CASE REPORT  
Niger J Paed 2015; 42 (2): 158 –161  
Adisa AK  
Adamu H  
Asani MO  
Aliyu I  
Systemic lupus erythematosus in a  
7
-year-old girl: A first case report  
from northern Nigeria  
DOI:http://dx.doi.org/10.4314/njp.v42i2.18  
Accepted: 7th January 2015  
Abstract: Systemic lupus erythe-  
matosus is an autoimmune multi-  
systemic inflammatory disease  
that is rare in children. Though a  
disease of the black race it is  
rarely diagnosed in black African  
children. Only few cases have  
been report in Nigeria and these  
were in the south. We report a  
case of a 7-year-old girl who pre-  
sented with recurrent body swell-  
ings, an unusual rash, pericardial  
effusion and gastro-intestinal dis-  
turbances. The diagnosis was  
made after serology was found to  
be positive for Anti-nuclear anti-  
body (ANA). She later developed  
stroke which was characterized by  
a convulsive episode, loss of con-  
sciousness and subsequent right-  
sided hemiplegia. She gradually  
regained consciousness after three  
days with residual weakness of the  
right side of her body. She has  
commenced prednisolone and is  
currently on follow-up at our  
clinic.SLE though rareshould be  
considered in any child with multi-  
organ disease, nephritis or stroke,  
especially after common condi-  
tions in our environment have  
been excluded.  
(
)
Adisa AK  
Adamu H, Asani MO, Aliyu I  
Department of Paediatrics,  
Aminu Kano Teaching Hospital, Kano  
Nigeria  
Email: adisakolly@yahoo.com  
Key words; Systemic lupus ery-  
thematosus, serositis, stroke, ne-  
phritis, rashes.  
Introduction  
cough or changes in her behavior or school performance.  
An initial abdominal ultrasound showed bilaterally  
enlarged kidneys and urine culture had a positive growth  
for Escherichia coli sensitive to cefixime. Cefixime was  
given at 100mg twice daily and she was also given fu-  
rosemide at 20mg twice daily. Thereafter, she developed  
facial rash which later progressed to involve the trunk,  
extremities and lower lip. The rashes were itchy and  
more on the sun-exposed parts of her body.  
Systemic Lupus Erythematosus (SLE) is a chronic multi  
1
-systemic autoimmune connective tissue disease .It was  
th  
first described by Rogenius in the 13 century when he  
coined the wordlupus”meaning “wolf bite” . It is char-  
acterized by periods of increased disease activity (flares)  
and then remission . Its incidence increases with age and  
it has a remarkable predilection for the females, espe-  
cially after puberty . SLE is prevalent among black  
children resident in Europe and America but rare among  
2
1
1
,3  
1
-3  
Fig 1: Peri-orbital  
edema and fading  
facial rashes  
blacks in Africa . It is very uncommon in the first dec-  
ade of life and only 20% of all cases occur in the second  
1
decade therefore many paediatricians miss the diagnosis  
at first presentation.  
Case report  
A 7-year-old girl was first seen at the Paediatric Out-  
Patient Department (POPD) of the Aminu Kano Teach-  
ing Hospital (AKTH) 5weeks prior to her admission.  
She presented with recurrent facial and abdominal swell-  
ings (Fig.1) for the past one year. The facial swelling  
would improve as the day went by. The abdominal  
swelling was associated with easy satiety. There was no  
swelling of her feet and she had no urinary symptoms.  
She had no abdominal pain or difficulty with breathing.  
She had no history of jaundice, sore throat, joint pain or  
swelling. There was no history of blood transfusion,  
The urinalysis showed proteinuria (2+) and hematuria  
(
1+) and her electrolytes, blood urea nitrogen,  
creatinine, lipid profile, liver enzymes were essentially  
normal (Tab. 1.0).  
One week later, she was rushed into our Emergency  
Paediatric Unit (EPU) with complaints of recurrent  
vomiting and abdominal pain which started two days  
prior. She had two episodes of similar complaints twice  
in the preceding month but not severe enough to warrant  
1
59  
admission. At presentation, she had multiple bouts of  
copious coffee-ground vomitus and was weak, sweaty  
and cold. The rashes had worsened, despite withdrawal  
of her medications (cefixime and furosredmide). She had a  
normal height of 115cm (above the 3 centile for age)  
While awaiting the result she was placed on 1g of ceftri-  
axone twice daily, intravenous furosemide (2mg/kg/day  
in two divided doses) and spironolactone (12.5mg twice  
daily) and intravenous fluid for the persistent vomiting,  
intravenous cimetidine (200mg twice daily) was also  
added and pentazocine (1.5mg/kg thrice daily) given for  
abdominal pain. Review of ANA result (6.5 which is  
above lab reference of 1.2) led us to a diagnosis of Sys-  
temic Lupus Erythematosus when combined with three  
other criteria that she had fulfilled viz; serositis, rashes  
and nephritis. She was commenced on high dose predni-  
soloneat 1mg/kg/day, aspirin and sunscreen lotion while  
awaiting renal biopsy and ophthalmologic examination  
prior to commencing hydroxychloroquine. She im-  
proved and was discharged to clinic for follow up. A  
week later, she developed another episode of vomiting,  
abdominal pain and subsequently had an episode of gen-  
eralized convulsion associated with loss of conscious-  
ness and right-sided body weakness which was worst at  
onset. Computerized Tomograph (CT) of the brain  
showed a large left hemispheric infarct. Subsequently,  
she improved regained of consciousness but had residual  
aphasia and right-sided hemiparesis. She is still on pred-  
nisolone and has commenced physiotherapy  
rd  
and weight of 25kg (above the 3 centile). There were  
widespread doughnut-shaped, target-like maculopapular  
rashes with erythematous border on the face, upper trunk  
and upper and lower limbs with relative sparing of the  
extensor aspect of the upper limbs and flexor aspect of  
the lower limbs, pulses were feeble and regular, blood  
pressure on the right arm in sitting position was  
th  
8
0/50mmHg (<50 centile for gender, age and height),  
jugular venous pressure was not elevated, apex was dif-  
fuse and she had muffled first and second heart sounds,  
no added sounds. Abdomen was distended with as cites  
demonstrable by shifting dullness. There was hepa-  
tomegaly with liver span of 14cm, firm and non-tender,  
no ballotable kidneys. While on admission she devel-  
oped fever, worsening of abdominal pain, therefore  
spontaneous bacterial peritonitis was considered.  
An urgent abdominal ultrasound confirmed marked as-  
cites, chest radiograph showed enlargement and globular  
appearance of the cardiac silhouette (CTR=0.72) with  
apparently clear lung field. Electrocardiography showed  
low-voltage waves with no rhythm abnormality and  
echocardiography revealed moderate pericardial effu-  
sion with normal systolic function (Fig. 2). A repeat  
dipstick analysis showed moderate proteinuria (2+),  
hematuria (1+) with no other abnormality.  
Summary of some essential investigations  
Parameter  
Result  
Normal Range  
35-50  
Complete blood count  
Packed cell volume(%)  
White cell c3ount  
30.6  
Total /mm  
7000  
2300  
4000  
403 x 10  
4000-11,000  
1500-7000  
1000-3700  
3
Neutrophils/mm  
Lymphocytes/mm  
Platelet count/mm  
3
Fig 2a: Parasternal  
long axis/M-mode  
Echo-cardiography  
showing anterior  
and posterior effu-  
sions  
3
3
3
150-450x10  
Ascitic fluid  
Protein (g/l)  
Glucose (g/dl)  
43  
0.5  
50  
3
WCC (/mm )  
Liver Function tests(U/L)  
Enzymes  
ALP  
ALT  
AST  
Bilirubin-Total (µmol/L)  
Direct (µmol/L)  
Serum Protein( g/l)  
Total  
Albumin  
Globulin  
10  
3
2
10  
3
42-110  
4-34  
7-45  
4-18  
0-7  
Fig 2b: Dimensional  
apical 4-chamber  
view  
68  
36  
32  
59-86  
32-51  
20-43  
Clotting Profile  
PT  
13s  
36s  
13-16s  
35-43s  
PTTK  
Blood chemistry  
Urea (mmol/L)  
Sodium(mmol/L)  
Potassium (mmol/L)  
Bicarbonate (mmol/L)  
Chloride (mmol/L)  
Creatinine (µmol/L)  
Lipid Profile(mmol/L)  
Total chol.  
HDL  
LDL  
TRIG  
Urine Microscopy  
Pus cells  
6.7  
137  
3.6  
25  
97  
85  
2.1-6.9  
130-146  
3.0-5.6  
20-28  
94-108  
30-111  
Full Blood Count (FBC) showed relative lymphocytosis,  
and platelet count was normal (Tab. 1.0), mantoux test  
was non-reactive, erythrocyte sedimentation rate was  
slightly elevated(35mm/hr), sputum for Acid fast bacilli  
was negative and ascitic tap yielded no organism with  
normal cell count and glucose, Serum-Ascites Albumin  
Gradient (SAAG) was 2.5g/dl (transudative). Serologic  
investigations for hepatitis and HIV were negative and  
Anti-Nuclear Antibodies (ANA) assay was requested.  
2.8  
0.8  
1.54  
0.8  
2.5-6.4  
0.8-2.6  
0.8-4.3  
0.5-2.8  
2
Scanty  
0
Epithelial cells  
RBC  
1
60  
Discussion  
ascites (peritoneal serositis) seen in our patient are com-  
mon gastro-intestinal manifestations in the disease. Gas-  
trointestinal involvement occurs in one-third of patients  
manifesting as serositis, vasculitis, pancreatitis or enteri-  
Systemic Lupus Erythematosus is uncommon in chil-  
dren and rarer in African black children with only few  
case reports . Less than 10% of SLE cases worldwide  
are diagnosed in the first decade . Our patient presented  
at the age of 7 years which is comparable to the median  
4
1
tis and abdominal pain . Most patients respond to diuret-  
1
ics and steroids and a few may require chloroquine to  
achieve complete resolution of the ascites. Our patient  
improved without the need for chloroquine. Similarly,  
our patient had a stroke which is seen in 8-22% of all  
2
age (9.2years) at diagnosis among Indian children .  
However, Olowu in Ile-Ife (Southwest Nigeria) studied  
5
9
1
1 children and found the median age to be 11.2 years.  
cases of SLE . Cerebrovascular event in SLE is due to  
The youngest among his patients was 6 years at diagno-  
sis. The median age at diagnos1,i6s in Ile-Ife was similar to  
the global average of 12years.  
one or combination of accelerated atherosclerosis, vas-  
culitis, coagulopathy in those that are positive anti-  
phospholipid,3a,9ntibodies and or thromboembolism from  
1
endocarditis . The most likely mechanisms in our  
Recognised trigger factors in SLE include infections,  
medications (antihypertensives and anticonvulsants) and  
patient is vasculitis in view of her vasculitic rash, nor-  
mal coagulation profile (Tab. 1) and the brain CT that  
showed an extensive area of ischemic infarct on the left  
cerebral hemisphere. However,the inability to do lupus  
anticoagulant was a limitation in our evaluation of this  
patient. Other neuropsychiatric symptoms seen in SLE  
include seizures, headache and behavioural abnormali1--  
ties and may be seen in as many as 90% of SLE cases  
1
-3  
hormonal changes . Our patient had culture-positive  
urinary tract infection which was a possible trigger. Fur-  
thermore, it was possible that sun exposure contributed  
as a trigger in this instance considering the time of the  
year in the northern part of the country. Generally, the  
disease is thought to result from a combination of hu-  
moral and environmental factors in genetically predis-  
posed individuals. Human Leucocyte Antigen (HLA)  
class II alleles DR2 and DR3 contribute to disease sus-  
ceptibility in some patients as inherited complement  
3
,9  
.
SLE may presents with pancytopenia or isolated cell  
1
line depletion, erythrocyte sedimentation rate (ESR) is  
usually elevated depending on disease activity but  
1
deficiencies .  
C-reactive protein (CRP) is usually normal except in the  
1
,3  
presence of infection . Our patient had a slightly ele-  
vated ESR but her blood count parameter appeared nor-  
mal even during the febrile episode. The presence of  
fever or absence of white cell abnormalities, as in our  
patient, does not confirm or exclude respectively the  
presence of infection.  
The most frequent presenting symptoms of SLE are pro-  
1
longed fever, malaise weight loss and lymphadenopathy  
-3  
.
This was not the case with our patient who first pre-  
sented with features of nephritis. Renal disease is the  
greatest contributor to morbidity and mortality in paedi-  
atric SLE occurring in 60-80% within the first year of  
Th1e0 American College of Rheumatology (ACR) crite-  
ria for the diagnosis of SLE revised in 1992 require  
that at least 4 out of the 11 criteria should be present  
1
7
disease onset . According to Odetunde et al , nephritis  
with skin rashes was the first manifestation in a 9- year-  
old boy reported to have SLE in Enugu. Likewise in Ile-  
2
either serially or simultaneously . Our patient fulfilled  
5
Ife , the mean time of onset of renal disease was 1.22 ±  
the following five of the ACR criteria; serositis, nephri-  
tis, skin rash, positive ANA and neurologic disorder.  
0
.93 years after onset of systemic illness.  
Our patient had skin rash which was more of vasculitic  
lesions that are not as common as the malar (butterfly)  
rash. The use of cefixime around the time of appearance  
of the rash suggested the possibility of a drug reaction  
but these rash got worse after the withdrawal of the  
drug. Moreso, cephalosporins are not recognized trigger  
Conclusion  
SLE is a rare disease of the black race with very diverse  
manifestations. Nephritis is the most important manifes-  
tation that often leads to its diagnosis in most Nigerian  
series. However, stroke of an unidentified aetiology  
should also make a paediatrician consider SLE like our  
patient clearly demonstrated.  
5
factor. Olowu had noted that only 3 out of the 11 cases  
had the typical malar rash but none of them had the vas-  
culitic rash. It may be that rash (regardless of morphol-  
ogy) is not common manifestations in Nigerian children  
with SLE.  
Virtually all (10/11) of Olowu’s patients presented with  
arthritis involving one or more site(s), which was not the  
case with our patient who had no evidence of joint in-  
volvement. Our patient had echocardiography-confirmed  
moderate pericardial effusion. Pericarditis was the first  
presentation in an 11-year-old girl reported by Elusiyan  
Limitations  
Our patient would have benefitted from renal biopsy,  
ophthalmologic examination and some more specific  
serologic tests which were not done due to logistic chal-  
lenges that are peculiar to our setting.  
8
and Olowu in Ile-Ife . Pericardial effusion is the com-  
monest cardiac presentation and is often a cause of  
recurrent chest pain .  
Acknowledgement  
1
,6  
We sincerely appreciate the efforts of Dr R. Hashim and  
Dr A. Yahaya in the evaluation of this patient.  
The recurrent vomiting and abdominal pain as well as  
1
61  
Authors’ contributions  
ography, reviewed and edited the manuscript and Aliyu  
Ibrahim also reviewed and edited the manuscript.  
Conflict of interest: None  
Adisa Abdulhafeez conceptualized the report and manu-  
script writing. Adamu Halima was involved in manu-  
script writing. Asani Mustafa carried out the echocardi-  
Funding: None  
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