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Systemic lupus erythematosus associated with sickle-cell disease: a case report and literature review.
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PMID:  23101910     Owner:  NLM     Status:  PubMed-not-MEDLINE    
Abstract/OtherAbstract:
INTRODUCTION: The occurrence of systemic lupus erythematosus has been only rarely reported in patients with sickle-cell disease.
CASE PRESENTATION: We describe the case of a 23-year-old North-African woman with sickle-cell disease and systemic lupus erythematosus, and discuss the pointers to the diagnosis of this combination of conditions and also present a review of literature. The diagnosis of systemic lupus erythematosus was delayed because our patient's symptoms were initially attributed to sickle-cell disease.
CONCLUSIONS: Physicians should be alerted to the possible association of sickle-cell disease and systemic lupus erythematosus so as not to delay correct diagnosis and initiation of appropriate treatment.
Authors:
Mouna Maamar; Zoubida Tazi-Mezalek; Hicham Harmouche; Wafaa Mounfaloti; Mohammed Adnaoui; Mohammed Aouni
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Type:  Journal Article     Date:  2012-10-26
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Title:  Journal of medical case reports     Volume:  6     ISSN:  1752-1947     ISO Abbreviation:  J Med Case Rep     Publication Date:  2012  
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Created Date:  2012-12-04     Completed Date:  2013-02-07     Revised Date:  2014-07-14    
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Nlm Unique ID:  101293382     Medline TA:  J Med Case Rep     Country:  England    
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Journal ID (nlm-ta): J Med Case Rep
Journal ID (iso-abbrev): J Med Case Rep
ISSN: 1752-1947
Publisher: BioMed Central
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Received Day: 3 Month: 8 Year: 2012
Accepted Day: 28 Month: 9 Year: 2012
collection publication date: Year: 2012
Electronic publication date: Day: 26 Month: 10 Year: 2012
Volume: 6First Page: 366 Last Page: 366
PubMed Id: 23101910
ID: 3512532
Publisher Id: 1752-1947-6-366
DOI: 10.1186/1752-1947-6-366

Systemic lupus erythematosus associated with sickle-cell disease: a case report and literature review
Mouna Maamar1 Email: maamarmouna@yahoo.fr
Zoubida Tazi-Mezalek1 Email: z.tazi@menara.ma
Hicham Harmouche1 Email: hharmouche@yahoo.fr
Wafaa Mounfaloti1 Email: wmounfaloti@yahoo.fr
Mohammed Adnaoui1 Email: madnaoui@yahoo.fr
Mohammed Aouni1 Email: aounisimo@yahoo.fr
1Department of Internal Medicine, Ibn Sina Hospital, Rabat, Morocco

Introduction

Sickle-cell disease (SCD) is a prevalent genetic disorder that includes sickle-cell anemia (the homozygous and most common form of SCD (SS)), sickle-cell hemoglobin C (SC) and sickle-cell β thalassemia (S/β thal) [1]. The protean clinical features of SCD result from chronic variable intravascular hemolysis and microvascular ischemia, leading to damage in multiple organs [2]. The occurrence of connective tissue diseases, in particular systemic lupus erythematosus (SLE), has only been rarely reported in patients with SCD [2]. The incidence of SLE in patients with SCD is not known because most of the published studies are case reports. Due to similar clinical manifestations, diagnosis of SLE in patients with SCD may be difficult and is often delayed. We report the case of a patient who developed symptoms initially attributed to SCD, but on further investigation underlying SLE was revealed.


Case presentation

A 23-year-old North-African woman with no family history of SCD was admitted to our department of internal medicine with symptoms of anemia, bone pain, arthralgia and fever. Her symptoms had been developing for six weeks with alteration of her general condition and abdominal pain. On physical examination our patient was pale, she had a temperature of 39.5°C, her blood pressure was 130/75mmHg and heart rate was 100 beats/minute. The patient had slight splenomegaly, pain on pressure in the long bones and arthritis in her knees.

Blood test results showed normocytic anemia at 6.6g/dL with a high reticulocyte count (230,000 cells/mm3), hyperleukocytosis with granulocytosis (leukocyte count 16,500 cells/mm3, polymorphonuclear cells 9500 cells/mm3) and moderate thrombopenia (100,000 cells/mm3). Further investigations showed diminished haptoglobin (0.08mg/L), elevated lactate dehydrogenase (4670UI/L) indirect hyperbilirubinemia (21mg/L) with moderate cytolysis and cholestasis (aspartate aminotransferase 43U/L, alanine aminotransferase 65U/L, phenylalanine ammonia lyase 217U/L and γ-glutamyl transpeptidase 188U/L). Hemoglobin (Hb) electrophoresis test results showed Hb S at 50.3 percent, Hb C at 44 percent and Hb A1 at 0 percent, confirming a diagnosis of SCD (hemoglobin S/C).

Our patient’s erythrocyte sediment rate was 110mm/first hour, her C-reactive protein level was 38mg/L (range <6mg/L), fibrinogen was 6.4g/L (24g/L) and serum protein electrophoresis showed a polyclonal IgG 24g/L (range 9 to 13g/L) with normal immunofixation. Results of a chest X-ray were normal. Abdominal ultrasonography, transthoracic and transesophageal echocardiography results were also normal. A thoraco-abdominal scan revealed numerous splenic infarctions. The results of a bone scan showed diffuse bone infarcts.

Her symptoms were attributed to SCD and hence our patient received blood transfusions, antibiotics and analgesics, but with no improvement. Her fever and arthritis failed to respond to this treatment. Instead, the evolution of her condition was marked by the development of arthritis in her hands and relapse of anemia.

Blood culture test results were negative, and the result of a tuberculin skin test was an 8mm induration. There was no BK virus found in repeated sputum and urine examinations, and procalcitonin test results were negative.

Serology test results for human immunodeficiency virus, hepatitis B, hepatitis C, brucellosis and typhoid fever were all negative. Cytobacteriological urine analysis revealed no bacteria but microscopic hematuria (670 cells/mm3) and leukocyturia (50 cells/mm3). Proteinuria results were negative.

The results of a Coombs test performed on admission were strongly positive for IgG. Immunological investigations revealed a positive anti-nuclear antibody (1/2600) result, and a positive anti-Sm result. Anti-DNA antibody tests were negative. A test for anti-extractable nuclear antigen antibodies (anti-ENA) was negative. C3 levels and C4 levels were normal (respectively, 0.95g/L and 0.3g/L). Tests for anti-phospholipid antibodies were negative. A diagnosis of SLE associated to SCD was established, with five of the diagnostic criteria of the American College of Rheumatology being met. Steroids were administered as a pulse of methylprednisolone 1g/day for three days followed by oral prednisone at 1mg/kg/day with hydroxychloroquine. Her symptoms quickly improved. At her 18-month follow up, she was in clinical remission on prednisone 5mg per day and hydroxychloroquine; she had not experienced a sickle-cell crisis and her lupus is still quiescent.


Discussion

In the present report we described the case of a Moroccan woman with SCD and coexistent SLE. The overlap of SLE and SCD is of interest, but the limited number of patients that have been reported previously implies that the association is uncommon [3]. Only 40 similar cases have been reported in the literature over the last 50 years [2-16] (Table 1). The African/Afro-Caribbean/African-American population is predisposed to contracting both SCD and SLE, explaining the fact that most patients with this association are African women (70 percent in Table 1 and 73 percent in the series by Michel et al.). All reported cases were relatively young at the time of lupus diagnosis (mean age 23 years, range eight to 57 years). All of them had SCD several years before SLE. Articular involvement is the most frequent lupus-related symptom, present in 84 percent of cases, followed by serositis (36 percent), and glomerulonephritis class III or IV (11 percent). Cutaneous manifestations are not frequently mentioned. Positive anti-nuclear antibody (ANA) results were found in 34 cases. Prognosis was favorable in 80 percent of cases (Table 1). Patients with SCD present with a defective activation of the alternate pathway of the complement system; this is the reason why these patients are at increased risk of capsulate bacteria infection, such as from pneumococci [15]. Some authors have suggested the hypothesis that this defect may lead to immune complex disorders secondary to failure to eliminate antigens, predisposing these patients to autoimmune diseases, but this has not been confirmed in other studies [3,11,13]. The clinical features of SLE and SCD have certain elements in common. Diverse manifestations such as polyarthritis, anemia, fever, visceral pain, renal, cardiovascular and pulmonary involvement are common in both conditions. Owing to the overlap of clinical features in the two diseases it may easy to confuse them, as occurred with our patient.

Further, the frequency and titers of antibodies in SCD have been reported as relatively higher than in population controls, making the diagnosis more challenging in clinical practice [17].

Toly-Ndour et al. reported that 50 percent of 88 patients with SCD had positive anti-nuclear antibody results and 20 percent had titers greater than one in 200, but only one patient developed rheumatoid arthritis five years later and no patients developed SLE [18]. In this series, patients treated with hydroxyurea had ANA-positive results less frequently than non-treated patients (P=0.053) [18].

Large prospective epidemiological studies are necessary to determine whether the prevalence of immune complex diseases is increased in patients with SCD.


Conclusions

This report illustrates the importance of considering associated diseases when clinical findings are unexplained by SCD alone, or are unresponsive to the conventional treatment. Early diagnosis and the initiation of appropriate treatment may decrease morbidity and mortality in these patients.


Consent

Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Competing interests

The authors declare that they have no competing interests.


Authors’ contributions

MM was the major contributor to the writing of the manuscript. ZTM reviewed the manuscript and prepared the final draft. HH and WM made substantial contributions to the acquisition and interpretation of clinical data and performed the literature research in PubMed. MAd and MAo gave final approval for the version to be published. All authors read and approved the final manuscript.


References
Steinberg MH,Management of sickle cell diseaseNew Engl J MedYear: 19993401021102910.1056/NEJM19990401340130710099145
Michel M,Habibi A,Godeau B,Bachir D,Lahari A,Galacteros F,Fifi-Mah A,Arfi S,Characteristic and outcome of connective tissue diseases in patients with sickle cell disease: report of 30 casesSemin Arthritis RheumYear: 20083822824010.1016/j.semarthrit.2007.10.00318177923
Cherner M,Isenberg D,The overlap of systemic lupus erythematosus and sickle cell disease: report of two cases and a review of the literatureLupusYear: 20101987588310.1177/096120330935629120064908
Appenzeller S,Fattori A,Saad ST,Costallat TL,Systemic lupus erythematosus in a patient with sickle cell diseaseClin RheumatolYear: 20082735936410.1007/s10067-007-0779-718000698
Kanodia VK,Vanikar AV,Goplani KR,Gupta SB,Trivedi HL,Sickle cell nephropathy with diffuse proliferative lupus nephritis: a case reportDiagn PatholYear: 200828918307766
Oqunbiyi AO,Geoge AO,Brown O,Okafor BO,Diagnostic and treatment difficulties in systemic lupus erythematosus coexisting with sickle cell diseaseWestr Afr J MedYear: 200726152155
Khalidi NA,Ajmani H,Varga J,Coexisting systemic lupus erythematosus and sickle cell disease. A diagnostic and therapeutic challengeJ Clin RheumatolYear: 200511869210.1097/01.rhu.0000158549.92844.9616357709
Saxena VR,Mina R,Moallem HJ,Rao SP,Miller ST,Systemic lupus erythematosus in children with sickle cell diseaseJ Pediatr Hematol OncolYear: 20032566867110.1097/00043426-200308000-0001912902927
Shetty AK,Baliga MR,Gedalia A,Warrier RP,Systemic lupus erythematosus and sickle cell diseaseIndian J PediatrYear: 19986561862110.1007/BF0273090910773915
Pham TP,Lew SQ,Balow JE,Sickle cell nephropathy during the postpartum period in a patient with SLEAm J Kidney DisYear: 19973087988310.1016/S0272-6386(97)90099-89398137
Katsanis E,Hsu E,Luke KH,McKee JA,Systemic lupus erythematosus and sickle hemoglobinopathies: A report of two cases and review of the literatureAm J HematolYear: 19872521121410.1002/ajh.28302502113605068
Warrier RP,Sahney S,Walker H,Hemoglobin sickle cell disease and systemic lupus erythematosus.J Nate Med AssocYear: 19847610301031
Luban NL,Boeckx RL,Barr O,Sickle cell anemia and SLEJ PediatrYear: 19809611206154794
Karthikeyan G,Wallace SL,Blum L,SLE and sickle cell diseaseArthritis RheumYear: 198021862863697956
Wison WA,Nicholson GD,Hughes GR,Amin S,Alleyne G,Serjeant GR,Hemoglobin sickle cell disease and systemic lupus erythematosusBr Med JYear: 1976181310.1136/bmj.1.6013.8131260347
Wilson FM,Clifford GO,Wolf PL,Lupus erythematosus associated with sickle cell anemiaArthritis RheumYear: 1964744344910.1002/art.178007041114202583
Baethge BA,Bordelon TR,Mills GM,Bowen LM,Wolf RE,Bairnsfather L,Antinuclear antibodies in sickle cell diseaseActa HaematolYear: 19908418618910.1159/0002050602125783
Toly-Ndour C,Rouquette AM,Obadia S,M’bappe P,Lionnet F,Hagege I,Boussa Khettab F,Tshilolo L,Girot R,High titers of autoantibodies in patients with sickle cell disease.J RheumatolYear: 20113830230910.3899/jrheum.10066721123321

Tables
[TableWrap ID: T1] Table 1 

Summary of previous case reports of SCD and SLE[2-16]


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.



Article Categories:
  • Case Report

Keywords: Sickle-cell disease, systemic lupus erythematosus.

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