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Table 1 Summary of previous case reports of SCD and SLE[216]

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     
  1. 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.