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Vol. 18. Núm. 6.
Páginas 374-376 (Junio - Julio 2022)
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Vol. 18. Núm. 6.
Páginas 374-376 (Junio - Julio 2022)
Case report
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Heart Transplantation in Systemic Sclerosis: A Therapeutic Option. Presentation of a Case and Literature Review
Trasplante cardíaco en esclerosis sistémica: una opción terapéutica. Presentación de un caso y revisión de la bibliografía
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Andrea de Diego-Solaa,
Autor para correspondencia
adediegosola@hotmail.com

Corresponding author.
, César A. Egües Dubuca, Cristina Goena Vivesb, Juan José Intxausti Irazabalc, Olga Maíz Alonsoa, Manuel Cobo Belaustegid
a Rheumatology Department, Donostia University Hospital, Paseo Doctor Begiristain 107-115, 20006 Donostia, Gipuzkoa, Spain
b Cardiology Service, Mendaro Hospital, C/Mendarozabal s/n, 20850 Mendaro, Gipuzkoa, Spain
c Rheumatology Service, Mendaro Hospital, C/Mendarozabal s/n, 20850 Mendaro, Gipuzkoa, Spain
d Cardiology Service, Marqués de Valdecilla University Hospital, Av. De Valdecilla s/n, 39008 Santander, Cantabria, Spain
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Table 1. Published cases of heart transplantation in patients with Systemic Sclerosis: demographic, laboratory and clinical characteristics, used immunosuppressive therapies and progression.
Abstract

Cardiac involvement in systemic sclerosis (SSc) is rare but leads to poor short-term prognosis. Evidence regarding heart transplantation (HT) is scarce and is based on experience with isolated cases. We present this case with the aim of analysing the characteristics of a patient with SS who has undergone a successful transplant.

Keywords:
Systemic sclerosis
Cardiac transplant
Scleroderma
Heart disease
Resumen

El compromiso cardíaco en la esclerosis sistémica (ES) es raro, pero conlleva un mal pronóstico a corto plazo. La evidencia relativa al trasplante cardíaco (TC) es escasa y se basa en la experiencia con los casos aislados. Presentamos este caso con el objetivo de analizar las características de un paciente con esclerosis sistémica sometido a un trasplante exitoso.

Palabras clave:
Esclerosis sistémica
Trasplante cardíaco
Escleroderma
Cardiopatía
Texto completo
Introduction

SSc is a systemic autoimmune disease of heterogeneous pathogenesis in which vascular, cutaneous and internal organ fibrosis are prominent.1 Primary symptomatic cardiac involvement occurs in 15–35% of patients and is estimated to cause up to 30% of deaths.1,2

Case

We present the case of a 58 year-old male diagnosed with anti-SCL70 positive diffuse cutaneous SSc (dcSSc). At diagnosis he presented with myositis, arthralgias and scleroderma (Rodman index 23/51), as well as interstitial disease of subpleural predominance in both lung bases. No oesophageal or renal involvement was observed. After 11 months of evolution it presented elevation of myocardial damage markers (CK, CK-Mb, Troponin and NT-proBNP). Echocardiogram and cardiac resonance showed pericardial effusion, dilation and biventricular systolic dysfunction. The initial affectation was mild (LVEF 51% and RVEF 40% without pathological gadolinium enhancement or myocardial oedema) but despite the support treatment with neurohumoral inhibitors (ACEI, beta-blockers and aldosterone antagonists) and immunosuppressive treatment (corticosteroids, mycophenolate mofetil, cyclophosphamide pulses and Rituximab) it progressed unfavourably to severe affectation (LVEF 26% and RVEF 24% with patchy subendocardial gadolinium enhancement). Interstitial lung involvement was controlled with immunosuppressors, whereas residual myocardial involvement triggered episodes of cardiogenic shock and malignant ventricular arrhythmias. Five months after the diagnosis of cardiomyopathy, HT was chosen as the therapeutic option. Thirty-six months after HT the patient is in stage I of the NYHA, with good biventricular function of the graft, respiratory stable and has presented regression of the skin affectation (Rodman's index of 17/51 at 16 months post-transplant) with the usual immunosuppressive treatment.

Discussion

Myocardial involvement is associated with ventricular systolic dysfunction in 10% of patients3 with a two-year mortality of 60%.4 Although it can occur in both subtypes it is more severe in patients with cdSSc, often evolving in parallel with skin progression.5 It is also more frequently associated with certain antibodies: anti-SCL70, anti-U3 RNP, anti-Ku and anti-Th/To. Due to the importance in terms of mortality of pulmonary involvement, pulmonary and cardio-pulmonary transplantation are accepted options in the face of therapeutic failure. In the case of cardiac affectation there are fewer reported cases of CT, being an option frequently limited by the concomitant affectation of other organs. After a systematic search, another 15 cases of CT were identified in patients with cardiac involvement by ES (Table 1). Eight presented a diagnosis of EScd (53.3%), 5 EScl (33.3%), 1 (6.6%) overlap syndrome with rheumatoid arthritis and in another case the ES subtype was not included. No cardiac recurrence of ES was reported in any case. Only one other published case presented associated pulmonary involvement,6 although its evolution is not assessed since it died 6 weeks after the CT scan due to infectious complications. It is worth noting the skin improvement after immunosuppression for CT, also observed in other published cases.

Table 1.

Published cases of heart transplantation in patients with Systemic Sclerosis: demographic, laboratory and clinical characteristics, used immunosuppressive therapies and progression.

Author  Sex  Age at Dx  Sub-type  ANA  ILD  Other organs involved  Enhancement on IRM  FEVI pre-HT  Interval Dx-HT  ISx. pre-HT  Follow-up  Dead 
Turcotte (2020)4  42  dcSSc  NS  No  uDT; RP  Yes  25%  6 y and 6 mo  NR  4 y  No 
Faust (2019)6  32  dcSSc  NI  No  FR, uDT  NI  40%  12 y  MMF  2 wk  No 
Faust (2019)6  30  dcSSc  NI  Yes  FR, uDT  NI  53%  14 y and 2 mo  GC, CFM, CSA  6 semanas  Yes 
Martens (2012)7  31  NI  NI  No  No  Yes  42%  3 y  MTX, MMF  1 y  No 
Bennasar (2015)8  16  dcSSc  NS  No  RP; myositis; xerophthalmia; xerostomía  Yes  25%  3 y  AZA, GC  15 d  Yes 
Johnson (2013)9  14  dcSSc  NS  No  Arthritis  NI  42%  4 y and 7 mo  MTX; CFM  22 mo  Yes 
Ikic (2014)  15  dcSSc  Scl-70  No  uDT; artritis; miositis  Yes  35%  NI  MMF, GC  0.5 y  No 
Ikic (2014)10  12  dcSSc  NS  No  uDT; iDT; arthritis; myositis  NI  25%  NI  D-PEN  3 y  No 
Ikic (2014)10  35  lcSSc  NS  No  TDs; arthritis; myositis  No  17%  NI  GC  0.1 y  Yes 
Ikic (2014)10  46  lcSSc  Neg  No  uDT  NI  27%  NI  No  10 y  No 
Ikic (2014)10  32  lcSSc  Neg  No  uDT  No  15%  NI  No  12 y  No 
Ikic (2014)10  24  Overlap – RA  NS  No  Arthritis  No  15%  NI  TCZ, MTX, GC  2.5 y  No 
Lenaerts (2018)11  50  dcSSc  Neg  No  No  Yes  20%  5 mo  NR  109 mo  No 
Lenaerts (2018)11  49  lcSSc  RNA – polimerasa III  No  Myositis  Yes  40%  5 y  GC, MTX  23 mo  No 
Lenaerts (2018)11  47  dcSSc  PM – Scl – 100  No  NI  Yes  40%  27 mo  NR  23 mo  No 
De Diego (2020)a  58  dcSSc  Scl-70  Yes  Myositis, arthralgias  Yes  26%  14 mo  MTX, CFM, RTX  36 mo  No 

Dx: diagnosis; ANA: anti-nuclear antibody; ILD: interstitial lung disease; Interval Dx-TC: interval between Dx and HT; ISx: immunosuppressant; y: years; mo: months; wk: week; M: man; W: woman; NI: not included; NS: no specificity; uDT: upper digestive tract; iDT: inferior digestive tract; RP: Raynaud's phenomenon; lcSSc: limited cutaneous SSc; cdSSc: cutaneous diffuse SSc; Neg: negative; RA: rheumatoid arthritis; MTX: methotrexate; MMF: mycophenolate mofetil; D-PEN: D-penicillamine; AZA: azathioprine; GC: glucocorticoids; CFM: cyclophosphamide; CSA: cyclosporine A; TCZ: tocilizumab; RTX: rituximab.

a

Case reported in this article.

Conclusions

Although the literature is limited, the case presented, along with those previously published, demonstrates that HT is a valid therapeutic option in SSc with severe cardiac involvement especially if non-cardiac systemic involvement is ruled out and/or controlled.

Consents and approval of the ethics committee

The publication of the case was approved by the ethics committee of the Donostia University Hospital. Informed consent was obtained from the patient for the publication of his case.

Conflict of interest

The authors declare that they have no conflicts of interest. Also, all authors read and approved the final manuscript.

References
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Copyright © 2021. Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología
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