Systemic Lupus Erythematosus

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WHO-ICD-10 version:2010

Diseases of the musculoskeletal system and connective tissue

Systemic connective tissue disorders

OMIM Number

152700

Mode of Inheritance

Autosomal dominant

Gene Map Locus

1p13.2, 1q23.3,2q33.2,3p21.31,4p16-p15.2, 4q22-q24,6p21.3,11q14,12q24,13q32,16p13.3

Description

Systemic Lupus Erythematosus (SLE) is a chronic autoimmune disorder, characterized by production of auto-antibodies against the patient's own nuclear antigens. This is a disease affecting multiple systems, and the symptoms range from chronic fever, malaise, joint pain, myalgia and fatigue to more serious dermatological (malar rash, alopecia, vaginal ulcers), musculoskeletal (arthalgia, arthritis, osteonecrosis, and osteoporosis), hematological (anemia, Raynaud's phenomenon), cardiac (pericarditis, myocarditis, Libman-Sacks endocarditis, and atherosclerosis), pulmonary (pleuritis, pneumonitis, pulmonary emboli, pulmonary hemorrhage), renal (nephritis and renal failure) and/or neurological (seizures and psychosis) complications. The auto-antibodies produced are mainly anti double stranded DNA, anti Smith, anti RNP, anti Ro, anti La etc. The varied manifestations of the disease are due to deposition of immune complexes in the various tissues.

SLE is more common among females than males, increasing to 15 times more during childbearing years. The disease also shows a racial preference, being more prevalent among African American blacks than among the Caucasians. The annual incidence of SLE ranges from six to 35 new cases per 100,000 population in relatively low-risk to high-risk groups.

Diagnosis of the disease is made on the basis of a criteria established by the American College of Rheumatology (ACR) in 1982. The chief test, however is for antinuclear antibody; more specifically for anti dsDNA, anti Smith antigen, and anti extractable nuclear antigen (ENA) antibodies. No cure exists for SLE. Corticosteroids and immunosuppressants are administered to control the disease. Antimalarial drugs like hydroxychloroquine are also sometimes used for the arthritic complications. Nephrological complications may require drugs like Cyclophosphamide.

Not much is known about the genetics of SLE, although the disorder is known to be influenced by multiple genes. One of the most important genes is the Receptor for Fc Fragment of IgG, Low Affinity IIa (FCGR2A). These receptors play a very important role in immune complex clearance. Mutations in the gene, therefore, lead to systemic deposition of immune complexes, resulting in high susceptibility to SLE. Presence of race specific genes is a feature of SLE genetics. SLEB 1 (Susceptibility to Systemic Lupus Erythematosus) and SLEB5 are common in African American families, while the genes SLEB3, SLEB4, and SLEB6 are seen in European American families.

Epidemiology in the Arab World

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Subject IDCountrySexFamily HistoryParental ConsanguinityHPO TermsVariantZygosityMode of InheritanceReferenceRemarks
152700.1United Arab EmiratesFemale Autoimmunity; VasculitisNM_014002.4:c.541-5G>AHeterozygousAutosomal, DominantFathalla et al. 2021 variant-disease asso...

Other Reports

Bahrain

Ebrahim et al. (2002) conducted the first study about systemic lupus erythematosus (SLE) in Bahrain. The clinical and laboratory manifestations of 50 Bahraini patients were studied retrospectively over the ten year period 1991-2000 and the findings were compared with those of the SLE patients in the surrounding area and Caucasian patients. Male to female ratio was 1:17, which was close to that of the UAE. Antinuclear antibodies (ANA) test, latex slide test, radial immuno-diffusion method, enzyme immunoassay, hematological tests, urine analysis, and renal biopsy diagnosis were the main dependant laboratory methods. The initial presenting symptoms were arthralgia/ arthritis (78%) and fever (66%). Renal involvement appeared in 25 patients (50%) in the form of proteinuria, hematuria, and/or biopsy changes. Bahrain series showed significantly less urinary casts than other series. Only 14 patients had renal biopsy, and they were classified according to WHO system. Mesangioproliferative changes (WHO II) formed 44% of the biopsies, diffuse proliferative changes (WHO IV) formed 36%, and membranous glomerulonephritis (WHO V) was seen in 20%. Sixteen patients with renal disease had low serum complement C3 and C4 levels, two patients had only low C3, and one patient had only low C4. It was noticed that low C3 level is an indicator of active disease. Another clinical finding in SLE patients was the neuropsychiatric disorders which involved 13 patients (26%). Of those patients, five had seizures, and two demonstrated cerebral atrophy. In addition, seven had low C3 and C4, while two had only low C3. It is noticeable that neurological manifestations were less in Bahrain series than in UAE series. Skin disorders were also found in 28 patients (56%) in the form of alopecia, malar rash, photosensitivity, and/ or purpuric rash. However, Bahraini patients revealed less photosensitivity than the other compared patients. Hematological findings showed the presence of anemia (84%), Coomb's positive hemolytic anemia (4%), leucopenia (56%), and thrombocytopenia (16%) in Bahrain series. Ebrahim et al. (2002) attributed the presence of low frequency of neuropsychiatric symptoms with the apparent absence of antibody- mediated hemolytic anemia to a coincidence. Positive ANA test had the frequency of 100% and the antibodies were IgG type. On the other hand, double strand DNA antibodies (anti- dsDNA) were found in 72% of patients which was close to that of Caucasians. Only two female patients of eight tested were positive to anticardiolipin antibodies and lupus anticoagulant (25%). Rheumatoid factor was tested in 29 patients and only three had positive result. Ten percent of the patients died from cerebral vasculitis, septicemia, renal failure and infection, and multiple organ failure. [Ebrahim RA, Farid EMA, Greally JF. SLE in Bahrain: A review of clinical and laboratory data in 50 Bahraini patients. Emirates Med J. 2002; 20(2):147-52.]

Al-Naqdy and Al-Shukaily (2005) tested patients with SLE (30 patients), recurrent abortions (44), and thrombosis/thrombocytopenia (36) for the presence of anticardiolipin (ACA) and anti beta2-glycoprotein antibodies. All groups of patients showed a significantly higher frequency of the antibodies, ACA (SLE-23%, recurrent abortions-27%, thrombosis-36%) and anti beta2 GP (SLE-16.6%, recurrent abortions-18%, thrombosis-22%), when compared to 30 age and gender matched healthy controls (ACA-6%, anti beta2 GP-3%). Both antibodies were detected in 10% of the SLE patients, 13.6% of the patients with recurrent abortions, and 1.4% of patients with thrombosis. Incidentally, all patients with SLE who showed the presence of both antibodies also showed clinical characteristics of thrombosis/thrombocytopenia. The authors suggested that both antibodies be used together in the diagnosis of APS, in order to circumvent the lack of specificity of ACA.

[See also: Egypt > Ebrahim and Chawla, 1995].

Egypt

Ebrahim and Chawla (1995) reported a 31-year-old Egyptian woman in whom SS-A(Ro), SS-B(La) antibody positive SLE was diagnosed five years after she gave birth to a daughter with the congenital complete heart block. She had two years history of intermittent pain and swelling of the joints of hands, feet, shoulder, neck, knees and ankles. There was a history of early morning stiffness lasting two to three hours. She complained of loss of hair from her scalp. The patient had two daughters. The younger daughter was affected with neonatal lupus syndrome. The first ECG of the daughter done at three months of age revealed a complete heart block, but echocardiography did not reveal any structural cardiac abnormality. Immunological work-up revealed anti nuclear antibodies positive, anti double stranded DNA antibody positive, LE cell phenomenon positive, antibodies against extranuclear antigens AA-A(Ro) positive, SS-B(La) positive. On the other hand, immunological work-up for the daughter showed that Anti SS-A(Ro), Anti SS-B(La), Anti RNP and Anti SM antibodies were all negative. [Ebrahim RA, Chawla AK. Neonatal lupus syndrome: the role of maternal SS-A(Ro) and SS-B(La) antibodies: a case report. Bahrain Med Bull. 1995; 17(3):118-20.]

Helal et al. (2001) presented the clinical, morphological and immunohistochemical features of 10 cases having the lymphnodal histological pattern of Kikuchi disease. Two of these were diagnosed as systemic lupus erythematosus (SLE). Morphologically, Kikuchi disease and SLE were nearly indistinguishable. Plasma cells, neutrophilic infiltration, hematoxyphilic bodies and vasculitis were not useful in differentiating the conditions.

Kuwait

Khuffash et al. (1990) conducted a hospital-based retrospective study over a period of 8-years on the epidemiology of arthritis and other connective tissue disorders among children in Kuwait. A total of 20 children under the age of 12-years with SLE were identified, giving an average annual incidence and prevalence of 0.53 and 3.37 per 100,000, respectively.

El-Reshaid et al. (1995) found a patient to be serologically positive for SLE in a study of 13 patients with Takayasu's Arteritis. This comorbid patient manifested recurrent thrombosis and laboratory tests confirmed the presence of a hypercoagulable state secondary to protein S deficiency.

Al-Jarallah et al. (1998) undertook a retro-prospective study to characterize the clinical features of SLE patients in Kuwait. The study group included all patients with SLE seen in Kuwait hospitals during a period of five years (1992-1996). Of the 108 patients (98 females, 10 males), 69% were Kuwaitis, while another 80% of the remaining were Arab expatriates. Unlike results of similar studies done in the UAE, the average age of the patients in this study was higher at 31.5 years. The female to male sex ratio in this study (10:1) was comparable to that reported in Western countries, but was lower than that in the Emirati studies. The main clinical features in these patients involved the musculoskeletal system (87%), followed by the mucocutaneous, hematological, constitutional, and renal symptoms. Comparing to other populations, the study population showed a significantly lower frequency of malar rash, oral ulcers, and renal involvement, whereas hematological manifestations were significantly increased. Infection was noticed in 19 patients, with only one of these patients succumbing to it. Very little mortality was attributable to renal involvement. Al-Jarallah et al. (1998) suggested that this could be due to the early detection, and aggressive treatment the patients were subjected to.

Abutiban et al. (2009) compared clinical and serological features between familial and sporadic cases of SLE in Kuwait to establish whether the two constituted a single entity. The study included 135 patients (119 females) seen in 2006 and 2007; 37 of these cases were familial, while the remaining 98 were sporadic. The 37 familial cases (five males) came from 21 families. No statistically significant difference could be observed between the two groups in terms of clinical features or serological manifestations. The sporadic group was found to have a higher frequency of La/SSB antibodies. However, even this was statistically insignificant. The most common initial features in both groups were articular manifestations. Abutiban et al. (2009) concluded that familial and sporadic SLEs were not different disease entities.

Alfadhli and Nizam (2013) studied 22 Kuwaiti patients with SLE and compared the spliced variants in the CTLA4 gene in these patients with those in 22 healthy controls. Patients with SLE showed a reduced frequency of the mCTLA4-672, sCTLA4-562 and N-CTLA4-292 variants. Alfadhli and Nizam (2013) concluded that the expression pattern of the CTLA4 gene could also be playing a role in autoimmunity.

Lebanon

Uthman et al. (2001) undertook a retrospective review of the medical records and renal histologies of 50 Arab patients (43 females and seven males) diagnosed with lupus nephritis and who underwent a kidney biopsy and were admitted to the American University of Beirut Medical Center, in Lebanon, between 1979 and 1999. Of these, 45 patients were Lebanese, while the remaining five included three Palestinians, one Syrian, and one Egyptian. The median age of the patients was 24 years. Renal histology slides from these patients were assessed according to the World Health Organization classification, and were distributed as follows: class I (n = 3, 6%); class II (n = 14, 28%); class III (n = 11, 22%); class IV (n = 19, 38%); class V (n = 1, 2%); class VI (n = 2, 4%). The most common symptoms were articular manifestations, followed by hematological abnormalities. Over 90% of the patients had detectable levels of antinuclear antibodies, while anti dsDNA antibodies were detected in 80%. Nephrotic range proteinuria was seen in 26% of the patients, and 8% had severely impaired renal function. Similar to reports from other Arab countries, the most common histopathological finding was diffuse proliferative LN (class IV). This was followed by pure mesangial (class II). All patients were treated with oral prednisone, while nearly half of them also received monthly pulse intravenous cyclophosphamide. On their last evaluation, out of 37 patients who were followed, 20 patients (54%) had controlled disease, eight patients (22%) were still on active medical treatment, four patients (11%) were on chronic hemodialysis, and five others (13%) had died.

Oman

Al-Four et al. (1996) reported a 30-year old Omani patient diagnosed with SLE who presented with psychiatric symptoms. She presented with a two-month history of episodes of fever, arthralgia, blood stained stools, and abnormal behavior. She was tachycardic, normotensive, with generalized lymphadenopathy. She also had typical facial butterfly rash, right pleural effusion, and hepatosplenomegaly. There was no neurological deficit but she had cognitive dysfunction. Investigations revealed hypochromic microcytic anemia, ESR of 82mm/hr, positive antinuclear and double stranded DNA antibodies but negative rheumatoid factor and LE cells, low compliment C3 and C4 levels, and positive VDRL after initial negativity. Ultrasound confirmed the hepatosplenomegaly, and the right pleural effusion which was sterile (negative gram and ZN stains, no growth on routine culture, and AFB culture). With the above findings, she was then diagnosed as SLE with neuropsychiatric manifestation, and was managed by steroids. Despite that, her condition deteriorated and she began to show features of frank psychosis. Suspecting this to be lupus encephalopathy, the steroid dose was increased. The response could not be monitored, as the patient left medical advice, coming back again after two weeks with an increased state of delirium which did not respond to decreasing the steroid dose from 60mg to 40mg, as a diagnosis of steroid induced psychosis was suspected. She then again left medical advice. Al-Faur and colleagues (1996) diagnosed two other cases with SLE during a period of two years, but neither had shown features of psychiatric illnesses. [Al-Four NS, Shastri KB, Gilani SN. Neuropsychiatric manifestations of systemic lupus erythematosus. Oman Med. J. 1996; 12(3):47-8.]

Al-Maini et al. (2000) studied whether the soluble form of the FAS apoptosis antigen (sFas) had a role in the disease activity or organ damage in SLE by measuring the sFas levels (by double antibody ELISA) of 39 Arab patients with SLE (male:female ratio of 2:37) with different degrees of disease activity and organ damage over a four year period and comparing them to those of 22 race-, gender-, and age-matched healthy individuals. A total of 21 patients had renal involvement (17 confirmed as lupus nephritis by biopsy), 15 had neuropsychiatric manifestation, nine had cardiac involvement, and two had pulmonary involvement. All patients received disease-modifying drugs after initial evaluation. The patients were subdivided into group A (total cohort), group B (patients without major organ involvement), and group C (with major organ involvement) which was further subdivided into group D (renal involvement with other major organ involvement), group E (only renal involvement), group F (central nervous system involvement with other major organ involvement), and group G (only central nervous system involvement). Investigations carried out were full blood counts, renal and liver function tests, ESR, CRP, C3 and C4 compliment levels, and autoantibody [ANA, dsDNA, ENA- SS-A (Ro), SS-B (La), Jo-1, Sm, RNP, and Scl-70] levels. Organ damage was monitored using the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SLICC/ACR). The Student's t-test was used to compare data obtained from the control and the patients as well as for comparison among the patients' subgroups. The correlation between sFas and different variables was analyzed by linear regression analysis followed by stepwise multiple regression to exclude the effect of highly correlated variables and non significant ones. The strength of the correlations was then tested by the beta-coefficient. Al-Maini et al. (2000) founded that the level of sFas was higher among the patients (0.60 ng/ml SD 0.38) than the controls (0.26 ng/ml SD 0.11) and it was higher in all subgroups of group C (except groups F and G) than in group B. When the sFas levels were compared with organ damage index (SLICC/ACR), a positive correlation was found for the total cohort and group C but not group B. No correlation was found between sFas levels and either SLEADI or acute phase reactants. In the cohort and group C, correlation was found between sFas levels and renal function tests, liver function tests, lymphocyte and neutrophil granulocyte counts and anti Sm antibodies. No such correlation was found in group B and no significant difference of sFas levels among different drug treatment subgroups was found. Al-Maini et al. (2000) concluded that elevated serum levels of sFas could be associated with kidney or liver damage in SLE patients, although their study did not prove a causative connection. They also suggested that sFas may actually prevent further damage by inhibiting Fas-mediated apoptosis (could have a therapeutic role in SLE or other conditions with organ damage).

Two years later, Al-Maini et al. (2003) studied the disease presentation during the period of 1994-2000, in 83 Omani SLE patients from two groups, Omani gulf Arabs-OGA (63 patients, mean age-24.5), and Omani Arabs of Persian descent-OAP (20 patients; mean age-26.5), which represented 52 and three tribes, respectively. Their demographic details were included and the disease activity was assessed by SLE disease activity index (SLEDAI) and the 24 SLEDAI descriptors were studied as well. ANA and dsDNA were measured by standard indirect immunofluorescence technique and both IgG and IgM of anticardiolipin (ACA) and anti-beta-2glycoprotein1 antibodies were determined in both groups. Differences between the two groups were analyzed statistical analysis. Antinuclear antibodies (ANA) were found in all patients, while anti dsDNA was found in all OGAs and in only 15 out of 20 OAPs. OGA patients were found to have higher levels of IgG isotype of APL than OAP patients who had higher levels of IgM ACA antibodies than the OGA group. Lower prevalence of skin rash and joint complications was found among the OAP than the OGA patients, and analysis of geographical variation revealed higher risk of joint complications in patients from Dakiliyah region than those from Muscat which might reflect on the large number of OAPs resident there. These two observations, as suggested by Al-Maini and colleagues (2003), explained both genetic and environmental influences on disease presentation. When comparing the APL antibodies and the clinical features within the ethnic groups, it was found that the Farsi tribe (one of the three tribes of OAPs) had a higher prevalence of protinuria and skin rash than the rest of OAPs and OGAs when the anti-beta2-glycoprotein 1 IgM antibodies were the dependant variable, while when the anti-beta2-glycoprotein 1 IgG antibodies were the dependant variable, the other two tribes of OAPs (Belushi and Zadjali) had the higher prevalence of skin rash than the OGAs. It was also found that the ACA had no effect on the patterns of the clinical presentation of SLE in this study. Al-Maini et al. (2002) concluded by highlighting the role of genetic and environmental factors which attributed to the etiopathology of the disease and recommended further studying of the genetic markers to elaborate more in such variation in the disease presentation among different tribes.

El-Ageb et al. (2002) described the clinical profile of patients with Behcet's disease, and determined the levels of their antiphospholipid antibodies, while comparing them with those of SLE patients. The study group, who were all Omanis, consisted of 34 patients affected with Behcet's disease (18 females, 16 males; mean age- 32.8 years), 73 SLE patients (70 females, 3 males; mean age- 23.7 years) and 27 healthy controls (13 females and 14 males; mean age: 26.2 years). The antiphospholipid antibody levels (both IgG and IgM isotypes of anticardiolipin and anti-beta2 glycoprotein I) were measured by ELISA and the antinuclear antibodies (ANA) and anti-dsDNA were measured by standard indirect immunofluorescence techniques. Data obtained were statistically analyzed by chi-square distribution and unpaired t-test. At least one APL antibody was present in 32% and 74% of patients with Behcet's disease and SLE, respectively, while none of the control subjects had these antibodies. Patients with BD were found to have normal values for the mean APL antibody levels (except for IgG anti-beta2 glycoprotein I antibodies), in contrast to a significantly higher range in SLE patients. No significant association between the APL antibodies levels and organ involvement was found in Behcet's disease patients or SLE patients, and all Behcet's disease patients had the same general patterns of the disease regardless of their antibodies levels. When studying the distribution of the antibody types among the two groups of patients, it was found that 41/54 and 4/11 of SLE and Behcet's disease patients, respectively, had both isotypes of APL (ACA and anti-beta2 glycoprotein I) with no difference in its isotype distribution. IgM isotype was found to be more prevalent among the Behcet's disease patients (6/11) than in the SLE patients (10/54), but the opposite was true when the anti-beta2 glycoprotein I antibodies were present alone (4/6 and 7/8 of Behcet's disease and SLE, respectively). ANA were found in all SLE patients but in only 21% of Behcet's disease (two had both ANA and APL antibodies), while anti dsDNA antibodies were present in 93% of SLE patients and none was detected in Behcet's disease patients.

Al-Maini et al. (2003) conducted a retrospective study over a five year period (between 1996-2001) on 73 Omani SLE patients (female: male ratio of 24:1) who fulfilled a minimum of four criteria of the revised American College of Rheumatology (ACR) criteria to determine the demographic, clinical and laboratory features of this disease as well as the association of certain autoantibodies with various system pathologies. In the study group, 62% of the patients were younger than 20-years at the onset of the disease and 48% of the cases had positive family history. The extremely high female: male sex ration and the low age of onset are different from the demographic profile described in patients from other Arab and Asian countries. Al-Maini et al. (2003) suggested that the low age of onset could be linked to the high prevalence of HLA-DR2 antigen in the Omani population. The following hematological and serological manifestations of SLE were most commonly found in the patients: raised ESR of >15mm/hr (75%), anemia (64%), lymphopenia (49%), and albuminemia (44%), Studying the autoantibodies prevalence revealed that ANA was positive in 97% (59% were homogenous and 37% were speckled pattern) and 55% of these titers were >1:640 when compared to only 25% of anti-dsDNA antibodies which was positive in 92% of the patients (both measured by indirect immunofluorescence). Other autoantibodies with their frequencies studied in this cohort were extractable nuclear antigen (ENA) antibodies which were measured by countercurrent immunoelectrophoresis (64%), antineutrophil cytoplasmic antibodies (ANCA) (58%), antiphospholipid antibodies (80%), and rheumatoid factor measured by agglutination test (22%). The antibodies prevalence were not found to be significantly related to the age at onset except for anti-RNP which was found in 33% of younger patients and in only 7% of older patients. The mean disease activity was expressed by SLE disease activity index (SLEDAI) which was found to be 13.5 SD 11.4. Although the score was found higher among the younger age group (14.2 SD 12) than in the older group (12 SD 10.7) it was not statistically different. Systemic involvement seen in the patients involved the immunological (95%), musculoskeletal (48%), neurological (34%), and dermal (33%) systems, among others. Anti-MPO antibody and anti-PR3 antibodies (types of ANCA measured by ELISA) with cumulative frequencies of 52% and 40%, respectively, were found in association with renal pathology and mouth ulcer (anti-MPO) and with vasculitis (anti-PR3). Simple and multinomial regression analysis for the association between ANA/ENA antibodies with all nine SLEDAI systems and eight individual SLEDAI descriptors (arthritis, protinuria, pyuria, new rash, alopecia, hypocomplementemia, fever and leucopenia) was carried out. Anti-ENA antibodies were found to be predictors for neurological and serosal pathologies, while individual anti-ENA antibodies were inhibitory of other systems pathology. Anti-Sm antibody was found to be a predictor for serosal pathology, alopecia and hypocomplementemia, while anti-SS-A antibody was found to be a predictor for musculoskeletal pathology, and inhibitory for the development of fever and arthritis. Anti-Scl-70 antibody was found to be a predictor of pathologies in the immunological system. Pyuria was predicted by high titers of ANA (>1:320). Al-Maini et al. (2003) concluded that such information might help in the understanding of the pathogenesis of SLE as well as determining the long term prognosis of the disease patterns for the Omani patients.

Al Riyami et al. (2003) reported a female patient (14 years old) with a unique variant of urticarial vasculitis syndrome (UVS), who later went on to develop SLE. The patient presented with urticarial rash, and skin biopsy revealed typical leukocytoclastic vasculitis without evidence of IgG or complement deposition. She had three similarly affected siblings and five unaffected siblings. The parents were unaffected and there was no family history of autoimmune diseases, urticaria or allergies. The patient and her affected siblings had low C4 levels but normal C3 and no C1q antibody was detected, and they had positive ANA with speckled pattern, but antibodies to double-stranded DNA, rheumatoid factor and antineutrophil cytoplasmic antibody (ANCA) were negative. Other investigations were normal which included liver function tests, electrolytes, urinalysis and urine microscopy, 24-h urine protein, chest radiography and serology for hepatitis B and C. The condition of the patient improved upon starting high dose steroids and iron supplements and this was evident by normalization of the Hb and ESR with relief of the symptoms. Elective bronchopulmonary alveolar lavage (BAL) revealed friable bronchial mucosa which bled easily and the aspirate had 3.3, 0.6, and 0.4 cells x 106/ml and macrophage levels which were hemosiderin laden of 95, 34, and 88, respectively. Pulmonary function tests revealed restrictive lung disease which was due to the subclinical pulmonary hemorrhage (evident by the BAL results, lung biopsy of the first case, and the iron deficiency anemia in the absence of gastrointestinal bleeding). After a follow up period of five years, the patient developed SLE with high ANA titers, positive anti-Sm antibodies and protinuria along with the skin and lung involvement. Al Riyami et al. (2003) described this disorder as a new variant of urticarial vasculitis syndrome as there was ANCA negative vasculitis, restrictive lung disease and low C4 levels with normal C3 and C1q and absent C1q inhibitor.

Laproscopic cholecystectomy performed in a pregnant lady (gravida 6, para 4, 1 abortion) with SLE, was reported by Machado (2004). She was diagnosed with SLE four years earlier when she presented with multiple joint pains, erythematous skin rash, face puffiness, Raynaud's phenomenon and intermittent thrombocytopenia, and the diagnosis was confirmed by positive anti-nuclear antibodies (1:640), low compliments C3 and C4, positive ribonucleoprotein antibodies and negative antiphospholipid antibodies. She was then started on immunosuppressive therapy. At 26 weeks of gestation, she presented with right hypochondriac pain with tenderness and guarding in that region, along with fever, nausea and vomiting. Ultrasonography revealed an edematous thick-walled gallbladder with a solitary gallstone (1 cm), with normal common bile duct, and confirmed fetal viability. Management was initially conservative with intravenous antibiotics, but no improvement occurred within 48 hours. Laproscopic cholecystectomy (inflamed gallbladder with omental adhesions around the fundus was found) was carried out uneventfully and with minimal bleeding. She was discharged after two days on her regular treatment and was followed up in the outpatient clinic. She then delivered a healthy baby boy at 38 weeks of gestation, who was followed up for three years and was passing through normal milestones. Machado (2004) encouraged the use of laproscopic cholecystectomy in pregnant ladies because of its advantages, having lower incidence of wound complication, less need for postoperative narcotics, and reduced periods of immobilization. Machado (2004) also delineated the advantage of laproscopy in patients with immunosuppressive therapy as they would resume their immunotherapy within short period, as well as reduce the incidence of immunotherapy related wound complications.

Alnaqdy et al. (2004) reported an unusual case of SLE with negative ANA. This 31-year old Omani female had presented two years earlier with one month history of joint pain, especially in hands, feet and the hip joints, associated with early morning stiffness, which improved by the end of the day. She also had increased loss of hair and intolerance to sunlight exposure (eyes and skin). Examination was unremarkable and investigations revealed weakly positive ANA with a titer of 1:40, which was subsequently negative when repeated. On the other hand, an elevated level of anti dsDNA antibodies was detected, with a titer greater than 1:640, and levels of 970units/ml on ELISA. The serum IgM subclass of anticardiolipin antibodies was positive (27 MPL) while the IgG subclass was negative (15 MPL). Other tests were normal which included extracted ENA, complement components C3 and C4, full blood count, ESR, urine microscopy, urea and electrolytes, liver function tests, echocardiogram and bone densitometry analysis. A diagnosis of SLE with predominantly musculoskeletal manifestation was made and the patient was managed successfully with hydrochloroquine (200 mg twice daily) and prednisolone to a maintenance dose of 5 mg per day. Throughout the follow up period the ANA remained negative while the anti dsDNA continued to be significantly high. [AlNaqdy A, AlSiddiqui M, ElAgib E. Anti-nuclear antibody (ANA) negative systemic lupus erythematosus . Oman Med J. 2004; 19(1):4-6.]

Rao et al. (2004) described the case of a 27-year-old Omani lady who presented with a 3-year history of progressive skeletal muscle weakness and clinical and skeletal changes of amyotropic lateral sclerosis. Investigation revealed that the patient, in fact, had SLE. Rao et al. (2004) suggested that this association could explain the autoimmune pathogenesis of the disease.

Abdwani et al. (2013) undertook a retrospective hospital-based review of all pediatric cases of SLE in Oman over a 15-year period (1995-2010). Of the 104 children studied, the most came from the Sharqiya region (41%), followed by Batina (18%), Muscat (17%), and Dakhiliya (10%) regions. This finding was opposed to what would be expected based on population densities of these regions. The patients from Sharqiya region showed several features distinct from those in the rest of Oman. These patients included a significantly greater number of boys (42% vs. 15%), younger patients (10.3 years vs 16.5 years), earlier age of diagnosis (6.4 years vs 9.4 years), and more cases of familial SLE (58% vs. 33%). Abdwani et al. (2013) were unable to comment on whether these variations were due to environmental factors, cultural habits, or genetic differences. Consanguinity was observed in 43% of the cases; 80% of this being first-degree consanguinity.

Palestine

[See: Lebanon > Uthman et al., 2001].

Qatar

Hammoudeh and Siam (1995) described a female patient with abdominal pain and ascites, mesenteric lymphadenitis and peritoneal panniculitis. Initially her ANA was negative. The abdominal pain recurred again three years later and in between the two episodes she had had skin rash, alopecia, arthralgia, and positive Coombs' test-hemolytic anemia. Her ANA became positive a few years after the initial episode. In 2001, Hammoudeh et al. described a 22 year old female patient with SLE who went on to develop bilateral patellar tendon rupture within three months of diagnosis. The patient presented with arthritis, fever, lethargy, hair loss, and pruritic chest pain. She was found to have swellings of knuckles and PIPs of both knees. ESR was 52mm/h, ANA 1:5120 with a speckled pattern, raised Anti-DNA, weakly positive Anti-RNP and Anti-Sm. She was treated with corticosteroids. Three months later, she complained of weakness in her knees, and sustained a fall. She was found to have hematoma on both knees, and was unable to extend the knees. MRI revealed complete and partial rupture of the left and right patellar ligaments, respectively, both of which were surgically repaired. Her steroid dose was tapered gradually. Although the ligaments showed healing, she later spontaneously ruptured her right triceps tendon as well as the Achilles tendon. Both these ruptures occurred while she was on a small dose of prednisolone. Hammoudeh et al. (2001) could not comment on why some patients with SLE were more prone to tendon rupture.

Saudi Arabia

Hussain et al. (2005) described the clinical features of 54 patients with SLE in a retrospective study at a Makkah hospital. All except two of these patients were Saudis, while all, except one patient, were females. Mucocutaneous involvement was noticed in 35 patients; oral ulcers (23), photosensitivity (13), alopecia (16), malar rash (12), and discoid rash (3). Renal involvement was seen in 23 patients, and renal biopsy identified lupus nephritis in 13 patients. Neuropsychiatric lupus erythematosus (NPLE) was seen in 18 patients, seizures (11), and psychosis (9) being the main features. Musculoskeletal system involvement was noticed in 29 cases. Hematological disorders were seen in 36 cases, which included lymphopenia (24), leucopenia (17), raised ESR (17), thrombocytopenia (7), and hemolytic anemia (2). Co-morbidity and over-lapping syndromes recorded among the patients included secondary anti-phospholipid syndrome (10), fibromyalgia (6), hypothyroidism (5), hypertension (4), pulmonary tuberculosis (3), type 2 diabetes mellitus (3), Sjorgen's syndrome (1), rheumatoid arthritis (1), and bronchial asthma (1).

[Hussain WM, Karima TM, Fatani MI, Bukhari SZ. Systemic lupus erythematosus (SLE) in makkah, saudi arabia. Kuwait Med J. 2005; 37(4):267-70.]

Syria

[See: Lebanon > Uthman et al., 2001].

Tunisia

In 2004, Ayed et al. compared HLA-DRB1*, DQA1, DQB1* and C4 allotypes in 62 Tunisian patients with systemic lupus erythematosus and 100 matched controls. HLA-DRB1*0301, -DRB1*1501 and C4AQO alleles were increased in SLE patients, while the frequencies of HLA-DRB1*04 and DQB1*03 were decreased. C4A*QO and C4B*QO were increased in frequency in SLE patients compared to the controls, but only C4A null was significantly increased. Eleven of 17 SLE patients with the C4 null allele were HLA-DRB1*0301 positive.

United Arab Emirates

In 1995, Al-Attia and George studied 28 SLE patients (Arabs and Asians) in the UAE. The F:M ratio was markedly high; 27:1 in the group as a whole and 21:1 among Arabs. Local patients (Emiratis) developed the disease at an earlier age compared to their expatriate Arab compatriots. Arthropathy occurred in 86% and nephropathy in 43% of cases. Next in frequency were leucopenia, mucocutaneous manifestations and serositis An unusually high prevalence of anti ds (DNA) antibodies (92.5%) as compared to ANF (82.5%) was detected (P = NS). 

Al Dhanhani et al. 2017 reported on the age-adjusted prevalence of SLE in the United Arab Emirates. Based on data in 2012, the age standardized prevalence was 103 per 100,000 individuals (95% confidence interval 84.5-124.4), higher than previously reported, and higher relative to other Arab countries.  

 

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