Systemic lupus erythematosus associated with sickle-cell disease: a case report and literature review

Maamar, Mouna; Tazi-Mezalek, Zoubida; Harmouche, Hicham; Mounfaloti, Wafaa; Adnaoui, Mohammed; Aouni, Mohammed

doi:10.1186/1752-1947-6-366

Journal of Medical Case Reports

Table 1 Summary of previous case reports of SCD and SLE[2–16]

From: Systemic lupus erythematosus associated with sickle-cell disease: a case report and literature review

Lead author/year/reference	Sex/origin	Age of SCD onset	Age of SLE onset	SLE features	Immunologic features	Hemoglobin type	Treatment	Outcome
Cherner 2010[3]	F/Afro-Caribbean	13	21	Arthritis, fever	ANA+	SS	Prednisone	Clinical improvement
				Malar rash	Anti-CCP+		Methotrexate
				Gut vasculitis	Anti-RNP+		Rituximab
					ACL+		Cyclophosphamide
Cherner 2010[3]	F/Afro-Caribbean	7	41	Skin rash	ANA+	SS	Prednisone	Clinical improvement
				Renal disease (biopsy not performed)	Anti-DNA+
					Anti-Ro+
Appenzeller 2008[4]	F/African-American	NA	16	Fever, arthritis	ANA+	SS	Prednisone	Clinical improvement
				Photosensitivity	Anti-SM+		Azathioprime
				Cardiomyopathy
				Pericarditis
Appenzeller 2008[4]	F/African-American	15	21	Arthritis	ANA+	SS	Prednisone	Clinical improvement
				Pleuritis	Anti-DNA+	SS	Hydroxychloroquine
				Lymphadenopathy	Anti-Sm+
Appenzeller 2008[4]	F/African-American	NA	57	Arthritis	Anti-Sm+	SS	Prednisone
				Photosensitivity			Hydroxychloroquine	Clinical improvement
				Discoid lesions
				Raynaud’s phenomenon
Michel 2008[2]	F/NA	NA	30	Arthritis	ANA+	SS	Prednisone	Deceased
				Pericarditis	Anti-DNA+
				Pleuritis	Anti-SSA+
				GN class II
Michel 2008[2]	M/NA	NA	40	Arthritis	ANA+	SS	Prednisone	Remission
				Discoid lesions			Hydroxychloroquine
				Thrombocytopenia
Michel 2008[2]	F/NA	NA	32	Thrombocytopenia	ANA+	SC	Hydroxychloroquine	Remission
					Anti-DNA+
Michel 2008[2]	F/NA	NA	35	Arthritis	ANA+	SS	Prednisone	Deceased
				Cutaneous vasculitis	Anti-DNA+		Hydroxychloroquine
				Raynaud’s phenomenon	Anti-Sm+		Methotrexate
				GN class II	Anti-SSA+
					Anti-RNP
Michel 2008[2]	F/NA	NA	27	Arthritis	ANA+	SS	Prednisone	Remission
					Anti-DNA+		Hydroxychloroquine
Michel 2008[2]	F/NA	NA	25	Arthritis	ANA+	SS	Prednisone	Remission
				GN class III	Anti-DNA+		Hydroxychloroquine
				Jaccoud arthropathy	ACL+
				Major depression
Michel 2008[2]	M/NA	NA	26	Arthritis	ANA+	SC	Hydroxychloroquine	Clinical improvement
					Anti-DNA+
					Anti-RNP+
					ACL+
Michel 2008[2]	F/NA	NA	28	Arthritis	ANA+	SS	Prednisone	Persistent renal disease
				GN class IV	Anti-DNA+		Hydroxychloroquine
				Bullous lupus	Anti-Sm+		Dapsone
					Anti-RNP+
Michel 2008[2]	F/NA	NA	32	Arthritis	ANA+	SS	Prednisone	Remission
				Kikuchi’s disease	RF+
				Autoimmune hepatitis
Michel 2008[2]	F/NA	NA	40	Arthritis	ANA+	SS	Hydroxychloroquine	Clinical improvement
				Discoid lupus	ANA+
				Venous thrombosis	Anti-Ro+
					ACL
Michel 2008[2]	F/NA	NA	38	Arthritis	ANA+	SS	Prednisone	Clinical improvement
							Hydroxychloroquine
Michel 2008[2]	F/NA	NA	17	Arthritis	ANA+	SS	Prednisone	Clinical improvement
				Thrombocytopenia	Anti-Ro+		Hydroxychloroquine
					Anti-La+
					ACL+
Michel 2008[2]	F/NA	NA	35	Pedal and peri-orbital edema	ANA+	SC	Prednisone	Dialysis
				Ascites and renal failure	Anti-DNA+		Cyclophosphamide
				GN class IV
Oqunbiyi 2007[6]	M/African	NA	8	Malar rash			Prednisone	Clinical improvement
				Arthritis			Hydroxychloroquine
				Seizures
				Fever
Khalide 2005[7]	F/NA	16	24	Heart failure	Anti-DNA+	SC	Prednisone	Clinical improvement
				Renal failure	Anti-Sm+
				Pericarditis	Lupus anticoagulant+
				Pulmonary emboli
				Polyneuropathy
				Generalized seizures
Khalide 2005[7]	M/NA	NA	16	Discoid rash	ANA+	SS	Prednisone	Clinical improvement
				Polyarthritis	Anti-DNA+		Hydroxychloroquine,
				Partial seizures			azathioprine
Khalide 2005[7]	M/NA	NA	23	Skin rash	ANA+	SS	Hydroxychloroquine	Lost to follow up
				Pleuritis	ACL+
				Arthritis
				Raynaud’s phenomenon
Khalide 2005[7]	F/NA	NA	28	Arthritis	ANA+	SS	Prednisone	Clinical improvement
				Oral ulcers	Anti-DNA+
				GN class III	ACL
Saxena 2003[8]	M/African-American	NA	9	Arthritis	ANA+	SS	Prednisone	Clinical improvement
				Fever	Anti-DNA+		Cyclophosphamide
				Acute chest syndrome	Anti-SSA+
				Pericarditis
				Seizures
Saxena 2003[8]	F/African-American	NA	7	Fever	ANA+	SS	Prednisone	Clinical improvement
				Arthritis	Anti-DNA+		Cyclophosphamide
				Alopecia			Azathioprine
				GN class II
Saxena 2003[8]	F/African-American	NA	11	Fever	ANA+	SS	Prednisone	Clinical improvement
				Arthritis			Cyclophosphamide
				Skin rash
				Seizures
				Cardiomegaly
Saxena 2003[8]	F/African-American	NA	14	Seizures	ANA+	SS	Prednisone	Septic shock due to pneumococcal bacteremia
				Malar rash	Anti-DNA+		Cyclophosphamide
				Splenomegaly			Azathioprine
				Arthritis			Plasmapheresis
				Pericarditis			Splenectomy
Saxena 2003[8]	M/African-American	NA	17	Malar rash	ANA+	SS	Prednisone	Hemodialysis dependent
				Alopecia			Cyclophosphamide
				Pericarditis
				Cardiomegaly
				GN class V
Shetty 1998[9]	F/Afro-Carribbean	Nine months	10	Arthritis	LE cells in pericardial effusion	SS	Prednisone	Clinical improvement
				Pulmonary infiltrate
				Pericarditis
				Myocarditis
Pham 1997[10]	F/Afro-Caribbean	NA	18	Arthritis	ANA+	SS	Prednisone	Clinical improvement
				Nephrotic syndrome	Anti-DNA
Katsanis 1987[11]	F/Afro-Caribbean	NA	16	Arthritis	ANA+	SS	Prednisone	Clinical improvement
				Malar rash	Anti-DNA+		Hydroxychloroquine
				Photosensitivity	Anti-Sm+
				Pleuritis
				Pericarditis
				Renal class II
Katsanis 1987[11]	F/Afro-Caribbean	NA	15	Arthritis	ANA+	SS	Prednisone	Clinical improvement
				Pleuritis	Anti-DNA borderline	SC	Prednisone	Clinical improvement
Warrier 1984[12]	F/Afro-Caribbean	NA	11	Malar rash	ANA+
				Alopecia	Anti-DNA+
				Arthralgia	Anti-ENA+
				Seizures
				Hepatosplenomegaly
Luban 1980[13]	F/African-American	NA	8	Discoid lesions	Positive LE	SC	Prednisone	Clinical improvement
				Pericarditis	ANA+
				Myocarditis
Luban 1980[13]	F/African-American	NA	14	Fever	ANA+	SS	Prednisone	Clinical improvement
				Renal disease	Positive LE
Karthikeyan 1978[14]	F/African	4	15	Arthritis	ANA+	SS	Prednisone	Clinical improvement
				Raynaud’s phenomenon	Positive LE cell test
				Photosensitivity
Wilson 1976[15]	F/African-American	30	40	Arthritis	Positive LE cells	SS	Prednisone	Deceased
				Pleuritis
				Libman-Sacks endocarditis
Wilson 1976[15]	F/African-American	Four months	16	Arthritis	ANA+	SS	Prednisone	Clinical improvement
				Hepatitis	Anti DNA +
				Pneumonitis
Wilson 1976[16]	F/African-American	NA	27	Arthritis	Histopathologic evidence for SLE on post-mortem examination	SS	No treatment for SLE	Deceased
				Malar rash
				Pulmonary congestion
				Hepatomegaly
				Nephrotic syndrome
				Cerebral and subarachnoid hemorrhage

ACL=anti-cardiolipin antibodies; ANA=anti-nuclear antibodies; anti-ENA=anti-extractable nuclear antigen antibodies; GN=glomerulonephritis; NA=not available; RF=rheumatoid factor; anti-RNP=anti-ribonucleoprotein antibodies; SCD=sickle-cell disease; SLE=systemic lupus erythematosus; anti-SSA=anti-Sjögren syndrome antigen A antibodies.

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ISSN: 1752-1947

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