Coexistence of lupus nephritis and sickle cell trait, an electron microscopic assessment of renal glomerular damage
Case report of a rare association
Keywords:
Systemic lupus erythematosus, Lupus nephritis, Sickle cell trait, Aplastic anemia, Febrile neutropeniaAbstract
Because of similarities of the musculoskeletal, central nervous system, and renal manifestations in both diseases, diagnosing systemic lupus erythematosus (SLE) in sickle cell disease (SCD) patients can be difficult to establish. Although Sickle Cell Trait (SCT) is still considered a benign form of SCD, its impact on kidney injury and other renal manifestations is well recognized in the literature. In this case report, we look at the challenges that develop when diagnosing patients with a concurrence of both diseases and the importance of early recognition and treatment of lupus nephritis in SCT patients. We present a case of a male patient with sickle-cell trait who was admitted to our hospital complaining of low grade fever and pancytopenia for investigations proven to be SLE. A renal biopsy on electron microscopy assessment with different staining modalities as well as immune fluorescence revealed mixed pathological changes. We emphasize the importance in considering the presence of a coexisting autoimmune disease in a patient with sickle hemoglobinopathies even in the milder forms like SCT which may display an atypical and/or multisystem presentation. Also, the impact of the two conditions on the renal pathological changes should be expected to be more damaging even at early onset of SLE flare, and hence, an urge to start with more intensified immunosuppressive medications.
References
Ismail Hossain M, Sarwer jahan SM, Ashraful haque M, Mobasher Alam ABM, Ahmed M, Zakir Hossain
M. Systemic lupus Erythematosus in male: two case reports. Bangladesh Journal of Medicine. 2013; 24(2).
Schur PH. Systemic Lupus Erythematosus (SLE). John Wiley & Sons; 2015: 1–11. doi:
1002/9780470015902.a0002147.pub3.
Kanodia KV, Vanikar AV, Goplani KR, Gupta SB, Trivedi HL. Sickle cell nephropathy with diffuse
proliferative lupus nephritis. Diagnostic Pathology. 2008; 3: 9. doi: 10.1186/1746-1596-3-9.
Emilia Chalayer, Martine F French, Pascal Cathébras
Saxena VR, Mina R, Moallem HJ, Rao SP, Miller ST. Systemic lupus erythematosus in children with sickle
cell disease. J Pediatr Hematol Oncol. 2003; 25(8): 668-71. doi: 10.1097/00043426-200308000-00019.
PMID: 12902927.
Stuart MJ, Nagel RL. Sickle cell disease. Lancet. 2004; 364(9442): 1343-60. doi: 10.1016/S0140- 6736(04)17192-4.
Flanagan G, Packham DK, Kincaid-Smith P. Sickle cell disease and the kidney. Am J Kidney Dis. 1993;
(3): 325-7. doi: 10.1016/S0272-6386(12)80755-4.
Shariff S, Al-Hemi B, Al Yousif R, Jabreel N, Al Gharreb S, Balaji D. Spectrum of renal disease in sickle
cell anemia. Indian J Nephrology. 2006; 16: 157-63.
Khalidi NA, Ajmani H, Varga J. Coexisting systemic lupus erythematosus and sickle cell disease: a
diagnostic and therapeutic challenge. J Clin Rheumatol. 2005; 11(2): 86-92. doi:
1097/01.rhu.0000158549.92844.96.
Saborio P, Scheinman JI. Sickle cell nephropathy. J Am Soc Nephrol. 1999; 10(1): 187-92. PMID:
Naik RP, Lanzkron S, Morgan E, Grams ME, Coresh J. Sickle Cell 'Trait' Linked to Chronic Kidney
Disease. JAMA. 2014.
Yildirim-Toruner C, Diamond B. Current and novel therapeutics in the treatment of systemic lupus
erythematosus. J Allergy Clin Immunol. 2011; 127(2): 303-12. doi: 10.1016/j.jaci.2010.12.1087. PMID:
, PMCID: PMC3053574.
Kalloo S, Aggarwal N, Mohan P, Radhakrishnan J. Lupus Nephritis: Treatment of Resistant Disease. Clin J
Am Soc Nephrol. 2013; 8: 154-61. doi: 10.2215/CJN.05870612. PMID: 23296380.
Sterner RM, Hartono SP, Grande JP. The Pathogenesis of Lupus Nephritis. J Clin Cell Immunol. 2014;
(2): 205. doi: 10.4172/2155-9899.1000205. PMID: 25133068, PMCID: PMC4131745.
Weening JJ, D'Agati VD, Schwartz MM, Seshan SV, Alpers CE, Appel GB, et al. The Classification of
Glomerulonephritis in Systemic Lupus Erythematosus Revisited. J Am Soc Nephrol. 2004; 15: 241-50.
PMID: 14747370.
Published
Issue
Section
License
Copyright (c) 2020 KNOWLEDGE KINGDOM PUBLISHING
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.