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 Table of Contents  
Year : 2022  |  Volume : 2  |  Issue : 2  |  Page : 58-63

Childhood-onset systemic lupus erythematosus presenting with features mimicking Kawasaki disease

1 Division of Rheumatology, Maternity and Children Hospital, Medina, Saudi Arabia
2 Division of Paediatrics, Maternity and Children Hospital, Medina, Saudi Arabia

Date of Submission15-Jan-2022
Date of Decision21-Feb-2022
Date of Acceptance26-Feb-2022
Date of Web Publication25-Jan-2023

Correspondence Address:
Dr. Mohammed Olfat
Division of Rheumatology, Maternity and Children Hospital, Prince Abdul Majeed Road, Medina
Saudi Arabia
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ara.ara_2_22

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Childhood-onset systemic lupus erythematosus (cSLE) may rarely present initially with features mimicking Kawasaki disease (KD). Here, we report a 10-year-old girl presenting with high-grade fever, generalized skin rash, conjunctivitis, cervical lymphadenopathy, dry fissured lips, strawberry tongue, polyarthritis, abdominal pain, and neck stiffness. She was initially diagnosed and treated as KD, and the diagnosis of cSLE was confirmed 4 weeks later when she presented with a malar rash, nasal ulcer, seizures, and polyarthritis. In addition, her antinuclear antibodies, anti-double-stranded DNA, and direct Coombs test were positive. Therefore, with the difficulties in differential diagnosis and the rarity of such presentation, children with KD who develop new or unexpected features need to be evaluated for the development of cSLE.

Keywords: Childhood, Kawasaki disease, systemic lupus erythematosus

How to cite this article:
Olfat M, Jokhdar A, Al-Rewaithy B, Alhejaily A. Childhood-onset systemic lupus erythematosus presenting with features mimicking Kawasaki disease. Ann Rheumatol Autoimmun 2022;2:58-63

How to cite this URL:
Olfat M, Jokhdar A, Al-Rewaithy B, Alhejaily A. Childhood-onset systemic lupus erythematosus presenting with features mimicking Kawasaki disease. Ann Rheumatol Autoimmun [serial online] 2022 [cited 2023 Feb 4];2:58-63. Available from: http://www.ara.ssr.com/text.asp?2022/2/2/58/367437

  Background Top

Childhood-onset systemic lupus erythematosus (cSLE) is a chronic autoimmune disease with a wide range of disease manifestations that can involve any organ system and lead to significant morbidity and mortality. In children, most patients diagnosed with cSLE fulfill at least four criteria of the Systemic Lupus International Collaborating Clinics (SLICC) classification criteria for systemic lupus erythematosus (SLE).[1] These criteria were designed for use in research studies, and the diagnosis of SLE should not be solely based on fulfilling these criteria.

Childhood SLE has a broad differential diagnosis and may mimic other inflammatory diseases, as they share similar characteristics. Diagnosing cSLE can be challenging, particularly when the initial symptoms are vague, and the malar rash is absent. Therefore, a high index of suspicion is crucial for the diagnosis when the patient presents with an unusual constellation of symptoms.

Kawasaki disease (KD) is a common systemic vasculitis in childhood characterized by high-grade fever, conjunctivitis, dry fissured lips, strawberry tongue (ST), unilateral cervical lymphadenopathy, skin rash, and hand and foot changes.[2]

To our knowledge, the association of KD and cSLE is very rare and only a few cases reported such association. Most reports described cases of cSLE presenting initially with features mimicking KD, and only one case reported an association of KD and cSLE. Furthermore, the prevalence of this association between the two diseases was not reported.[3],[4],[5],[6],[7],[8] This report is intended to familiarize physicians with this unusual presentation of cSLE and review the medical literature of SLE presenting with features mimicking KD.

Ethical approval

The case was selected from the Department of Pediatric Rheumatology in Medina Children Hospital, Saudi Arabia. The parents provided informed consent for publication of this case. This article does not contain any studies with human participants or animals.

  Case Report Top

A 10-year-old Afghani girl presented to our hospital with a 2-week history of high-grade fever. This was associated with generalized body rash, fatigue, polyarthritis involving the knees, ankles, wrists, and small joints of the hands, generalized abdominal pain, and neck stiffness. In addition, she had a history of on and off joint pains associated with significant morning stiffness for 1 month prior to admission. Her systemic review was unremarkable. Her grandmother is diagnosed with rheumatoid arthritis.

Physical examination revealed an unwell, dehydrated girl with a high-grade fever of 40°C, erythematous maculopapular rash over her trunk, nonpurulent conjunctivitis, ST, red edematous fissured lips, and anterior cervical and inguinal lymphadenopathy. There was polyarthritis of the knees, ankles, wrists, and Proximal interphalangeal joints (PIPs). She was noticed to have mild erythema over the cheeks (malar-like a rash) and upper eyelids, but there were no other signs suggestive of lupus. Other systemic examinations were unremarkable.

Laboratory investigations revealed mild anemia, hypoalbuminemia, and elevated erythrocyte sedimentation rate [Table 1].
Table 1: Laboratory findings at onset

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Her renal function, liver function, C-reactive protein (CRP), and all infectious workup including viral studies (including adenovirus, enterovirus, herpesvirus, influenza, cytomegalovirus, and Epstein–Barr virus), blood culture, and urine examination and culture were normal. Echocardiography (echo) and ultrasound abdomen were normal.

She was assessed and managed by a general pediatrician as having KD and treated with a single dose of intravenous immunoglobulin (IVIG) 2 g/kg and aspirin 80 mg/kg. She showed significant improvement and was discharged home on aspirin 81 mg once daily, and a re-evaluation visit was given within 6 weeks after discharge.

Four weeks post discharge, she presented to our emergency department with high-grade fever, lethargy, and severe generalized pruritic rash. Physical examination revealed an ill, febrile, dehydrated girl with pallor, cracked lips, extensive maculopapular rash over the trunk, face, and extremities, typical malar rash, nasal ulcer, palatal erythema, puffy eyes, alopecia, and polyarthritis. At the emergency room, she had two attacks of seizures. At that time, the rheumatology service was consulted to see the patient for suspicion of cSLE.

Laboratory investigations revealed hemolytic anemia, elevated inflammatory markers, normal CRP, renal azotemia, hypoalbuminemia, elevated transaminases, hypocomplementemia, positive antinuclear antibodies (ANAs), and positive anti-double-stranded DNA [Table 2].
Table 2: Laboratory findings 4 weeks after Kawasaki disease diagnosis

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Other extractable nuclear antigen antibodies, antineutrophil cytoplasmic antibodies, antiphospholipid antibodies, and coagulation profile were normal. Urine analysis showed trace of protein. Cerebrospinal fluid study was normal. Magnetic resonance imaging showed mild brain atrophic changes, mild dilatation of the ventricular system, cortical sulci, and basal cistern. Magnetic resonance angiography was normal.

She was diagnosed with cSLE and started on steroids, hydroxychloroquine, and mycophenolate mofetil. After 1 week of hospitalization, she showed significant clinical and laboratory improvement and was discharged on mycophenolate mofetil, prednisolone, hydroxychloroquine, and cholecalciferol. During her follow-up, she showed significant improvement and went into remission.

  Discussion Top

cSLE is a chronic inflammatory disease of unknown cause that can affect the skin, joints, heart, kidneys, lungs, nervous system, or other body organs. The presenting manifestations of cSLE in children are as diverse as in adults, and any organ system may be involved.[9] A patient is classified as having cSLE if the patient has at least four criteria of the SLICC classification criteria for SLE.[1]

KD is an acute systemic vasculitis of childhood characterized by fever, bilateral nonexudative conjunctivitis, changes of the lips and oral mucosa, changes of perineal and peripheral extremities, polymorphous rash, and cervical lymphadenopathy. In children, the diagnosis of KD is according to the criteria established by the European League against Rheumatism/Paediatric Rheumatology European Society consensus criteria.[2]

The clinical presentation of KD varies over time, and the course of the disease may differ.[3] Some KD patients may develop other autoimmune disorders like cSLE.[3] On the other hand, cSLE is a great mimicker and may present with features of systemic vasculitis like KD.[4],[5],[6],[7],[8] However, the frequency of this association of the two diseases is rare. Moreover, there are few case reports of SLE who initially presented with KD features.

Here, we reported a rare case of cSLE who was initially diagnosed as having KD. Our patient presented initially with features mimicking KD. She was treated initially as KD for which IVIG and aspirin were administered. She was discharged home in good condition but presented with classical features of cSLE 4 weeks later. Our literature review encompassed a thorough search of the electronic database of PubMed and Embase from 1966 to the present for children <18 years of age who were diagnosed with cSLE presenting at the onset with features mimicking KD. Only English articles were reviewed.

Our literature search revealed only seven pediatric cases worldwide [Table 3], confirming the rarity of such presentation of cSLE. In some reported patients, little information provided.
Table 3: Childhood-onset systemic lupus erythematosus presenting with features mimicking Kawasaki disease

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The first reported case was a 5-year-old girl who developed cSLE 3.5 years after having had KD and was found to be homozygous at both class I and class II Major histocompatibility complex (MHC) loci.[3] Diniz reported another two cases of cSLE who had features of KD. One was a 13-year-old girl who presented fulfilling all six KD criteria. Her echo showed pericardial effusion, dilatation, and tortuosity of the right and left coronaries, and her symptoms improved promptly after treatment with IVIG. However, cSLE diagnosis was established a few days later. The other patient was a 4-year-old girl who had also met all six KD criteria, and cSLE diagnosis was made 1 year later.[4] The fourth case reported was a 6-year-old girl who was treated with two courses of IVIG for refractory incomplete KD. A few days later, she was readmitted with low-grade fever, autoimmune hemolytic anemia (AIHA), and arthritis, and her investigation confirmed the diagnosis of cSLE. Furthermore, her echo showed coronary dilatation.[5] In 2017, Jindal et al. reported a 5-year-old boy who had features of KD treated with IVIG and aspirin but simultaneously had features of cSLE treated successfully with prednisolone. This patient had peeling and Beau's line, but his coronaries were normal.[6] The sixth reported case was a 13-year-old boy who had features of incomplete KD with coronary dilatation and aortic regurgitation and simultaneously had features of cSLE. His blood work confirmed cSLE diagnosis, and he was treated with intravenous methylprednisolone (IVMP).[7] In 2020, Saez-de-Ocariz et al. reported a 16-year-old boy who had features of KD treated with IVIG and aspirin but simultaneously had features of cSLE treated successfully with IVMP, methotrexate, and hydroxychloroquine.[8]

Looking at the data from our patient and other reported cases, the mean age of presentation was 9 (4–16) years. Females were affected more than males. All patients had a fever, rash, bilateral nonpurulent conjunctivitis, and cervical lymphadenopathy at initial presentation. Only four patients had polyarthritis of small and large joints. Although coronary arteritis was seen in three patients, our patient had normal coronaries. However, other features of KD, such as oral changes, peeling, and Beau's line, were less frequently seen.[3],[4],[6] Only three patients had cSLE features simultaneously at the initial presentation. On the other hand, five patients, including our patient, developed cSLE features after discharge from the hospital and were diagnosed and treated as KD with IVIG and/or aspirin. The time from the initial presentation with KD features to the final diagnosis of cSLE was variable ranging from a few days to a few years (few days: 3.5 years).

While coronary arteritis is commonly the predominant complication in KD, few case reports have previously documented coronary arteritis as a presenting feature of cSLE.[5],[10],[11] In our literature review, coronary arteritis was a significant finding. Therefore, it would be reasonable to investigate whether cSLE patients have coronary arteritis at the onset to assess the overall risk as well as to determine the optimal treatment of their cSLE. Thus, it might be useful to include echo in the initial cSLE workup.

Almost all patients, including our patient, were noticed to have hypocomplementemia and positive ANA and anti- anti-double-stranded DNA. Therefore, for early diagnosis of cSLE, we suggest that these investigations should be requested in any patient with suspected KD who presents after the age of 4 years with unexpected features or any adolescent presenting with persistent high-grade fever, hemolytic anemia, and rash.

In our patient, the diagnosis of cSLE was delayed at her initial presentation because she had features of KD. n addition, the findings of normal white blood cell (WBC) and platelet count at initial presentation could have been suggestions that this was an unusual finding in KD as one usually expects high WBC and platelet counts in KD. Furthermore, the findings of AIHA and elevated CRP in our patient and the reported cases at the time of cSLE diagnosis suggest areas to explore with a large cohort study.

Our patient showed significant improvement with a single dose of IVIG 2 g/kg and aspirin 80 mg/kg. She was discharged home after 72 h in good general condition and given aspirin 81 mg once daily for 6 weeks. IVIG is the standard treatment of KD and may be used in certain cases of cSLE. Therefore, in our patient and other patients reported, we think that IVIG may have initially treated those patients with cSLE presenting with features of KD. Moreover, the classical features of cSLE appeared later on when the effect of IVIG is not enough to control the cSLE disease activity. On the other hand, since positive direct Coombs test (DCT) at the second presentation of our patient could have been related to prior exposure to IVIG, we recommend requesting DCT before starting IVIG in such a situation.

All patients reported including our patient presented initially with features suggesting the diagnosis of KD, but simultaneously or later on, their clinical features and blood work confirmed the diagnosis of cSLE. The question is whether these patients originally had KD and associated cSLE or primarily cSLE patients who presented with features of KD. These observations indicate that the overlap of KD and cSLE is extremely rare; therefore, most likely, these patients are primarily cases of cSLE.

  Conclusion Top

Knowing the wide heterogeneity of lupus manifestations, cSLE may present initially with features of KD. Therefore, such a group of children must be closely observed, followed up, and evaluated for cSLE. Furthermore, we suggest that ANA, complement levels, and echo should be requested for the differential diagnosis of fever and rash in children, especially when KD is suspected in patients over the age of 5 years. A large cohort study to assess the frequency of cSLE presenting initially with features of KD is required.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient's parents have given consent for images and other clinical information to be reported in the journal. The patient's parents understand that the name and initial will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Petri M, Orbai AM, Alarcón GS, Gordon C, Merrill JT, Fortin PR, et al. Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus. Arthritis Rheum 2012;64:2677-86.  Back to cited text no. 1
Ozen S, Ruperto N, Dillon MJ, Bagga A, Barron K, Davin JC, et al. EULAR/PReS endorsed consensus criteria for the classification of childhood vasculitides. Ann Rheum Dis 2006;65:936-41.  Back to cited text no. 2
Laxer RM, Cameron BJ, Silverman ED. Occurrence of Kawasaki disease and systemic lupus erythematosus in a single patient. J Rheumatol 1988;15:515-6.  Back to cited text no. 3
Diniz JC, Almeida RT, Aikawa NE, Sallum AM, Sakane PT, Silva CA. Kawasaki disease and juvenile systemic lupus erythematosus. Lupus 2012;21:89-92.  Back to cited text no. 4
Agarwal A, Biglarian S, Lim-Stavros S, Votava-Smith JK, Ramanathan A. Pediatric systemic lupus erythematosus presenting with coronary arteritis: A case series and review of the literature. Semin Arthritis Rheum 2015;45:42-7.  Back to cited text no. 5
Jindal A, Suri D, Gupta A, Rawat A, Singh S. 135 Skin Peeling and Irritability in a Young Boy with Systemic Lupus Erythematosus – Is there an Overlap with Kawasaki Disease? In: LUPUS 2017 & ACA 2017 (12th International Congress on SLE & 7TH Asian Congress on Autoimmunity), Melbourne, Australia; March 26-29, 2017.  Back to cited text no. 6
Zhang H, Zhang L, Guo N. Pediatric-onset systemic lupus erythematosus with coronary artery dilation: A case report. Medicine (Baltimore) 2020;99:e18946.  Back to cited text no. 7
Saez-de-Ocariz M, Pecero-Hidalgo MJ, Rivas-Larrauri F, García-Domínguez M, Venegas-Montoya E, Garrido-García M, et al. A teenager with rash and fever: Juvenile systemic lupus erythematosus or kawasaki disease? Front Pediatr 2020;8:149.  Back to cited text no. 8
Stichweh D, Arce E, Pascual V. Update on pediatric systemic lupus erythematosus. Curr Opin Rheumatol 2004;16:577-87.  Back to cited text no. 9
Mehta PK, Samady H, Vassiliades TA, Book WM. Acute coronary syndrome as a first presentation of systemic lupus erythematosus in a teenager: Revascularization by hybrid coronary artery bypass graft surgery and percutaneous coronary intervention: Case report. Pediatr Cardiol 2008;29:957-61.  Back to cited text no. 10
Shen CC, Chung HT, Huang YL, Yeh KW, Huang JL. Coronary artery dilation among patients with paediatric-onset systemic lupus erythematosus. Scand J Rheumatol 2012;41:458-65.  Back to cited text no. 11


  [Table 1], [Table 2], [Table 3]


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