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Table of Contents
CASE REPORT
Year : 2022  |  Volume : 15  |  Issue : 1  |  Page : 42-44

Behcets disease presenting as renal failure in a 5-year-old child from Bahrain: A case report and literature review of renal manifestations of Behcets disease


Department of Pediatrics, Salmaniya Medical Complex, Manama, Bahrain

Date of Submission31-May-2021
Date of Decision08-Dec-2021
Date of Acceptance12-Dec-2021
Date of Web Publication25-Mar-2022

Correspondence Address:
Deena Mohammed Almastoor
Department of Pediatrics, Salmaniya Medical Complex, Manama
Bahrain
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/hmj.hmj_30_21

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  Abstract 


Background: Behcet's disease (BD) is a chronic vasculitis that results in multisystem involvement including cardiovascular, gastrointestinal, musculoskeletal, and nervous system. BD is more commonly found in adults. Children account only for 15%-20% of cases. Generally, the renal manifestations of BD are not predominant and less reported. Case Presentation: A 5-year-old boy presented with renal failure, and he was noted to have multiple oral ulcers, cutaneous lesions, and arthritis. In view of the multisystem involvement, BD was highly suspected, and the diagnosis was confirmed by a positive pathergy test and the presence of the HLA-B52 gene. The patient was commenced on peritoneal dialysis and anti-inflammatory medications. Conclusion: The diagnosis of BD in children is challenging and requires a high index of suspicion. Especially if the patient is presenting with renal symptoms. Early recognition of the disease is crucial to prevent irreversible complications such as end-organ damage including renal failure.

Keywords: Behcet's disease, glomerulonephritis, renal failure


How to cite this article:
Almastoor DM, Aljazaf HA, Abdan LK, Albinzayed R. Behcets disease presenting as renal failure in a 5-year-old child from Bahrain: A case report and literature review of renal manifestations of Behcets disease. Hamdan Med J 2022;15:42-4

How to cite this URL:
Almastoor DM, Aljazaf HA, Abdan LK, Albinzayed R. Behcets disease presenting as renal failure in a 5-year-old child from Bahrain: A case report and literature review of renal manifestations of Behcets disease. Hamdan Med J [serial online] 2022 [cited 2022 May 28];15:42-4. Available from: http://www.hamdanjournal.org/text.asp?2022/15/1/42/341009




  Introduction Top


Behcet's disease (BD) was initially described by Hulusi Behçet, a dermatologist from Turkey as recurrent ulcers involving the oral cavity and genitalia along with uveitis. It is a chronic vasculitis that results in multisystem involvement including cardiovascular, gastrointestinal, musculoskeletal and nervous systems. It is more common in adults, only 15%–20% of cases develop in children.[1],[2] Generally, the renal manifestations of BD are not predominant and less reported.[3],[4] In this case report, we describe a 5-year-old boy from Bahrain with BD and renal failure.


  Case Report Top


A 5-year-old boy, known case of bronchial asthma presented with a history of reduced urine output, fatigue and pallor of 3-day duration. The history was significant for recurrent oral ulcers and one episode of gross haematuria. Family history was not significant. On examination, he was afebrile, high blood pressure 160/110 mmHg, conscious, alert, pale, oedematous and dyspnoeic with subcostal recession, had multiple oral ulcers. The physical examination of the respiratory system highlighted the presence of coarse crepitations in both lungs, while the cardiovascular examination documented the presence of grade two heart murmur, which was heard best along the left lower sternal border. Abdominal examination: Ascites, no organomegaly. Neurological examination was grossly intact. Musculoskeletal examination revealed swollen tender left elbow and wrist, tender shoulder with restricted range of movement. Skin: Painful erythematous papules in the right forearm.

Laboratory investigations showed the following

Laboratory investigations were done and shown on the table below.



Normal serum complement levels, normal ASO titer, negative anti–double-stranded DNA and anti-nuclear antibodies. Virus screening was negative. Urine analysis showed heavy proteinuria and hematuria. Renal sonography revealed small kidneys with loss of corticomedullary differentiation, Chest X-rays revealed pulmonary oedema and minimal pleural effusion bilaterally. Echocardiography showed left ventricular hypertrophy which might reflect long-standing hypertension. Pathergy test was positive.

Management

The initial management of our patient included peritoneal dialysis, correction of anemia, electrolytes imbalances and acidosis. In view of the multisystem involvement and the presence of oral ulcers, arthritis and cutaneous lesions, BD was highly suspected. This was later confirmed by a positive pathergy test and the identification of HLA-B52 gene. There was no evidence of uveitis. Topical and systemic steroids were used in addition to colchicine and aspirin prophylaxis. The oral ulcers, arthritis and skin lesions have responded to treatment and resolved. However, he is still on renal replacement therapy due to end-stage renal disease.


  Discussion Top


BD is classified as a systemic vasculitis, currently seen all over the world due to immigration and not only limited to the historical area around the Silk Road where it was originally described. Interestingly, the old geographical distribution shared a common genetic predisposition linked to the HLA-B51 gene. In recent years, several genetic studies have introduced new genetic associations with BD, both HLA and non-HLA related.[1] The acknowledged onset of adult BD is between the second and fourth decades of life; there are insufficient data about the paediatric onset of BD. Moreover, there are marked differences in the mean age of onset stated in different cohorts; in general, it ranges between 4.9 and 12.3 years.[5] The diagnosis is challenging due to the non-specific presentation and the absence of a specific confirmatory test. Significant delay in diagnosis is reported worldwide; 3 years is the approximate time from the onset of symptoms to diagnosis.[5],[6] This delay can cause irreversible complications and end-organ damage. Many classifications have been proposed to help the physicians reach the diagnosis, although in most cases, the expertise of the physician is crucial to make the provisional diagnosis.[7] In Bahrain, the exact prevalence of paediatric BD is unknown. There were nine cases reported by Al Mosawi et al. in 2012. The most common manifestations were oral ulcers, followed by cutaneous involvement and musculoskeletal manifestations. However, none of them had renal manifestations.[8] In the United Kingdom, Nanthapisal et al. reported their largest case series of children with BD and showed a distinctive clinical phenotype in their population with less ocular, cutaneous and vascular involvement, Gastrointestinal and neurological involvement was more frequent compared to other cohorts. However, none of their cases had renal manifestations.[6] The reported incidence of renal manifestations in BD varies widely between 1% and 29%. It includes a broad clinical spectrum ranging from asymptomatic proteinuria and or microscopic haematuria to advanced kidney disease.[3],[4] BD affects the kidney in various ways, through vascular, glomerular and tubulointerstitial involvement. In addition to the possible side effects resulting from the drugs used in the treatment of the disease.[9],[10] Akpolat et al. reported the age range of patients with BD and glomerulonephritis (GN) varied between 13 and 70 years. Eight years is the mean time interval from the diagnosis until the occurrence of GN. The most common histopathological glomerular lesions are proliferative GN, crescentic GN and immunoglobulin A nephritis.[10] Secondary amyloidosis is reported to be the most frequent renal manifestation associated with vascular involvement and poorly controlled inflammation. Its incidence has reduced significantly due to the improvement in the management and better control of the disease activity; however, reactive amyloidosis is still considered a significant element in BD supporting the fact of being an autoinflammatory disease.[10] The renal vascular involvement includes renal artery stenosis, renal artery aneurysm and renal venous thrombosis. Renal failure in BD is usually secondary to long-standing GN and amyloidosis. Moreover, renal failure can be induced as a side effect of nephrotoxic medications used in the treatments such as cyclosporine, cyclophosphamide and high doses of NSAIDs.[9],[10],[11] Treatment and management: There is a lack of general consensus for the management of paediatric BD. EULAR adult recommendations are generally followed worldwide.[12] The main aim of the treatment is to control the disease activity, to suppress the inflammation and to prevent end-organ damage. The conventional treatment includes corticosteroids in addition to other immunosuppressants and anti-inflammatory drugs. Marked advancement in the management of the disease has been made by the introduction of new biological therapies. Multidisciplinary approach is essential for best outcome.[12],[13]


  Conclusion Top


The diagnosis of Behcet disease in children is challenging and requires a high index of suspicion. Early recognition of the disease is crucial to prevent irreversible complications such as end-organ damage including renal failure.

Declaration of patient consent

The authors certify that an appropriate parental consent form has been obtained with permission to use images and clinical information to be reported for the journal. The parent understands that the name of the child and theirs will not be published and due efforts will be made to conceal the identity.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Yildiz M, Haslak F, Adrovic A, Sahin S, Koker O, Barut K, et al. Pediatric Behçet's disease. Front Med 2021;8:627192.  Back to cited text no. 1
    
2.
Yıldız M, Köker O, Adrovic A, Şahin S, Barut K, Kasapçopur Ö. Pediatric Behçet's disease – Clinical aspects and current concepts. Eur J Rheumatol 2019;7 Suppl 1:1-10.  Back to cited text no. 2
    
3.
Akpolat T, Dilek M, Aksu K, Keser G, Toprak O, Cirit M, et al. Renal Behçet's disease: An update. Semin Arthritis Rheum 2008;38:241-8.  Back to cited text no. 3
    
4.
Cho SB, Kim J, Kang SW, Yoo TH, Zheng Z, Cho S, et al. Renal manifestations in 2007 Korean patients with Behçet's disease. Yonsei Med J 2013;54:189-96.  Back to cited text no. 4
    
5.
Pain CE. Juvenile-onset Behçet's syndrome and mimics. Clin Immunol 2020;214:108381.  Back to cited text no. 5
    
6.
Nanthapisal S, Klein NJ, Ambrose N, Eleftheriou D, Brogan PA. Paediatric Behçet's disease: A UK tertiary centre experience. Clin Rheumatol 2016;35:2509-16.  Back to cited text no. 6
    
7.
Koné-Paut I. Behçet's disease in children, an overview. Pediatr Rheumatol Online J 2016;14:10.  Back to cited text no. 7
    
8.
Al Mosawi ZS, Madan W, Fareed E. Pediatric-onset Behçet disease in Bahrain: Report of nine cases and literature review. Arch Iran Med 2012;15:485-7.  Back to cited text no. 8
    
9.
Ozen S. The “other”z; vasculitis syndromes and kidney involvement. Pediatr Nephrol 2010;25:1633-9.  Back to cited text no. 9
    
10.
Akpolat T, Akkoyunlu M, Akpolat I, Dilek M, Odabas AR, Ozen S. Renal Behçet's disease: A cumulative analysis. Semin Arthritis Rheum 2002;31:317-37.  Back to cited text no. 10
    
11.
Akpolat T, Diri B, Oğuz Y, Yilmaz E, Yavuz M, Dilek M. Behçet's disease and renal failure. Nephrol Dial Transplant 2003;18:888-91.  Back to cited text no. 11
    
12.
Hatemi G, Christensen R, Bang D, Bodaghi B, Celik AF, Fortune F, et al. 2018 update of the EULAR recommendations for the management of Behçet's syndrome. Ann Rheum Dis 2018;77:808-18.  Back to cited text no. 12
    
13.
Costagliola G, Cappelli S, Consolini R. Behçet's disease in children: Diagnostic and management challenges. Ther Clin Risk Manag 2020;16:495-507.  Back to cited text no. 13
    




 

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