Chronic constrictive pericarditis: a rare cardiac involvement in primary Sjögren’s syndrome

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This case report describes a rare association between primary Sjögren’s syndrome and constrictive pericarditis, emphasizing the importance of clinical suspicion and multimodal imaging for diagnosis. Over four years, the patient’s condition progressed from pericardial thickening to constriction, with partial symptom relief after pericardiectomy, highlighting the potential for connective tissue disorders to cause this rare cardiac complication.

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BackgroundConstrictive pericarditis represents a chronic condition and systemic inflammatory diseases are a known, yet uncommon, cause. Pericardial involvement is seldom reported in primary Sjögren’s syndrome, usually occurring in association with pericardial effusion or pericarditis.We report a case of constrictive pericarditis with an insidious course and unusual evolution associated with primary Sjögren’s syndrome. Due to the challenging nature of the diagnosis, clinical suspicion and multimodality imaging are essential for early identification and prompt initiation of treatment. Long-term outcomes remain uncertain.To the best of our knowledge, no other cases linking this autoimmune disease to constrictive pericarditis have been reported.Case presentationWe present the case of a 48-year-old male patient with moderate alcohol habits and a history of two prior hospitalizations. On the first, the patient was diagnosed with primary Sjögren’s syndrome after presenting with pleural effusion and ascites, and empirical corticosteroid regiment was initiated. On the second, two-years later, he was readmitted with complaints of dyspnea and abdominal distension. Thoracic computed tomography revealed a localized pericardial thickening and a thin pericardial effusion, both of which were attributed to his rheumatic disease. A liver biopsy showed hepatic peliosis, which was considered to be a consequence of glucocorticoid therapy. Diuretic therapy was adjusted to symptom-relief, and a tapering corticosteroid regimen was adopted.Four years after the initial diagnosis, the patient was admitted again with recurrent dyspnea, orthopnea and ascites. At this time, constrictive pericarditis was diagnosed and a partial pericardiectomy was performed.Although not completely asymptomatic, the patient reported clinical improvement since the surgery, but still with a need for baseline diuretic therapy.ConclusionAlbeit uncommon, connective tissue disorders, such as primary Sjögren’s syndrome, should be considered as a potential cause of constrictive pericarditis, especially in young patients with no other classical risk factors for constriction.In this case, after excluding possible infectious, neoplastic and autoimmune conditions, a primary Sjögren´s syndrome in association with constrictive pericarditis was assumed. This case presents an interesting and challenging clinical scenario, highlighting the importance of clinical awareness and the use of multimodal cardiac imaging for early recognition and treatment.

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  • Research Article
  • Cite Count Icon 22
  • 10.7326/0003-4819-46-2-403
CONSTRICTIVE PERICARDITIS AS SEQUEL TO HEMOPERICARDIUM: REPORT OF A CASE FOLLOWING ANTICOAGULANT THERAPY
  • Feb 1, 1957
  • Annals of Internal Medicine
  • Maurice H Laszlo

Case Reports1 February 1957CONSTRICTIVE PERICARDITIS AS SEQUEL TO HEMOPERICARDIUM: REPORT OF A CASE FOLLOWING ANTICOAGULANT THERAPYMAURICE H. LASZLO, M.D.MAURICE H. LASZLO, M.D.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-46-2-403 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptThe various causes of constrictive pericarditis have as yet not been thoroughly elucidated. Indeed, there is a wide divergence in various studies with respect to the incidence of the several known etiologic factors.1-4Undoubtedly tuberculous pericarditis is a fairly common precursor of constrictive pericarditis; pyogenic agents, polyserositis and previous nonspecific acute pericarditis are also at times implicated as causative factors. In addition, there still remains a large group of cases of indeterminate cause where the pathology of the pericardium, as found at surgery or autopsy, throws no light on the etiology. For example, no etiologic agent could be demonstrated in...Bibliography1. PaulCastlemanWhite OBPD: Chronic constrictive pericarditis: a study of 53 cases, Am. J. M. Sc. 216: 361, 1948. CrossrefMedlineGoogle Scholar2. AndrewsPickeringSellors GWGWTH: The aetiology of constrictive pericarditis with special reference to tuberculous pericarditis, together with a note on polyserositis, Quart. J. Med. 17: 291, 1948. MedlineGoogle Scholar3. ChamblissJaruszewskiBrofmanMartinFeil JREJBLJFH: Chronic cardiac compression (chronic constrictive pericarditis), Circulation 4: 816, 1951. CrossrefMedlineGoogle Scholar4. Moschcowitz E: Pathogenesis of constrictive pericardium, J. A. M. A. 153: 194, 1953. CrossrefMedlineGoogle Scholar5. Nichol ES: The risk of hemorrhage in anticoagulant therapy, Ann. West. 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OverholtBurwellWoodburyWalker RHCSJWJH: Constrictive pericarditis and constrictive pleuritis treated by pericardiectomy and pulmonary decortication, J. Thoracic Surg. 23: 1, 1952. CrossrefGoogle Scholar21. ElkinCampbell DCRE: Cardiac tamponade: treatment by aspiration, Ann. Surg. 133: 623, 1951. CrossrefMedlineGoogle Scholar22. MaguireGage CHM: A discussion of cardiac tamponade: treatment by aspiration, by Elkin, D. C., and Campbell, R. E.21 Google Scholar23. EhrenhaftTaber JLRE: Hemopericardium and constrictive pericarditis, J. Thoracic Surg. 24: 355, 1952. CrossrefGoogle Scholar This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAffiliations: New York, N. Y.*Received for publication February 24, 1956.From the Medical Service of the United States Public Health Service Hospital, Memphis, Tennessee. The opinions expressed are those of the author and do not necessarily reflect those of the U. S. Public Health Service.Requests for reprints should be addressed to Maurice H. Laszlo, M. D., Montefiore Hospital, New York 67, N. Y. PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited byLeserbreif zum Artikel: Kardiogener Schock 4 Stunden nach akut-PTCA bei HinterwandinfarktEarly constrictive pericarditis and anemia after Dressler's syndrome and inferior wall myocardial infarction.Constrictive pericarditis after myocardial infarction.Constrictive pericarditis after myocardial infarction. Sequela of anticoagulant-induced hemopericardiumCardiac Tamponade Complicating the Postpericardiotomy SyndromeTraumatic Hemopericardium and Chronic Constrictive PericarditisConstrictive pericarditis associated with hemangioma of the pericardiumAnticoagulant-Induced Hemopericardium with TamponadeThe evolution and current concepts of the surgical treatment of constrictive pericarditisA case of traumatic pericarditis with chronic tamponade and constrictionManagement of pericarditis secondary to myocardial infarctionSmall bowel obstruction due to hemorrhage secondary to anticoagulant therapyPostpericardiotomy syndrome following penetrating stab wounds of the chest: Comparison with the postcommissurotomy syndromeHemorrhagic pericardial effusion following myocardial infarction associated with a ventricular aneurysmPostpericardiotomy Syndrome Following Surgery for Nonrheumatic Heart Disease 1 February 1957Volume 46, Issue 2Page: 403-413KeywordsAutopsy pathologyElectrocardiographyEtiologyHemorrhageLesionsMedical servicesPericardiumResearch laboratoriesSurgeryTuberculosis ePublished: 1 December 2008 Issue Published: 1 February 1957 PDF downloadLoading ...

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  • Research Article
  • Cite Count Icon 14
  • 10.4061/2011/469602
Chronic Constrictive Pericarditis in Association with End-Stage Renal Disease
  • Jan 1, 2011
  • International Journal of Nephrology
  • Roman L Kleynberg + 3 more

Pericardial involvement in end-stage renal disease (ESRD) is manifested most commonly as acute uremic or dialysis pericarditis and infrequently as chronic constrictive pericarditis (CCP). We report a 27-year-old patient with a history of uncontrolled hypertension, end-stage-renal disease on hemodialysis, who presented with recurrent ascites, dyspnea, and hypotension. After diagnosis with CCP, a partial pericardiectomy was performed; however, the patient did not improve and a salvage total pericardiectomy soon followed. He continued to decompensate and expired following a terminal extubation. No definitive cause of constrictive pericarditis was found. Nonetheless, we surmise it may have developed secondary to his end-stage renal disease. A literature review revealed end-stage kidney disease as a relatively uncommon cause of CCP; only a few other such associations have thus far been reported.

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  • 10.1016/j.athoracsur.2005.09.026
Invited commentary
  • Jan 20, 2006
  • The Annals of Thoracic Surgery
  • Joseph W Rubin

Invited commentary

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  • Cite Count Icon 3
  • 10.2169/internalmedicine.1381-22
Multimodal Imaging of Constrictive Pericarditis Induced by Long-term Pergolide Treatment for Parkinson's Disease
  • Dec 15, 2023
  • Internal Medicine
  • Hiroyuki Aoyagi + 14 more

We herein report the first case of constrictive pericarditis (CP) induced by long-term pergolide treatment for Parkinson's disease that was assessed using multimodal imaging in a 72-year-old patient with leg edema and dyspnea. The patient was correctly diagnosed with CP using multimodal imaging and successfully treated with pericardiectomy. The treatment history of Parkinson's disease and pathological findings of the removed pericardium suggested that long-term pergolide was the cause of CP. Properly recognizing pergolide as the cause of CP and accurately diagnosing CP using multimodal imaging may contribute to the early detection and treatment of pergolide-induced CP.

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  • 10.1016/j.chest.2022.08.1325
RARE CASE OF CONSTRICTIVE PERICARDITIS SECONDARY TO METASTATIC SQUAMOUS CELL CARCINOMA
  • Oct 1, 2022
  • Chest
  • Christopher James + 4 more

RARE CASE OF CONSTRICTIVE PERICARDITIS SECONDARY TO METASTATIC SQUAMOUS CELL CARCINOMA

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  • Cite Count Icon 23
  • 10.1016/0002-9149(61)90423-4
Chronic constrictive pericarditis treated with pericardiectomy
  • Jan 1, 1961
  • The American Journal of Cardiology
  • Donald B Effler

Chronic constrictive pericarditis treated with pericardiectomy

  • Research Article
  • Cite Count Icon 137
  • 10.1016/j.athoracsur.2012.03.079
Contemporary Etiologies, Risk Factors, and Outcomes After Pericardiectomy
  • May 21, 2012
  • The Annals of Thoracic Surgery
  • Timothy J George + 5 more

Contemporary Etiologies, Risk Factors, and Outcomes After Pericardiectomy

  • Research Article
  • 10.18203/2349-3291.ijcp20164625
An unusual cause of refractory ascites with cirrhosis
  • Dec 21, 2016
  • International Journal of Contemporary Pediatrics
  • Nikhil Mehra + 1 more

Reports of chronic constrictive pericarditis presenting with hepatomegaly, cirrhosis and ascites but no cardiac symptoms are rare. A 10 year old child presented with refractory ascites and firm hepatomegaly of 2 years duration which partially responded to diuretics. Doppler study showed dilated hepatic veins and inferior vena cava and ruled out hepatic venous outflow tract obstruction. Chest X ray showed pericardial calcification and 2D Echo was suggestive of chronic constrictive pericarditis. A diagnosis of chronic calcified constrictive pericarditis with cirrhosis and refractory ascites was made. Based on previous series which showed that tuberculosis is the most common cause of constrictive pericarditis, tuberculosis as the cause of constrictive pericarditis was kept. Anti tuberculous treatment as per Revised National Tuberculosis Control Program guidelines was started along with oral steroids and diuretics. Child showed response to the treatment and was later referred to cardiac surgery department for further management (pericardectomy).

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  • Cite Count Icon 6
  • 10.1378/chest.59.5.476
Left Ventricular Function and Myocardial Contractility in Chronic Constrictive Pericarditis
  • May 1, 1971
  • Chest
  • Ralph Shabetai

Left Ventricular Function and Myocardial Contractility in Chronic Constrictive Pericarditis

  • Research Article
  • 10.9790/0853-2312054349
Clinical And Echocardiography Outcomes Following Pericardiectomy In Chronic Constrictive Pericarditis
  • Dec 1, 2024
  • IOSR Journal of Dental and Medical Sciences
  • Nootan Hadiya + 4 more

Introduction: Leading cause of chronic constrictive pericarditis differs according to geographic location, tuberculosis remains the most common cause of constrictive pericarditis in Africa and Asia where as in western country it remains a rare entity and idiopathic causes remains the most common etiology. Here our aim was to study the clinical and echocardiography outcomes post pericardiectomy & compare it with other previous clinical studies. Methods: This is a retrospective study of 12 months with mean follow up period of 2.40 ± 2.01 years. PHILIPS EPIQ7C machine was used for echocardiographic analysis. Mitral and tricuspid inflow velocities were detected using PW doppler in apical 4-chamber view with sample volume of 2-4 mm. Results: All the patients in the study population presented with dyspnea 19(100%). Annulus reversus was observed in all our patients (19, 100%), which resolved completely postoperatively. Mean duration of hospital stay was 26.63 ± 13.09 days with mean ICU stay of 5.89 ± 2.4 days. Discussion: Different from international studies, we observed pericarditis in younger age group with mean age of 26.58 ± 11.9 years which could be accountable to Tuberculosis in young generation in India, as also observed by other indian studies. In our study, there was significant respiro-phasic variation in the mitral and tricuspid E velocity in all patients. Conclusions: Studies on detailed echocardiographic evaluation in terms of various parameters like mitral and tricuspid E velocities and tissue doppler imaging are sparce. This study adds to the important role of echocardiography in assessment of chronic constrictive pericarditis.

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  • 10.1371/journal.pone.0068718
Left Atrial Systolic and Diastolic Dysfunction in Patients with Chronic Constrictive Pericarditis: A Study Using Speckle Tracking and Conventional Echocardiography
  • Jun 18, 2013
  • PLoS ONE
  • Shuang Liu + 6 more

BackgroundLeft atrial (LA) function plays an important role in the maintenance of cardiac output, however, in patients with constrictive pericarditis (CP), whether pericardial restriction and adhesion can lead to LA dysfunction, and the characteristics of LA function remain unclear. The aim of the study is to compare the left atrial (LA) function of patients with CP to that of healthy study participants using speckle tracking echocardiography (STE) and conventional echocardiography.Methods and ResultsThirty patients with CP and 30 healthy volunteers (controls) were enrolled in the study. The underlying cause of CP was viral pericarditis in 24 (80%) patients and unknown in 6 (20%) patients. The LA maximum volume (Vmax), LA minimal volume (Vmin), and LA volume before atrial contraction (Vpre-a) were measured using biplane modified Simpson’s method. The LA expansion index (LA reservoir function) was determined as follows: ([LAVmax - LAVmin]/LAVmin) ×100. The passive emptying index (LA conduit function) was calculated as follows: ([LAVmax - LAVpre-a]/LAVmax) ×100, and the active emptying index (booster pump function) was calculated as follows: ([LAVpre-a - LAVmin]/LAVpre-a) ×100. All the patients underwent two-dimensional STE. The LA global systolic strain (S), systolic strain rate (SrS), early diastolic strain rate (SrE) and late diastolic strain rate (SrA) were measured. The LA expansion index, passive emptying index, the active emptying index and the LA global S, SrS, SrE, SrA were found to be significantly lower in patients with CP than in the control participants (P <0.001). LA function was correlated with the early diastolic velocity of the lateral mitral annulus (P <0.05).ConclusionsAlthough left ventricular systolic function was preserved in patients with CP, the LA reservoir, conduit, and booster functions were impaired. Pericardial restriction and impairment of the LA myocardium may play an important role in the reduction of LA function in patients with CP.

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  • Cite Count Icon 1
  • 10.1016/j.clinpr.2021.100071
An unusual case of purulent constrictive pericarditis with Hyper-IgG4-related histological findings on biopsy
  • Apr 1, 2021
  • Clinical Infection in Practice
  • Eduardo Corona-Rodarte + 5 more

An unusual case of purulent constrictive pericarditis with Hyper-IgG4-related histological findings on biopsy

  • Supplementary Content
  • Cite Count Icon 43
  • 10.1136/hrt.2003.024828
Pericardial constriction: uncommon patterns
  • Feb 13, 2004
  • Heart
  • J Sagristà-Sauleda

Constrictive pericarditis can be defined as a syndrome (or syndromes) resulting from compression of the heart caused by rigid, thickened, and frequently fused pericardial membranes. This syndrome was known about...

  • Research Article
  • Cite Count Icon 2
  • 10.1177/23247096241248969
A Rare Case of Immunoglobulin G4-Related Constrictive Pericarditis Diagnosed Via Multimodality Imaging
  • Jan 1, 2024
  • Journal of Investigative Medicine High Impact Case Reports
  • Sacide S Ozgur + 8 more

Constrictive pericarditis (CP) presents as a pathophysiological state where the pericardium becomes inelastic due to fibrotic changes, most commonly secondary to a protracted inflammatory process. The disease is characterized by compromised diastolic cardiac function due to loss of pericardial compliance. Immunoglobulin G4 (IgG4)-related disease, an entity marked by the insidious proliferation of IgG4-positive plasma cells and subsequent fibrosis within various organs, is an infrequent but recognized cause of CP. A case of a 55-year-old male patient with clinical manifestations of dyspnea and edema in the lower extremities elucidates the diagnostic complexity inherent to CP. Echocardiography revealed a constellation of signs, including annulus reversus, septal bounce, and a congested inferior vena cava; cardiac magnetic resonance imaging (MRI) demonstrated diffuse pericardial thickening with delayed gadolinium enhancement, suggestive of a long-term inflammatory state; and right heart catheterization confirmed the hemodynamic hallmark of CP—equalization of diastolic pressures across the cardiac chambers. The serological analysis elicited elevated serum levels of IgG4 and IgE, pointing to the differential diagnosis of IgG4-related disease. Given the nonspecific clinical presentation of IgG4-related CP, a heightened index of suspicion combined with a systematic approach to imaging and serological evaluation is paramount.

  • Abstract
  • 10.1016/j.chest.2016.08.666
Recurrent Idiopathic Pleural Effusions: Do Consider Constricitve Pericarditis as a Cause
  • Oct 1, 2016
  • Chest
  • Susheela Hadigal + 2 more

Recurrent Idiopathic Pleural Effusions: Do Consider Constricitve Pericarditis as a Cause

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