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Lupus Nephritis in a Patient with Sickle Cell Disease

DOI: 10.1155/2013/907950

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Abstract:

Introduction. The diagnosis of systemic lupus erythematosus (SLE) in patients with sickle cell disease (SCD) can be difficult to establish because the musculoskeletal, central nervous system, and renal manifestations are similar in both diseases. In the presented case, we highlight the diagnostic challenge that can evolve in patients with a concurrence of both diseases and we establish the importance of early recognition and treatment of lupus nephritis in patients with SCD. Case Presentation. We present a case of a 31-year-old African American female with sickle-C disease (hemoglobin SC) who was admitted to our hospital with complaints of periumbilical abdominal pain associated with intractable nausea and vomiting, abdominal distension, and worsening lower extremity edema. Urine studies revealed nephrotic range proteinuria and the immunological investigations were consistent with lupus. A renal biopsy revealed focal proliferative lupus nephritis. Conclusion. It is important to consider the presence of a coexisting autoimmune disease in a patient with sickle hemoglobinopathy who displays an atypical and multisystem presentation that is unresponsive to conventional therapies. When a significant kidney disease is present, a renal biopsy is critical in identifying the etiology of a renal abnormality in the setting of coexisting SLE and SCD. 1. Background Systemic lupus erythematosus (SLE), or lupus, is a chronic, progressive, autoimmune disorder that affects multiple organ systems, with a broad range of clinical and laboratory manifestations [1]. Sickle cell disease (SCD) encompasses a group of autosomal-recessive genetic disorders characterized by the production of abnormal hemoglobin S (HbS). The protean clinical features of SCD result from chronic variable intravascular hemolysis and microvascular ischemia, leading to damage in multiple organs [2]. The diagnosis of SLE in patients with an underlying chronic hemoglobinopathy can be difficult to establish because the musculoskeletal, central nervous system, and renal manifestations are similar in both diseases. In the presented case, we highlight the diagnostic challenge that can evolve in patients with a concurrence of both diseases and we establish the importance of early recognition and treatment of lupus nephritis in patients with SCD. 2. Case Presentation A 31-year-old African American female with sickle-C disease (hemoglobin SC) was admitted to our hospital with complaints of periumbilical abdominal pain associated with intractable nausea and vomiting, abdominal distension, and worsening lower

References

[1]  S. L. Gurevitz, J. A. Snyder, E. K. Wessel, J. Frey, and B. A. Williamson, “Systemic lupus erythematosus: a review of the disease and treatment options,” The Consultant Pharmacist, vol. 28, no. 2, pp. 110–121, 2013.
[2]  M. Michel, A. Habibi, B. Godeau et al., “Characteristics and outcome of connective tissue diseases in patients with sickle-cell disease: report of 30 cases,” Seminars in Arthritis and Rheumatism, vol. 38, no. 3, pp. 228–240, 2008.
[3]  M. Cherner and D. Isenberg, “The overlap of systemic lupus erythematosus and sickle cell disease: report of two cases and a review of the literature,” Lupus, vol. 19, no. 7, pp. 875–883, 2010.
[4]  E. Katsanis, E. Hsu, K.-H. Luke, and J. A. McKee, “Systemic lupus erythematosus and sickle hemoglobinopathies: a report of two cases and review of the literature,” American Journal of Hematology, vol. 25, no. 2, pp. 211–214, 1987.
[5]  V. R. Saxena, R. Mina, H. J. Moallem, S. P. Rao, and S. T. Miller, “Systemic lupus erythematosus in children with sickle cell disease,” Journal of Pediatric Hematology/Oncology, vol. 25, no. 8, pp. 668–671, 2003.
[6]  C. Toly-Ndour, A.-M. Rouquette, S. Obadia et al., “High titers of autoantibodies in patients with sickle-cell disease,” Journal of Rheumatology, vol. 38, no. 2, pp. 302–309, 2011.
[7]  M. Maamar, Z. Tazi-Mezalek, H. Harmouche, W. Mounfaloti, M. Adnaoui, and M. Aouni, “Systemic lupus erythematosus associated with sickle-cell disease: a case report and literature review,” Journal of Medical Case Reports, vol. 6, article 366, 2012.
[8]  N. A. Khalidi, H. Ajmani, and J. Varga, “Coexisting systemic lupus erythematosus and sickle cell disease: a diagnostic and therapeutic challenge,” Journal of Clinical Rheumatology, vol. 11, no. 2, pp. 86–92, 2005.
[9]  S. Appenzeller, A. Fattori, S. T. Saad, and L. T. L. Costallat, “Systemic lupus erythematosus in patients with sickle cell disease,” Clinical Rheumatology, vol. 27, no. 3, pp. 359–364, 2008.
[10]  A. K. Shetty, M. R. Baliga, A. Gedalia, and R. P. Warrier, “Systemic lupus erythematosus and sickle cell disease,” Indian Journal of Pediatrics, vol. 65, no. 4, pp. 618–621, 1998.
[11]  K. López Revuelta and M. P. Ricard Andrés, “Kidney abnormalities in sickle cell disease,” Nefrologia, vol. 31, no. 5, pp. 591–601, 2011.
[12]  K. V. Kanodia, A. V. Vanikar, K. R. Goplani, S. B. Gupta, and H. L. Trivedi, “Sickle cell nephropathy with diffuse proliferative lupus nephritis: a case report,” Diagnostic Pathology, vol. 3, no. 1, article 9, 2008.

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