Abstract
Objectives: To study the characteristics of Systemic Lupus Erythematosus in Jordan
and compare results with other Arab countries in the region.
Methods: This descriptive study was conducted on 50 Jordanian patients with Systemic
Lupus Erythematosus attending the rheumatology clinic at King Hussein
Medical Center
between 2000 and 2007. King Hussein Medical Centre is one of the main
referral hospitals in Jordan. Data was collected from patients, relating to
the various disease manifestations and mode of presentation, age at disease
onset, disease duration, and family history of Systemic Lupus Erythematosus.
Statistical analysis was performed using simple descriptive and bivariate statistics,
such as mean, median, range and Chi square.
Results: Forty-four females and six male
patients were included. Female to male ratio was 7.3:1 with median age of 22
years (range 14-48). Mean disease duration was 43 months (range 12-90 months). Clinical manifestations of Systemic Lupus
Erythematosus in descending order were, musculoskeletal (90%), cutaneous and
oral ulcers (85%), renal complications (50%), neuropsychiatric (45%),
haematological (40%), pulmonary complications (30%), and cardiac complications
in 15% of cases. Secondary anti-phospholipid antibody syndrome (APS) occurred
in 30% of cases. Three patients died from severe chest infection. Another patient died from massive sagittal sinus thrombosis
and severe hydrocephalus. A comparison between our findings and published Systemic
Lupus Erythematosus studies from four Arab countries revealed no significant
clinical differences.
Conclusion: Systemic Lupus Erythematosus is an important rheumatological disease with serious
consequences including morbidity and mortality.
It behaves in a similar way to Systemic Lupus Erythematosus in other
Arab countries. Renal,
pulmonary and neurological complications are the main cause of morbidity. Chest
infection is a major cause of mortality.
Key words: SLE, Vasculitis, Jordan,
Arabs
JRMS September 2010; 13(3): 20-24
Introduction
Systemic Lupus Erythematosus (SLE) is a
chronic inflammatory autoimmune disease of unknown aetiology, which is recognized
worldwide. Many observations suggest a role for genetic predisposition,
hormonal, immunological and environmental factors in its pathogenesis.(1)
It is a multi-system disease that produces symptoms varying from mild to life
threatening. Marked variations in the frequencies of SLE amongst differing
racial groups have been noticed.(2) Studies from Arab
countries are few.(3-8) In this study, we attempt to analyze
SLE characteristics in Jordanian population and compare it with similar studies
in other Arab countries.
This study was conducted to study the characteristics of Systemic Lupus
Erythematosus in Jordan
and compare results with other Arab countries in the region.
Methods
This is a descriptive study to involving patients with SLE attending a
rheumatology clinic at King Hussein Medical Center (KHMC) between 2000 and 2007. Fifty Jordanian patients were included in the
study, all fulfilling the 1982 ACR revised criteria for the diagnosis of SLE
and followed up by same attending rheumatologist.(3)
Data collected from patients, relating to the
various disease manifestations and mode of presentation, age at disease onset,
disease duration, and family history of SLE.
All patients had full CBC, ESR, ANA, dsDNA, ENA, anti-cardiolipin
antibodies, RF, CRP, a-PTT, lupus anticoagulant and x-ray of chest, hands and
feet.
Statistical analysis was performed using
simple descriptive and statistics, such as mean, median and range and Chi square.
Results
Fifty patients with SLE were studied,
44 females and six males with a ratio of 7.3:1. All patients were Jordanians
from Arab descent. The median age of patients was 22 years (range 14-48 years).
The mean duration of follow up was 32 months (range 3-84 months).
The females: male median age of disease
onset was 20.5: 21 years respectively. The range of disease duration was 43
months. Three patients (6%) had a family history of SLE. Demographic data is shown
in Table I.
The different clinical manifestations among
the study group from Jordanian and other Arabic countries are summarized in Table
II. Forty patients (80%) presented with
skin lesions suggestive of SLE, alopecia (60%), butterfly rash (50%), painless
mouth ulcers (30%), photosensitivity (20%), and discoid lupus (8%).
Musculoskeletal manifestations are the
commonest presenting symptoms, and occurred in 90% of patients. Arthralgia was
noticed in 80% of cases, non-erosive arthritis in 60%, and myalgia in 40% of
cases. Aseptic necrosis of the hip was observed in four (8%) patients proved by
Magnetic Resonance Imaging (MRI).
Twenty five patients, (23 females and 2 males) had evidence of renal involvement ranging from mild to severe
proteinuria, abnormal urinary casts and raised serum creatinine. Twenty
patients had proteinuria more than one gram per 24 hours underwent kidney
biopsy. According to WHO classification of renal glomerulonephritis, two
patients had moderate mesangial lupus nephritis (class II), five patients had
focal segmental proliferative lupus nephritis (class III), ten patients had diffused
proliferative lupus nephritis (class IV) and three patients had membranous
glomerulonephritis (class V nephritis).
Two female patients needed temporary
haemodialysis, both responded well to steroid and immunosuppressive therapy.
Another two patients went into end stage renal disease and needed long-term renal
replacement therapy. There was no difference in the occurrence of renal
complications between both sexes though the number was small to make
statistical conclusion.
Many patients presented with central
nervous system involvement. Thirteen patients (26%) presented with different
neuropsychiatric complications of SLE. Six
patients (12%) presented with psychiatric symptoms ranging from mild depression
to severe psychosis. Seizures were
observed in three patients (6%), trigeminal neuralgia (4%), stroke (4%), and
aseptic meningitis (4%).
Forty percent of patients had evidence
of hematological disorder. Leucopenia was the commonest haematological finding
observed in 35% of patients. Anemia was seen in 26% of patients, 12% of these
patients had evidence of hemolytic anemia. Thrombocytopenia was noticed in 20%
of patients.
Fifteen
patients (30%), 14 females and one male patient had evidence of APS according
to antiphospholipid syndrome classification criteria (4).
Another two patients, one female and one male had deep vein thrombosis without
evidence of antiphospholipid syndrome. There was significant association
between APS and deep vein thrombosis (P≤0.05). Ten patients (20%) presented
with deep vein thrombosis and five (10%) patients with recurrent deep vein
thrombosis. Five patients (10%) developed sagittal sinus thrombosis. Two
patients had axillary vein thrombosis and another patient developed hepatic
vein thrombosis.
Five patients (30%) had positive lupus
anticoagulant, six patients (33%) had positive anticardiolipin antibodies and
six patients (33%) had both tests positive. Seven patients (50%) presented with recurrent abortions. Six patients (12%) died during the study period in spite of all treatment modalities. Three patients died of severe chest infection. Two patients died of severe haemorrhagic pneumonitis. One patient died of severe hydrocephalus secondary to massive sagittal sinus thrombosis.
Table I. Demographic characteristics of
50 Jordanian patients with SLE
|
|
No of
patients
|
%
|
|
Female
Male
Female:
male ratio
|
44
6
7.3:1
|
90
10
|
|
Mean age of
disease onset
Female
Male
|
21.9
22.3
|
|
|
Family
history
|
3
|
6
|
Table II. Clinical and serological
characteristics of 50 SLE patients seen at King Hussein Medical Centre compared
with SLE patients from other Arab countries
|
Country
|
Jordan
|
Lebanon(4)
|
Kuwait(5)
|
UAE(6)
|
SA(7)
|
|
No.
of patients
|
50
|
100
|
108
|
33
|
87
|
|
F:M
ratio
|
7.3:1
|
6.1:1
|
9.8:1
|
15.1:1
|
8.7:1
|
|
Median
age
|
23
|
25
|
31.5
|
26
|
28.5
|
|
Clinical features%
(ACR 1982
revised criteria)
|
|
|
Malar
rash
|
60
|
52
|
43
|
36
|
56
|
|
Discoid
lupus
|
16
|
19
|
10
|
3
|
18
|
|
Photosensitivity
|
25
|
16
|
48
|
42
|
26
|
|
Oral
ulcers
|
50
|
40
|
33
|
27
|
16
|
|
Arthritis
|
80
|
95
|
87
|
91
|
91
|
|
Serositis
|
26
|
40
|
29
|
33
|
56
|
|
Renal
|
50
|
50
|
37
|
63
|
63
|
|
Neuropsychiatric
|
20
|
10
|
23
|
9
|
25
|
|
Hematological
Leucopenia
Anaemia
Hemolytic anemia
Thrombocytopenia
|
40
35
28
12
20
|
47
17
-
10
33
|
53
83
-
-
26
|
45
30
-
9
21
|
78
33
-
-
21
|
|
Positive
dsDNA
|
85
|
50
|
58
|
97
|
93
|
|
Antiphospholipid
syndrome
|
30
|
5
|
21
|
33
|
10
|
|
Positive
ANA
|
95
|
87
|
94
|
89.5
|
98 |
Discussion
In this study, the clinical spectrum of
a group of Jordanian patients with SLE was presented and results were compared with
similar published studies in other Arab countries.
The female to male ratio is 7.3:1,
which is in agreement with published reports in Lebanon
and Saudi Arabia and less
than what was observed in Kuwait
and United Arab Emirate.(5-8) The study confirms that it is a
disease of young people. The median age of the disease onset is the third
decade, and there is no significant difference between ages of disease onset in
both sexes. These results are similar to
published studies in the region.
The clinical spectrum of the disease
was almost similar to the clinical manifestation of the disease in other
countries in the regions. Musculoskeletal involvement was the commonest
presenting feature of the disease (80%) and is comparable to those published
reports in the region. The cutaneous features are the second commonest presenting
feature (60%). Renal involvement was the main cause of increased morbidity. Lupus
nephritis affected half of the patients (50%) which is almost similar to the
published studies in UAE (54%), Lebanon
(50%), but less than those reported in Saudi
Arabia (63%) and more than those reported in Kuwait
(37%). Three patients went into end stage renal disease and needed renal
replacement therapy. The reason for the high rate of renal complications in our
cohort of patients is that we have easy access and direct referral system to
our clinics and good collaboration with hospital renal unit.
Secondary
antiphospholipid syndrome has a common association with SLE. It is an
autoimmune disease which is characterized by antiphospholipid antibodies and
one clinical manifestation, the most common being venous or arterial thrombosis
and recurrent fetal loss.(4) Love and Santro in their analysis of over 1000
patients with SLE found an average prevalence of 34% of lupus anticoagulant and
44% for anticardiolipin antibodies.(11) Secondary lupus
anticoagulant constituted 30% of our group of patients, which is significantly
higher than previously reported studies in Lebanon (5%), Kuwait (20%) and Saudi
Arabia (10%). The high rate of APS in our group of patients compared to other
Arab countries in the region might be that King Hussein Medical Centre is the
main referral center in Jordan
and the difficult and complicated cases are more likely to be referred to it.
Vascular complications are an important
cause of increased morbidity. Ten patients presented with deep vein thrombosis
including, five patients with recurrent deep vein thrombosis, five patients
presented with sagittal sinus thrombosis. Unfortunately, there are no published
data regarding vascular complication of the disease to compare with.
Haematological complications of the disease are almost similar to results in
other Arab countries.
SLE could be a serious disorder despite of all
available treatment options, though the mortality rate has improved remarkably
since the introduction of steroids and other immunosuppressant drugs. The
leading causes of mortality in SLE are irreversible renal damage, fulminant
infection and uncontrolled disease activity.(12,13)
Mortality rate in our study was 12%,
which is in concordance with other published international studies, though
there is no mortality data published in the region to compare with. The main
cause of mortality in our study was severe chest infection, then haemorrhagic
pneumonitis and massive sagittal sinus thrombosis. The spectrum of infection
was similar to that published in most major studies.(14)
Renal involvement was an important
cause of increased morbidity, however, this did not add to increase mortality
in our group of patients. This could be attributed to the aggressive management
and availability of renal replacement therapy. Similar observation was noticed by
Al-Jarallah in Kuwait.(5)
Conclusion
SLE in Jordan behaves
in a similar way to other Arab countries in the region. Chest infections and
haemorrhagic pneumonitis are the main cause of mortality. Secondary
antiphospholipid syndrome is a common associated complication.
Further
epidemiological studies are needed to find out the exact incidence and
prevalence of SLE in Jordan.
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