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 Table of Contents  
CASE REPORT
Year : 2020  |  Volume : 29  |  Issue : 2  |  Page : 317-320

Systemic lupus erythematosus masquerading as disseminated tuberculosis: Case report in a Nigerian adolescent


Department of Pediatrics, Obafemi Awolowo University Teaching Hospitals Complex, Ile-Ife, Osun State, Nigeria

Date of Submission04-Feb-2020
Date of Decision22-Feb-2020
Date of Acceptance11-Mar-2020
Date of Web Publication26-Jun-2020

Correspondence Address:
Dr. Sylvester Sunday Edward
Department of Pediatrics, Obafemi Awolowo University Teaching Hospitals Complex, Ile-Ife, Osun State
Nigeria
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/NJM.NJM_2_20

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  Abstract 


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 Jul 6];29:317-20. Available from: http://www.njmonline.org/text.asp?2020/29/2/317/287920




  Introduction Top


Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by multisystem inflammation of blood vessels and connective tissue.[1] 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.[2]

More than 90% of cases of SLE occur in female, usually starting at childbearing age.[3] The male: female ratio before puberty is 1:3, but after puberty, it increases to 1:9.[1] The manifestations of SLE are mediated by circulating immune complexes in various tissues or the direct effects of antibodies on cell components.[2] 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.[4]

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.[5],[6] 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.[7] Delay in diagnosis as well as presence of renal or CNS disease is associated with high mortality.


  Case Report Top


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.
Table 1: Tests and results from referring center

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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.
Table 2: Tests and results from our facility

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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 Top


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.[8] It accounts for 24.6% of pediatric rheumatic diseases in a study by Olaosebikan et al. in Lagos State, Nigeria.[9] Delay in diagnosis from symptom onset to diagnosis ranges from 1 month to 3.3 years.[10] This is due to its similar manifestation with other chronic tropical conditions such as tuberculosis.

Pediatric SLE usually presents in postpubescent females.[1] The mean ages at lupus diagnosis are 11–12 years.[1],[11] There is a 24%–56% concordance rate in monozygotic twins, compared with a 2%–5% risk in dizygotic twins, suggesting genetic tendency.[12] 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.[13]

Common manifestations of the disease include fatigue, fever, weight loss, lymphadenopathy, and hepatosplenomegaly.[1],[7] 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.[7]

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.[1],[7] Neuropsychiatric disease occurs in up to two-thirds of patients; it is the second leading cause of morbidity and mortality in affected population.[1] Headache is the most common CNS finding in SLE, occurring in 72% of children.[14] Pericarditis is the most common cardiac manifestation, while pulmonary involvement may manifest as pleuritis, pleural effusion, and pulmonary hypertension[1],[7]

ANA is found in 99% of patients with SLE, although it may be positive in mixed connective tissue disease and dermatomyositis.[1] 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.[1]

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[4] [Table 3]. Positive ANA result is cardinal to making the diagnosis of SLE.[4] 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[4]

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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).[7]

The average 10-year survival rate exceeds 90%;[6] 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.[8],[11]


  Conclusion Top


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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Weiss JE. Pediatric systemic lupus erythematosus: More than a positive antinuclear antibody. Pediatr Rev 2012;33:62-73.  Back to cited text no. 1
    
2.
Blank M, Shoenfeld Y, Perl A. Cross-talk of the environment with the host genome and the immune system through endogenous retroviruses in systemic lupus erythematosus. Lupus 2009;18:1136-43.  Back to cited text no. 2
    
3.
Rahman A, Isenberg DA. Systemic lupus erythematosus. N Engl J Med 2008;358:929-39.  Back to cited text no. 3
    
4.
Aringer M, Costenbader K, Daikh D, Brinks R, Mosca M, Ramsey-Goldman R, et al. 2019 European League Against Rheumatism/American College of Rheumatology classification criteria for systemic lupus erythematosus. Ann Rheum Dis 2019;78:1151-9.  Back to cited text no. 4
    
5.
Faurschou M, Dreyer L, Kamper AL, Starklint H, Jacobsen S. Long-term mortality and renal outcome in a cohort of 100 patients with lupus nephritis. Arthritis Care Res (Hoboken) 2010;62:873-80.  Back to cited text no. 5
    
6.
Kasitanon N, Magder LS, Petri M. Predictors of survival in systemic lupus erythematosus. Medicine (Baltimore) 2006;85:147-56.  Back to cited text no. 6
    
7.
Christie MB, Daniel M. Systemic Lupus Erythematosus (SLE). Medscape. Available from: https://emedicine.medscape.com/article/332244 overview. [Last updated on 2019 Oct 09; Last accessed on 2020 Jan 09].  Back to cited text no. 7
    
8.
Adelowo OO, Olaosebikan BH, Animashaun BA, Akintayo RO. Juvenile systemic lupus erythematosus in Nigeria. Lupus 2017;26:329-33.  Back to cited text no. 8
    
9.
Olaosebikan BH, Adelowo OO, Animashaun BA, Akintayo RO. Spectrum of paediatric rheumatic diseases in Nigeria. Pediatr Rheumatol Online J 2017;15:7.  Back to cited text no. 9
    
10.
Benseler SM, Silverman ED. Systemic lupus erythematosus. Pediatr Clin North Am 2005;52:443-67, vi.  Back to cited text no. 10
    
11.
Olowu W. Childhood-onset systemic lupus erythematosus. J Natl Med Assoc 2007;99:777-84.  Back to cited text no. 11
    
12.
Deng Y, Tsao BP. Genetic susceptibility to systemic lupus erythematosus in the genomic era. Nat Rev Rheumatol 2010;6:683-92.  Back to cited text no. 12
    
13.
Lehmann P, Hölzle E, Kind P, Goerz G, Plewig G. Experimental reproduction of skin lesions in lupus erythematosus by UVA and UVB radiation. J Am Acad Dermatol 1990;22:181-7.  Back to cited text no. 13
    
14.
Muscal E, Brey RL. Neurologic manifestations of systemic lupus erythematosus in children and adults. Neurol Clin 2010;28:61-73.  Back to cited text no. 14
    


    Figures

  [Figure 1], [Figure 2]
 
 
    Tables

  [Table 1], [Table 2], [Table 3]



 

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