|Year : 2020 | Volume
| Issue : 2 | Page : 317-320
Systemic lupus erythematosus masquerading as disseminated tuberculosis: Case report in a Nigerian adolescent
Sylvester Sunday Edward, Perpetua Okwuchi Obiajunwa, Olusola Ayewale Ayeleke
Department of Pediatrics, Obafemi Awolowo University Teaching Hospitals Complex, Ile-Ife, Osun State, Nigeria
|Date of Submission||04-Feb-2020|
|Date of Decision||22-Feb-2020|
|Date of Acceptance||11-Mar-2020|
|Date of Web Publication||26-Jun-2020|
Dr. Sylvester Sunday Edward
Department of Pediatrics, Obafemi Awolowo University Teaching Hospitals Complex, Ile-Ife, Osun State
Source of Support: None, Conflict of Interest: None
Systemic lupus erythematosus (SLE) is a connective tissue disorder whose manifestations may mimic other common chronic diseases in children. In developing countries, its diagnosis is often delayed or missed leading to delay in instituting appropriate treatment and invariably high mortality. We report the case of A. B who was a 13-year-old girl referred from a peripheral hospital with chronic cough, weight loss, and dyspnea. She had signs of heart failure and developed depression as well as oliguria. The patient also had pleural effusion, but aspirate result was negative for Mycobacterium tuberculosis and cytology. She commenced antituberculous drugs and dexamethasone with other supportive care but died after 19 days on admission. Serum assay was positive for antinuclear antibody. SLE is a potential masquerader of chronic diseases such as tuberculosis. Delay in diagnosis and treatment is associated with poor outcome; hence, there is a need for high index of suspicion for early diagnosis with prompt initiation of appropriate treatment.
Keywords: Adolescent, systemic lupus erythematosus, tuberculosis
|How to cite this article:|
Edward SS, Obiajunwa PO, Ayeleke OA. Systemic lupus erythematosus masquerading as disseminated tuberculosis: Case report in a Nigerian adolescent. Niger J Med 2020;29:317-20
|How to cite this URL:|
Edward SS, Obiajunwa PO, Ayeleke OA. Systemic lupus erythematosus masquerading as disseminated tuberculosis: Case report in a Nigerian adolescent. Niger J Med [serial online] 2020 [cited 2020 Sep 20];29:317-20. Available from: http://www.njmonline.org/text.asp?2020/29/2/317/287920
| Introduction|| |
Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by multisystem inflammation of blood vessels and connective tissue. The specific cause of SLE is unknown, but multiple factors are associated with the development of the disease, namely genetic, ethnic, hormonal, and environmental factors.
More than 90% of cases of SLE occur in female, usually starting at childbearing age. The male: female ratio before puberty is 1:3, but after puberty, it increases to 1:9. The manifestations of SLE are mediated by circulating immune complexes in various tissues or the direct effects of antibodies on cell components. The new classification by the European League Against Rheumatism (EULAR) and the American College of Rheumatology (ACR) requires an antinuclear antibody (ANA) titer of at least 1:80 or an equivalent positive test in addition to at least one clinical criterion and 10 or more points as diagnostic criteria for SLE.
The course of the disease is milder with higher survival rate in persons with isolated skin and musculoskeletal involvement than in those with renal disease and central nervous system (CNS) disease., SLE carries a highly variable prognosis in different patients; the natural history of the disease ranges from relatively benign disease to rapidly progressive and even fatal disease. Delay in diagnosis as well as presence of renal or CNS disease is associated with high mortality.
| Case Report|| |
A. B was a 13-year-old girl who was referred to our facility from a private teaching hospital with 4-month history of intermittent cough and fever. She also had progressive weight loss with dyspnea, leg swelling, and chest pain. The results of the investigations from the referring center are shown in [Table 1]. She had pericardiocentesis that yielded a culture-negative effluent; she was being managed for pulmonary tuberculosis with effusions and was commenced on antituberculous regimen with cefuroxime before she was referred to our facility.
At presentation, she was febrile and had clinical features of pleural effusion with congestive cardiac failure. She was assessed to have disseminated tuberculosis in heart failure, to rule out connective tissue disorder. Investigations showed elevated erythrocyte sedimentation rate, azotemia, leukocytosis with toxic granulation, proteinuria with glycosuria, as well as evidence of right-sided pleural with pericardial effusion [Table 2] and [Figure 1]. She recommenced the antituberculous drugs and also ceftriaxone, short course of frusemide, fresh frozen plasma, and dexamethasone; she had closed tube thoracostomy drainage [Figure 2] with subsequent intrapleural injection of streptokinase on the 13th day into admission. The patient developed irrational talk, headache, and features of depression on admission; she later became unconscious and oliguric shortly before demise. She was commenced on supportive management for acute kidney injury but died 6 h after, although the total duration of admission was 19 days. Her autopsy showed widespread fibrotic lesions in the lung, but no giant cell or granuloma was seen. She had positive ANA test, but the result was retrieved after demise.
|Figure 1: Chest X-ray showing right-sided homogenous opacity (pleural effusion)|
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|Figure 2: Chest X-ray after insertion of chest tube at site of pleural effusion with cardiomegaly|
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| Discussion|| |
Juvenile SLE is scarcely reported in sub-Saharan Africa despite the increasing reports of the adult form. Adelowo et al. posited that the juvenile type accounts for about one in five cases of SLE in a study from South West Nigeria. It accounts for 24.6% of pediatric rheumatic diseases in a study by Olaosebikan et al. in Lagos State, Nigeria. Delay in diagnosis from symptom onset to diagnosis ranges from 1 month to 3.3 years. This is due to its similar manifestation with other chronic tropical conditions such as tuberculosis.
Pediatric SLE usually presents in postpubescent females. The mean ages at lupus diagnosis are 11–12 years., There is a 24%–56% concordance rate in monozygotic twins, compared with a 2%–5% risk in dizygotic twins, suggesting genetic tendency. Chronic infections may induce anti-DNA antibodies or even lupus-like symptoms, and acute lupus flares often follow bacterial infections. Estrogen use in postmenopausal women appears to increase the risk of developing SLE. Photosensitivity is clearly a precipitant of skin disease since ultraviolet light stimulates autoantibody production.
Common manifestations of the disease include fatigue, fever, weight loss, lymphadenopathy, and hepatosplenomegaly., Fever may reflect active SLE or infection; patients with SLE are considered immunocompromised. Lupus patients may be functionally asplenic and may be at risk for encapsulated bacterial infections.
Renal disease manifests in about 50% of patients with SLE at presentation and is the greatest contributor to morbidity and mortality in pediatric SLE population., Neuropsychiatric disease occurs in up to two-thirds of patients; it is the second leading cause of morbidity and mortality in affected population. Headache is the most common CNS finding in SLE, occurring in 72% of children. Pericarditis is the most common cardiac manifestation, while pulmonary involvement may manifest as pleuritis, pleural effusion, and pulmonary hypertension,
ANA is found in 99% of patients with SLE, although it may be positive in mixed connective tissue disease and dermatomyositis. The anti-dsDNA, on the other hand, is very specific for SLE but less sensitive compared to ANA. It may be found in >75% of patients with pediatric SLE.
The new diagnostic EULAR/ACR criteria have sensitivity of 96.1% and specificity of 93.4%, compared with 82.8% sensitivity and 93.4% specificity of the 1997 ACR criteria [Table 3]. Positive ANA result is cardinal to making the diagnosis of SLE. In line with the new guideline, this index case met more than ten-point criteria to diagnose SLE.
|Table 3: 2019 European League Against Rheumatism/American College of Rheumatology Clinical and immunological domains and criteria for systemic lupus erythematosus|
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Treatment modality is medical and includes the use of hydroxychloroquine or nonsteroidal anti-inflammatory drugs, whereas severe disease is treated with steroids (methylprednisolone and prednisone) or steroid-sparing antirheumatic drugs (cyclophosphamide, methotrexate, azathioprine, belimumab, and rituximab).
The average 10-year survival rate exceeds 90%; The presence of renal or CNS manifestations is associated with poor prognosis. The high rate of misdiagnosis or delayed diagnosis is probably responsible for high mortality associated with lupus in resource-poor settings.,
| Conclusion|| |
SLE is rarely reported in Nigeria owing to frequent misdiagnosis. This is because of its similar manifestations with other chronic diseases that are endemic in Nigeria and hence the need for high index of suspicion for early diagnosis and prompt treatment so as to keep the mortality low.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]
[Table 1], [Table 2], [Table 3]