig 1 ). Sixty-two patients have died. The following characteristics were found to have no effect on survival: symptoms of dyspnea, chest pain, or orthopnea; the presence of clubbing; the presence of pulmonary hypertension seen on an echocardiogram; the number of POEMS syndrome criteria present on diagnosis; the presence of significant abnormality seen on a chest radiograph; and the presence of any of the PFT abnormalities. In contrast, the presence of cough and respiratory muscle weakness was associated with significantly shortened survival time (p = 0.0014 and 0.033, respectively) [Fig 2 ].

Our series is the first comprehensive review of both radiographic features and PFT findings in a large cohort of patients with POEMS syndrome. Respiratory symptoms were present at diagnosis in 28% of patients in our sample. Neuromuscular weakness, pulmonary hypertension, and restrictive lung disease were the most common objective pulmonary findings. Cough and respiratory muscle weakness were associated with a significantly reduced survival. Although patients with symptoms were more likely to undergo diagnostic testing, the presence or absence of respiratory symptoms did not affect the likelihood of obtaining an abnormal test result (Table 3), with the notable exception of neuromuscular weakness, suggesting that clinically relevant pulmonary manifestations are often overshadowed by other symptoms and signs of POEMS syndrome.

Pulmonary hypertension was seen in 8.5% of our patients within 2 years of their diagnosis; another 6% of patients had documented pulmonary hypertension over the ensuing years of follow-up. The exact pathophysiologic mechanisms of pulmonary hypertension in patients with POEMS syndrome are unclear, but they are likely to be mediated by cytokines.1823 Other investigators1112,14,1822 have reported instances of pulmonary hypertension associated with POEMS syndrome; it has been suggested9,24 that pulmonary hypertension develops in up to 25% of patients with POEMS syndrome. In our study, 48% of patients who were screened by echocardiogram within 2 years of presentation were found to have pulmonary hypertension. Although only a small number of patients were screened, this may suggest a high incidence of pulmonary hypertension in this population. Surprisingly, in our series, the presence of pulmonary hypertension did not significantly influence survival. It is unclear whether this lack of correlation with survival is a function of the reversibility of pulmonary hypertension after successful treatment of POEMS syndrome7,9,1112,14,1821 or merely a byproduct of patchy ascertainment that is inherent in a retrospective series. Alternatively, the sample size may have been too small to detect a survival difference.

Pulmonary function abnormalities were present in a significant proportion of patients who underwent PFTs. The most common abnormalities consisted of neuromuscular weakness, restrictive lung disease, and isolated low Dlco. Neuromuscular weakness, using a definition of maximal inspiratory and expiratory pressures of < 70% predicted, was seen in PFT results in at least 10 patients (7%). Five other patients in our group were found to have only one abnormal measurement of maximal respiratory pressure. Although maximal respiratory pressures are dependent on effort,25 and therefore are not among the most reproducible pulmonary studies, our data demonstrate that respiratory muscle weakness is an important feature of POEMS syndrome, based on its association with significant survival disadvantage. In general, patients with weakened respiratory muscles have a reduced capacity for airway protection with cough maneuvers and with severe enough involvement are at risk for respiratory failure. Phrenic nerve involvement has also been described.5,10,24,26

A restrictive lung process was evident from spirometry findings in 12 patients (8.5%), all of whom also had a low Dlco. Obesity was not a causative factor since no patients had a body mass index of > 35. Neuromuscular weakness was present in at least five of the patients with restrictive lung disease and was therefore a contributing cause, as were effusions, ascites, and organomegaly. Excluding findings of subtle basilar atelectasis on chest radiographs, there was no radiologic evidence of pulmonary parenchymal abnormalities to suggest an intrinsic interstitial problem.

The third most common abnormality found on PFT results was an isolated low Dlco when corrected for anemia, which was present in nine patients (6%). The isolated low Dlco is suggestive of a vascular abnormality; whereas, in patients with either neuromuscular weakness or a restrictive pattern, compression of the lung parenchyma may result in shunting with a resultant drop in the Dlco. Three of the four patients with isolated low Dlco who had an echocardiogram were found to have pulmonary hypertension. It is likely that more patients with undiagnosed pulmonary hypertension are present in this group. In our experience with patients who have POEMS syndrome and undergo high-dose chemotherapy with peripheral blood stem cell transplantation, the majority of patients with low Dlco before treatment will have Dlco values return to normal after transplantation.27 This is consistent with reports7,11,1314,20 that pulmonary hypertension appears to improve with the treatment of POEMS syndrome.

These findings of neuromuscular weakness and low Dlco in PFT results have been described previously in 15 of 16 patients with POEMS syndrome before undergoing peripheral blood stem cell transplantation, which is now an emerging treatment modality for POEMS syndrome.27 It is interesting to note that five of those patients, all of whom had received treatment with high-dose melphalan, were admitted to the ICU with respiratory failure during the course of the treatment.

Basilar fibrosis or atelectasis was present in half of the abnormal chest roentgenogram findings and may be a reflection of neuromuscular weakness affecting the diaphragm. Effusions were present in 13 patients and were associated with ascites in at least 3 patients. These forms of extravascular overload are probably related to the increase capillary permeability28 that is attributable to high vascular endothelial growth factor levels. Previous articles6,8,15,27 have also described the presence of pulmonary tumors in association with POEMS syndrome, which was not noted in our series.

Pulmonary manifestations are an important part of the POEMS syndrome. This study provides an overview of the abnormalities observed in a large series. Because this study is a retrospective review at a single institution, it has intrinsic limitations. First, not all patients had undergone extensive pulmonary screening, resulting in an underestimation of the prevalence, and possibly the prognostic value, of the pulmonary abnormalities seen in patients with POEMS syndrome. In addition, it is possible that cases were missed because they were listed under a diagnosis that was a synonym of POEMS syndrome. The effects of different therapies on outcomes after therapy also are beyond the scope of this review. These data should serve as a reminder for physicians caring for patients with POEMS syndrome that pulmonary involvement is common with or without symptoms and can easily be overshadowed by the severe physical limitations caused by severe peripheral neuropathy, which is the hallmark of this disease. All patients should be assessed for respiratory symptoms and pulmonary manifestations, with physical examinations, radiographs, PFTs, and echocardiograms performed in order to provide the best supportive pulmonary care while they are undergoing definitive therapy. Additional research is needed to specifically elucidate the impact of the presence of pulmonary manifestations on the outcomes of POEMS syndrome patients.

Abbreviations: Dlco = diffusing capacity of the lung for carbon monoxide; PFT = pulmonary function test; POEMS = polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin manifestations; RVSP = right ventricular systolic pressure

This work was supported in part by the Robert A. Kyle Hematological Malignancies Fund, National Cancer Institute grant CA062242, and the Mayo Foundation. The authors had complete control over data abstraction, database, statistical analysis, and manuscript production.

The authors have reported to the ACCP that no significant conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article.

Table Graphic Jump Location
Table 1. Clinical Characteristics*
* 

Values are given as No. (%), unless otherwise indicated.

Table Graphic Jump Location
Table 2. Clinical Signs at Presentation
Table Graphic Jump Location
Table 3. Characterization of Pulmonary Manifestations in POEMS Syndrome Within 2 Years of Diagnosis*
* 

Values are given as No. (%), unless otherwise indicated. The percentages for subcategories are expressed as the percentage of patients with the abnormality among those patients who had the test performed.

Figure Jump LinkFigure 2. Prognostic indicators for (left) cough and (right) neuromuscular weakness.Grahic Jump Location

The authors are grateful to Clint Doerr, MD, for his assistance with the database for this project.

Nakanishi, T, Sobue, I, Toyokura, Y, et al (1984) The Crow-Fukase syndrome: a study of 102 cases in Japan.Neurology34,712-720. [PubMed] [CrossRef]
 
Soubrier, MJ, Dubost, JJ, Sauvezie, BJ POEMS syndrome: a study of 25 cases and a review of the literature; French Study Group on POEMS Syndrome.Am J Med1994;97,543-553. [PubMed]
 
Dispenzieri, A, Kyle, RA, Lacy, MQ, et al POEMS syndrome: definitions and long-term outcome.Blood2003;101,2496-2506. [PubMed]
 
Zhu, WG, Wang, Y, Zeng, XJ, et al A clinical analysis of 32 cases of POEMS syndrome.Chung-Hua Nei Ko Tsa Chih2006;45,108-111. [PubMed]
 
Delalande, S, Stojkovic, T, Rose, C, et al Paralysie phrenique bilaterale, dysautonomie et cardiomyopathie restrictive dans un cas de syndrome POEMS.Rev Neurol (Paris)2002;158,737-740. [PubMed]
 
Gomez Rodriguez, F, Iriarte Garcia-Baquero, LM, Grilo Reina, A, et al Tumorlets pulmonares en el sindrome POEMS.An Med Interna1991;8,555-558. [PubMed]
 
Iwasaki, H, Ogawa, K, Yoshida, H, et al Crow-Fukase syndrome associated with pulmonary hypertension.Intern Med1993;32,556-560. [PubMed]
 
Kashima, M, Sasaki, M, Ito, T, et al A case of bronchioloalveolar cell carcinoma with bilateral diffuse interstitial infiltrative shadow during the treatment of Takatsuki’s disease.Nihon Kokyuki Gakkai Zasshi2001;39,399-404. [PubMed]
 
Lesprit, P, Godeau, B, Authier, FJ, et al Pulmonary hypertension in POEMS syndrome: a new feature mediated by cytokines.Am J Respir Crit Care Med1998;157,907-911. [PubMed]
 
Namekawa, M, Muramatsu, S, Hashimoto, R, et al A case of Crow-Fukase syndrome with respiratory failure due to bilateral diaphragmatic paralysis.Rinsho Shinkeigaku2002;42,635-638. [PubMed]
 
Paciocco, G, Bossone, E, Erba, H, et al Reversible pulmonary hypertension in POEMS syndrome: another etiology of triggered pulmonary vasculopathy?Can J Cardiol2000;16,1007-1012. [PubMed]
 
Okura, H, Gohma, I, Hatta, K, et al Thiamine deficiency and pulmonary hypertension in Crow-Fukase syndrome.Intern Med1995;34,674-675. [PubMed]
 
Jouve, P, Humbert, M, Chauveheid, MP, et al POEMS syndrome-related pulmonary hypertension is steroid responsive.Respir Med2007;101,353-355. [PubMed]
 
Ribadeau-Dumas, S, Tillie-Leblond, I, Rose, C, et al Pulmonary hypertension associated with POEMS syndrome.Eur Respir J1996;9,1760-1762. [PubMed]
 
Shinde, A, Matsumae, H, Maruyama, A, et al A patient with Crow-Fukase syndrome associated with pulmonary plasmacytoma.Rinsho Shinkeigaku2001;41,121-125. [PubMed]
 
Miller, A, Thornton, JC, Warshaw, R, et al Single breath diffusing capacity in a representative sample of the population of Michigan, a large industrial state.Am Rev Respir Dis1983;127,270-277. [PubMed]
 
Miller, A, Thornton, JC, Warshaw, R, et al Mean and instantaneous expiratory flows, FVC and FEV1: prediction equations from a probability sample of Michigan, a large industrial state.Bull Eur Physiopathol Respir1986;22,589-597. [PubMed]
 
Mukerjee, D, Kingdon, E, Vanderpump, M, et al Pathophysiological insights from a case of reversible pulmonary arterial hypertension.J R Soc Med2003;96,403-404. [PubMed]
 
Niimi, H, Arimura, K, Jonosono, M, et al VEGF is causative for pulmonary hypertension in a patient with Crow-Fukase (POEMS) syndrome.Intern Med2000;39,1101-1104. [PubMed]
 
Helmersen, DS, Ford, GT, Viner, SM, et al POEMS syndrome: a clue to understanding primary pulmonary hypertension? A review of current insights into the pathogenesis of primary pulmonary hypertension.Can J Cardiol2000;16,975-981. [PubMed]
 
Feinberg, L, Temple, D, de Marchena, E, et al Soluble immune mediators in POEMS syndrome with pulmonary hypertension: case report and review of the literature.Crit Rev Oncog1999;10,293-302. [PubMed]
 
Shiraki, T, Satoh, S, Sugiyama, H, et al Highly concentrated vascular endothelial growth factor in Crow-Fukase syndrome with high output heart failure: a case report.J Cardiol2005;45,75-80. [PubMed]
 
Dispenzieri, A, Gertz, MA Treatment options for POEMS syndrome.Expert Opin Pharmacother2005;6,945-953. [PubMed]
 
Mokhlesi, B, Jain, M Pulmonary manifestations of POEMS syndrome: case report and literature review.Chest1999;115,1740-1742. [PubMed]
 
American Thoracic Society, European Respiratory Society.. ATS/ERS statement on respiratory muscle testing.Am J Respir Crit Care Med2002;166,518-624. [PubMed]
 
de la Pena, A, Subtil, JC, Rodriguez-Rosado, R, et al Sindrome POEMS, a proposito de dos casos y revision de la literatura.An Med Interna1996;13,291-294. [PubMed]
 
Dispenzieri, A, Moreno-Aspitia, A, Suarez, GA, et al Peripheral blood stem cell transplantation in 16 patients with POEMS syndrome, and a review of the literature.Blood2004;104,3400-3407. [PubMed]
 
Loeb, JM, Hauger, PH, Carney, JD, et al Refractory ascites due to POEMS syndrome.Gastroenterology1989;96,247-249. [PubMed]
 

Figures

Figure Jump LinkFigure 2. Prognostic indicators for (left) cough and (right) neuromuscular weakness.Grahic Jump Location

Tables

Table Graphic Jump Location
Table 1. Clinical Characteristics*
* 

Values are given as No. (%), unless otherwise indicated.

Table Graphic Jump Location
Table 2. Clinical Signs at Presentation
Table Graphic Jump Location
Table 3. Characterization of Pulmonary Manifestations in POEMS Syndrome Within 2 Years of Diagnosis*
* 

Values are given as No. (%), unless otherwise indicated. The percentages for subcategories are expressed as the percentage of patients with the abnormality among those patients who had the test performed.

References

Nakanishi, T, Sobue, I, Toyokura, Y, et al (1984) The Crow-Fukase syndrome: a study of 102 cases in Japan.Neurology34,712-720. [PubMed] [CrossRef]
 
Soubrier, MJ, Dubost, JJ, Sauvezie, BJ POEMS syndrome: a study of 25 cases and a review of the literature; French Study Group on POEMS Syndrome.Am J Med1994;97,543-553. [PubMed]
 
Dispenzieri, A, Kyle, RA, Lacy, MQ, et al POEMS syndrome: definitions and long-term outcome.Blood2003;101,2496-2506. [PubMed]
 
Zhu, WG, Wang, Y, Zeng, XJ, et al A clinical analysis of 32 cases of POEMS syndrome.Chung-Hua Nei Ko Tsa Chih2006;45,108-111. [PubMed]
 
Delalande, S, Stojkovic, T, Rose, C, et al Paralysie phrenique bilaterale, dysautonomie et cardiomyopathie restrictive dans un cas de syndrome POEMS.Rev Neurol (Paris)2002;158,737-740. [PubMed]
 
Gomez Rodriguez, F, Iriarte Garcia-Baquero, LM, Grilo Reina, A, et al Tumorlets pulmonares en el sindrome POEMS.An Med Interna1991;8,555-558. [PubMed]
 
Iwasaki, H, Ogawa, K, Yoshida, H, et al Crow-Fukase syndrome associated with pulmonary hypertension.Intern Med1993;32,556-560. [PubMed]
 
Kashima, M, Sasaki, M, Ito, T, et al A case of bronchioloalveolar cell carcinoma with bilateral diffuse interstitial infiltrative shadow during the treatment of Takatsuki’s disease.Nihon Kokyuki Gakkai Zasshi2001;39,399-404. [PubMed]
 
Lesprit, P, Godeau, B, Authier, FJ, et al Pulmonary hypertension in POEMS syndrome: a new feature mediated by cytokines.Am J Respir Crit Care Med1998;157,907-911. [PubMed]
 
Namekawa, M, Muramatsu, S, Hashimoto, R, et al A case of Crow-Fukase syndrome with respiratory failure due to bilateral diaphragmatic paralysis.Rinsho Shinkeigaku2002;42,635-638. [PubMed]
 
Paciocco, G, Bossone, E, Erba, H, et al Reversible pulmonary hypertension in POEMS syndrome: another etiology of triggered pulmonary vasculopathy?Can J Cardiol2000;16,1007-1012. [PubMed]
 
Okura, H, Gohma, I, Hatta, K, et al Thiamine deficiency and pulmonary hypertension in Crow-Fukase syndrome.Intern Med1995;34,674-675. [PubMed]
 
Jouve, P, Humbert, M, Chauveheid, MP, et al POEMS syndrome-related pulmonary hypertension is steroid responsive.Respir Med2007;101,353-355. [PubMed]
 
Ribadeau-Dumas, S, Tillie-Leblond, I, Rose, C, et al Pulmonary hypertension associated with POEMS syndrome.Eur Respir J1996;9,1760-1762. [PubMed]
 
Shinde, A, Matsumae, H, Maruyama, A, et al A patient with Crow-Fukase syndrome associated with pulmonary plasmacytoma.Rinsho Shinkeigaku2001;41,121-125. [PubMed]
 
Miller, A, Thornton, JC, Warshaw, R, et al Single breath diffusing capacity in a representative sample of the population of Michigan, a large industrial state.Am Rev Respir Dis1983;127,270-277. [PubMed]
 
Miller, A, Thornton, JC, Warshaw, R, et al Mean and instantaneous expiratory flows, FVC and FEV1: prediction equations from a probability sample of Michigan, a large industrial state.Bull Eur Physiopathol Respir1986;22,589-597. [PubMed]
 
Mukerjee, D, Kingdon, E, Vanderpump, M, et al Pathophysiological insights from a case of reversible pulmonary arterial hypertension.J R Soc Med2003;96,403-404. [PubMed]
 
Niimi, H, Arimura, K, Jonosono, M, et al VEGF is causative for pulmonary hypertension in a patient with Crow-Fukase (POEMS) syndrome.Intern Med2000;39,1101-1104. [PubMed]
 
Helmersen, DS, Ford, GT, Viner, SM, et al POEMS syndrome: a clue to understanding primary pulmonary hypertension? A review of current insights into the pathogenesis of primary pulmonary hypertension.Can J Cardiol2000;16,975-981. [PubMed]
 
Feinberg, L, Temple, D, de Marchena, E, et al Soluble immune mediators in POEMS syndrome with pulmonary hypertension: case report and review of the literature.Crit Rev Oncog1999;10,293-302. [PubMed]
 
Shiraki, T, Satoh, S, Sugiyama, H, et al Highly concentrated vascular endothelial growth factor in Crow-Fukase syndrome with high output heart failure: a case report.J Cardiol2005;45,75-80. [PubMed]
 
Dispenzieri, A, Gertz, MA Treatment options for POEMS syndrome.Expert Opin Pharmacother2005;6,945-953. [PubMed]
 
Mokhlesi, B, Jain, M Pulmonary manifestations of POEMS syndrome: case report and literature review.Chest1999;115,1740-1742. [PubMed]
 
American Thoracic Society, European Respiratory Society.. ATS/ERS statement on respiratory muscle testing.Am J Respir Crit Care Med2002;166,518-624. [PubMed]
 
de la Pena, A, Subtil, JC, Rodriguez-Rosado, R, et al Sindrome POEMS, a proposito de dos casos y revision de la literatura.An Med Interna1996;13,291-294. [PubMed]
 
Dispenzieri, A, Moreno-Aspitia, A, Suarez, GA, et al Peripheral blood stem cell transplantation in 16 patients with POEMS syndrome, and a review of the literature.Blood2004;104,3400-3407. [PubMed]
 
Loeb, JM, Hauger, PH, Carney, JD, et al Refractory ascites due to POEMS syndrome.Gastroenterology1989;96,247-249. [PubMed]
 
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