Systemic lupus erythematosus in patients with sickle cell disease

Abstract

Sickle cell disease (SCD) is a prevalent genetic disorder that includes sickle cell anemia (hemoglobin SS), hemoglobin SC, and hemoglobin Sb-thalassemia. Patients with SCD present with a defective activation of the alternate pathway of the complement system that increases the risk of capsulate bacteria infection and failure to eliminate antigens, predisposing these patients to autoimmune diseases. The authors describe three patients with SCD that developed systemic lupus erythematosus (SLE). In all patients, SLE diagnosis was delayed because symptoms were initially attributable to SCD. Physicians should be alerted to the possible development of SLE in patients with SCD to not delay the diagnosis and start appropriate treatment.

Parvovirus B19 Associated Systemic Lupus Erythematosus in a Child with Sickle Cell Disease; a Diagnostic and Therapeutic Challenge

“…Coexistence of SCD and SLE has been previously reported but, because of the limited number of cases from previous reports it is not possible to conclude whether SLE is more or less prevalent in patients with sickle cell hemoglobinopathies [1,[3][4][5]. In fact, a MEDLINE search of the literature, from 1970 to 2004 revealed only 19 cases of coexisting SLE and SCD, from which only 14 fulfilled the 1997 revised criteria of the ACR, which highlights the rarity of the overlap between these entities [1] and since both conditions have a high prevalence in blacks, a protective effect of SCD in preventing SLE has been speculated [1,7].…”

“…In fact, a MEDLINE search of the literature, from 1970 to 2004 revealed only 19 cases of coexisting SLE and SCD, from which only 14 fulfilled the 1997 revised criteria of the ACR, which highlights the rarity of the overlap between these entities [1] and since both conditions have a high prevalence in blacks, a protective effect of SCD in preventing SLE has been speculated [1,7]. On the other hand, the defective alternate pathway of the complement system, characteristic of the SCD patients, leading to a failure to eliminate bacterial antigens with subsequent formation of immune complexes, may predispose these patients to the development of autoimmune diseases like SLE, although the data on the latter are conflicting [2,3]. Furthermore, ANA positivity seems to be more common in patients with SCD (23%) than in the general population (10-15% of healthy children) [2].…”

“…The classification of SLE is made when a patient fulfills at least four of the 11 revised criteria established by the American College of Rheumatology (ACR) in 1997. Fever, ar-Year 2012 Volume:1 Issue:1 71-76 thralgia/arthritis and myalgia are common in both conditions [3,4]. Additionally, both diseases commonly affect the renal, cardiovascular and central nervous systems [4].…”

“…Additionally, both diseases commonly affect the renal, cardiovascular and central nervous systems [4]. Although the etiology of this two conditions is different, their coexistence has been rarely reported [1][2][3][4][5].…”

Increased Prevalence of Autoimmunity and Connective Tissue Diseases in Sickle Cell Disease

“…This high prevalence of autoantibody reactivity adds to the diagnostic challenge in differentiating the onset of CTD in SCD. Additionally, clinical similarities in clinical presentation of CTDs and SCD can further lead to diagnostic imprecision and diagnostic delay [6,19] which can have a huge impact on diseases like RA and SLE where early intervention with disease modifying anti-rheumatic drugs (DMARD's) is crucial for prevention of disease progression.…”