Cardiology Research, ISSN 1923-2829 print, 1923-2837 online, Open Access
Article copyright, the authors; Journal compilation copyright, Cardiol Res and Elmer Press Inc
Journal website http://www.cardiologyres.org

Case Report

Volume 11, Number 2, April 2020, pages 118-128

Erdheim-Chester Disease With Extensive Pericardial Involvement: A Case Report and Systematic Review

Figures

Figure 1.
Figure 1. Large pericardial effusion.
Figure 2.
Figure 2. Right internal carotid artery occlusion.
Figure 3.
Figure 3. Reduction in pericardial effusion compared to Figure 1.
Figure 4.
Figure 4. Cardiac MRI with very scarce effusion. MRI: magnetic resonance imaging.
Figure 5.
Figure 5. Flow diagram according to the PRISMA statement. PRISMA: Preferred Reporting Items for Systematic Reviews and Meta-Analyses.

Tables

Table 1. Characteristics of Articles
 
CharacteristicN%
Type of article
  Case report2853.85%
  Review815.38%
  Letter47.69%
  Series of cases47.69%
  Original35.77%
  Other small publications59.62%
Year
  200111.92%
  200311.92%
  200411.92%
  200623.85%
  200711.92%
  201035.77%
  201135.77%
  201235.77%
  201323.85%
  201459.62%
  2015611.54%
  2016815.38%
  2017611.54%
  2018815.38%
  201923.85%

 

Table 2. Reports That Present Cases of Erdheim-Chester Disease With Pericardial Effusion
 
AuthorYSAFirst manifestationTSDCV manifestationsCPTreatmentOTO
Y: year; S: sex; A: age; TSD: time from start of symptoms to diagnosis; TO: time to outcome; CV: cardiovascular; UK: unknown; CP: cardiovascular procedure; ACE: angiotensin-converting enzyme; PUVA: photochemotherapy. aOnly one of the cases presented with pleural effusion.
Andre [12]2001M57ExophthalmosUKPericarditis and cardiac tamponadeMediastinoscopyPrednisone, diuretics ACEUKUK
Curgunlu [13]2003M43Swelling and pain in his right knee10 monthsModerate pericardial effusion-Prednisolone, palliative therapy for painStable6 months
Johnson [14]2004M34Migraines, headache3 yearsPericardial effusionPericardial drainageUKUK
Palotas [15]2006M57Already diagnosed at the presentationUKPericardial effusion with left ventricle enlarged-UKUK
Saboerali [16]2006M45Chest pain and a nonproductive cough2.5 yearsPericardial thickening and effusions without inflow disruption-UKUKUK
Palotas [17]2007M57Pericardial effusion and exophthalmosUKPericardial effusionSternotomyUKUKUK
Alharthi [10]2010F53Bilateral ankle swelling and shortness of breath1 yearLarge pericardial effusion and pan cardiac involvementPericardiocentesisCladribineStable12 months
Kim [18]2010M68Multiple yellowish plaques and a pinkish nodule on his face and scalp1.5 yearsModerate pericardial effusion-Interferon-αStable16 months
Lutz [19]2011F65Recurrent pericardial effusion3 monthsChronic pericardial effusion, a mass attached to the pericardium, and a thickened aortaPericardiocentesisGlucocorticoids and interferon-αDeadUK
Vega [20]2011M76Progressive congestive heart failure8 monthsPericarditis and cardiac tamponadePleuro-pericardial windowSteroids and methotrexateUKUK
Egan [21]2011F53Progressive dyspnea on exertion and lower extremity edema1 yearMild chronic fibrosing pericarditis and pericardial effusionPleuro-pericardial windowUKStable4 months
Ota [22]2012F77Dyspnea on exertion1 yearPericardial effusionPericardiocentesisMethylprednisolone and then interferon-αUKUK
Takeuchi [23]2012M56Left exophtalmusUKPericardial effusion-UKUK
Dagna [24]2012M56Recurrent pericardial effusion, recent cardiac tamponade and signs of heart failure2 yearsRecurrent pericardial effusion, recent cardiac tamponade and signs of heart failurePericardial windowInfliximab and methotrexateStable30 months
M60Already diagnosedUKPericardial effusion-Infliximab and methotrexateStable12 months
Dogan [25]2013M62Recurrent pericardial effusion5 monthsLarge amount of pericardial effusion, and a mass which surrounds the right atriumPericardiocentesis + pericardiocentesis + pleuro-pericardial windowMethylprednisolone and cyclophosphamideStableUK
Alexiou [26]2015F56Mesenteric panniculitis8 yearsRecurrent pericardial effusion-UKDead
De Miguel [27]2015F61Symptoms of congestive heart failure1 weekIrregular right atrial mass and moderate to severe pericardial effusion-CorticosteroidsStable12 months
Mishra [28]2015M57Worsening dyspnea, non-productive cough, reduced appetite and weight loss6 monthsRecurrent large pericardial effusion, pericardial thickening and right ventricular collapsePericardiocentesisGlucocorticoids and interferon-αDead
Abdellateef [29]2016F19Unilateral left exophthalmos6 yearsCardiac tamponadePericardiocentesisPrednisolone and azathioprineStable1 month
Azadeh [30]2016M61Pleural and cardiac effusion4 monthsCardiac muscle hypertrophy, pericardial effusion and adhesions-CladribineImproved6 months
Mathis [31]2016F59Chronic headaches and asthenia5 yearsRight peri-atrial infiltration, thickening of the wall of the aortic arch and pericardial effusion-Interferon-α and clopidogrelStable4 years
Nakhleh [32]2016M68Weight loss and general weakness6 monthsCardiomegaly and small pericardial effusionPericardiocentesisInterferon-αDead
Peric [33]2016M23Severe exophthalmos and vision loss of the left eye6 monthsPericardial thickening with effusion and right atrial collapse-CladribineImproved12 years
Kyriakopoulou [34]2016F77Bilateral exophthalmos, orbital xanthogranuloma and fatigue4 monthsAcute cardiac tamponadePericardiocentesisCorticosteroids + subcutaneous interferon-α-2b then changed to peginterferon-α-2aStable12 months
Bentel [35]2017M54Left adrenal massUKPericardial effusionPericardial windowNo specific treatmentStable8 years
Chasseur [36]2017F74Giant xanthelasma and progressive bilateral exophthalmos causing partial loss of vision3 monthsMassive cardiac tamponade with right ventricular compressionPericardiocentesisCorticosteroids and then interferon-αImproved4 months
Franco-Palacios [37]2017M20Subacute non-productive cough, new onset orthopnea and exertional dyspnea at rest1 monthPericardial effusionPericardial windowSteroids and interferon-αImproved13 months
Zhu [38]2017F51Shortness of breath, chest pain, recurrent edema of legs and night sweatsUKPericardial effusionPericardiectomyInterferon-αStable7 months
Lierscha [39]2017F75Ulcerating sub mammary plaques, yellowish rash on central trunk and keratotic plaques on her lower legs13 yearsSmall pericardial effusions-Interferon-α and local PUVA then methotrexateStable12 months
Monmany [40]2018F69Exophthalmos and facial pain6 monthsHeterogeneous mass and pericardial effusionPericardiocentesis + pericardiectomyPegylated interferon-α-2aImproved3 months
Tomelleri [41]2018M62Recurrent pericardial effusions1 yearRecurrent pericardial effusionsPleuro-pericardial windowAnakinraImproved2 months
Costaa [11]2018F64Already diagnosed at the presentationUKAtrial pseudotumor, pericardial thickening and effusion-UKUK

 

Table 3. Interventions Performed in the Cases of Erdheim-Chester Disease With Pericardial Effusion
 
ProcedureN%
None1339.39%
Pericardiocentesis824.24%
Pericardial window39.09%
Pleuro-pericardial window39.09%
Mediastinoscopy13.03%
Pericardectomy13.03%
Pericardial drainage13.03%
Sternotomy13.03%
Pericardiocentesis + pericardectomy13.03%
Pericardiocentesis + pericardiocentesis + pleuro-pericardial window13.03%