Clinical and laboratory characteristics of patients with sickle-cell and autoimmune/connective tissue diseases

Abstract

Patients with sickle-cell disease who develop autoimmune connective tissue disorders can present with similar clinical manifestations and/or complications making them indistinguishable. We report here a single-institution study on a cohort of 24 patients, with the above combination. The results suggest that there is often a significant delay in making the second diagnosis because of the overlapping clinical profile. It is emphasized that the clinician should be aware of and vigilant about such cases and should diligently follow up such cases to make an early diagnosis and start the appropriate treatment.

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

“…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]. This may suggest an etiopathological link between these two entities.…”

“…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].…”

“…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].…”

“…Its clinical manifestations range from an asymptomatic state detected only by routine hematologic testing to a chronic triad of recurrent painful vasoocclusive episodes (complicated by progressive organ damage), hemolytic anemia and a predisposition to severe infections [1,2]. Prominent late complications of SCD include avascular necrosis of bone, progressive renal impairment, leg ulcerations, proliferative retinopathy and cerebrovascular accidents [1].…”

“…Prominent late complications of SCD include avascular necrosis of bone, progressive renal impairment, leg ulcerations, proliferative retinopathy and cerebrovascular accidents [1]. Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with an unpredictable clinical presentation and course [2]. The hallmark of SLE is the multisystem involvement, with prominent cutaneous, musculoskeletal, hematological, renal and neurologic manifestations [1].…”