Successful treatment of pure red cell aplasia associated with systemic lupus erythematosus with cyclosporin A

Duarte-Salazár, Carolina; Cazarín‐Barrientos, Jorge; Goycochea-Robles, María Victoria; Collazo-Jaloma, Juan; Burgos‐Vargas, Rubén

doi:10.1093/rheumatology/39.10.1155

Cited by 10 publications

(4 citation statements)

References 12 publications

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“…Haematological abnormalities, if relapsing or refractory to steroids, have been shown to respond well to CyA. This is the case of lupus-associated thrombocytopenia [93,94], haemolytic anaemia [95], aplastic anaemia [96], red cell aplasia [97][98][99] and haemophagocytic syndrome [100].…”

Section: Cyclosporine a In Systemic Lupus Erythematosusmentioning

confidence: 99%

The Use of Cyclosporine A in Rheumatology: a 2016 Comprehensive Review

Chighizola

Ong

Meroni

2016

Clinic Rev Allerg Immunol

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Cyclosporine A, an inhibitor of calcineurin, exerts an immunomodulator action interfering with T cell activation. Even though novel therapeutic tools have emerged, CyA still represents a suitable option in several clinical rheumatology settings. This is the case of refractory nephritis and cytopenias associated with systemic lupus erythematosus. Furthermore, CyA is a valued therapeutic tool in the management of uveitis and thrombophlebitis in course of Behçet's disease. Topical CyA has been proven to be beneficial in the dry eye of Sjogren's syndrome, whereas oral treatment with CyA can be considered for the severe complications of adult onset Still's disease. CyA provides a therapeutic option in psoriatic arthritis, being rather effective in skin disease. CyA is currently regarded as a second-line option for patients with inflammatory myopathies refractory to standard regimen. CyA is used even in paediatric rheumatology, in particular in the management of juvenile dermatomyositis and macrophage activation syndrome associated with systemic juvenile idiopathic arthritis. Importantly, CyA has been shown to suppress the replication of HCV, and it can thus be safely prescribed to those patients with chronic hepatitis C. Noteworthy, CyA can be administered throughout the gestation course. Surely, caution should be paid to CyA safety profile, in particular to its nephrotoxicity. Even though most evidence comes from small and uncontrolled studies with few randomised controlled trials, CyA should be still regarded as a valid therapeutic tool in 2016 rheumatology.

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Section: Cyclosporine a In Systemic Lupus Erythematosusmentioning

confidence: 99%

The Use of Cyclosporine A in Rheumatology: a 2016 Comprehensive Review

Chighizola

Ong

Meroni

2016

Clinic Rev Allerg Immunol

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“…Hasta el momento la revisión de la literatura describe únicamente 13 casos en los que la APCR se diagnostica como debut o previo al inicio de los demás síntomas de LES (Tabla) [5][6][7][8][9][10][11][12][13][14][15][16] . Todos corresponden a mujeres, con una edad media de 30 años, siendo el caso actual el primero descripto de un varón, y además de edad avanzada (65 años).…”

Section: Discussionunclassified

“…Se considera que en los casos de APCR secundarios a enfermedades autoinmunes y del colágeno, las manifestaciones hematológicas deben responder al tratamiento específico de la enfermedad de base, que en la práctica suele consistir en la inmunosupresión en las enfermedades autoimnunes 1 . Los pacientes detallados en la Tabla así lo demuestran para la APCR como debut de LES; el pronóstico habitualmente es favorable con corticoides, habiéndose descripto solo en dos ocasiones el fracaso terapéutico en los que se requirió terapia alternativa con gammaglobulinas y ciclosporina A 10,11 .…”

Section: Discussionunclassified

Aplasia pura de células rojas como forma de presentación de lupus eritematoso sistémico

Martín-Serradilla

Tirados

Buergo

et al. 2023

Rev. Argent. Reumatol.

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La aplasia pura de células rojas (APCR) es un síndrome definido por anemia normocítica normocrómica, con reticulopenia severa y reducción importante o ausencia absoluta de precursores eritroides en la médula ósea. Ocasionalmente se desencadena en el curso de una colagenopatía o una enfermedad autoinmune. Presentamos el primer caso descripto en la literatura de un varón con APCR como forma de debut de lupus eritematoso sistémico (LES). Se trata de un hombre de 65 años que presentó anemia normocítica normocrómica, ANA 1/5120 y anti-Sm 2,61. Refería úlceras orales, poliartralgias, tumefacción de ambos tobillos y fotosensibilidad. Se realizó estudio de médula ósea con evidencia de hipoplasia de serie roja por paro madurativo a nivel de eritroblasto basófilo, ausencia casi completa de los elementos maduros y contenido muy elevado de proeritroblastos de gran tamaño. Con el diagnóstico de APCR como debut de LES, se lo trató con prednisona con buena respuesta. Podemos concluir que el despistaje de enfermedades sistémicas en pacientes con APCR es esencial para asegurar un correcto manejo y un mejor pronóstico.

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“…with variable results [10]. Cyclosporin A treatment has proved to be useful both in patients with secondary anemia following rHuEPO therapy [11] and in those with anemia secondary to other diseases such as systemic lupus erythematosus [12]. Likewise, cyclosporin A has been used as an effective treatment for the prevention of transfusional hyperimmunization in dialysis patients.…”

Section: Discussionmentioning

confidence: 99%

Antierythropoietin antibody-induced pure red cell aplasia: posttransplant evolution

et al. 2004

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Pure red cell aplasia is a rare complication of recombinant human erythropoietin (rHuEPO) treatment, which physicians should consider once the more frequent causes of hyporegenerative anemia have been excluded. To our knowledge, no pediatric cases have been described. In our patient, cyclosporin A treatment enabled a reduction in the number of transfusions and the risk of hyperimmunization. After transplantation, our patient's hemoglobin level has remained normal and stable.

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Successful treatment of pure red cell aplasia associated with systemic lupus erythematosus with cyclosporin A

Cited by 10 publications

References 12 publications

The Use of Cyclosporine A in Rheumatology: a 2016 Comprehensive Review

The Use of Cyclosporine A in Rheumatology: a 2016 Comprehensive Review

Aplasia pura de células rojas como forma de presentación de lupus eritematoso sistémico

Antierythropoietin antibody-induced pure red cell aplasia: posttransplant evolution

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