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 Table of Contents  
Year : 2023  |  Volume : 10  |  Issue : 3  |  Page : 115-117

A rare presentation of Rickettsia disease with Kawasaki syndrome: A case report

1 Department of Pediatric, Pediatric Intensive Care Unit, Jehangir Hospital, Pune, Maharashtra, India
2 Department of Pediatrics, Siddhivinayak Hospital, Ahmednagar, Maharashtra, India

Date of Submission08-Feb-2023
Date of Decision26-Mar-2023
Date of Acceptance28-Mar-2023
Date of Web Publication19-May-2023

Correspondence Address:
Dr. Sagar Sharad Lad
Pediatric Intensive Care Unit, Jehangir Hospital, Pune - 411 006, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jpcc.jpcc_9_23

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Rickettsia is a common zoonotic disease causing fever, malaise, rash, and eruption changing to eschar with lymphadenopathy. Very few cases of Rickettsia disease with Kawasaki syndrome (KS) have been reported. We report a case of a 4-year-old girl with rickettsial disease presented with acute renal failure, shock, and features of KS. She was treated successfully with doxycycline, ceftriaxone, and intravenous immunoglobulins along with supportive management.

Keywords: Intravenous immunoglobulin, Kawasaki syndrome, myocarditis, Rickettsia

How to cite this article:
Sunthwal S, Lad SS, Malpani A, Bangar R, Jindal A. A rare presentation of Rickettsia disease with Kawasaki syndrome: A case report. J Pediatr Crit Care 2023;10:115-7

How to cite this URL:
Sunthwal S, Lad SS, Malpani A, Bangar R, Jindal A. A rare presentation of Rickettsia disease with Kawasaki syndrome: A case report. J Pediatr Crit Care [serial online] 2023 [cited 2023 Jun 2];10:115-7. Available from: http://www.jpcc.org.in/text.asp?2023/10/3/115/377438

  Introduction Top

Rickettsia is a common zoonotic disease causing fever, malaise, rash, and eruption changing to eschar with lymphadenopathy. Eschar is found in 7%–75% and complication usually develops after 1st week of illness. Rickettsial complication includes pneumonitis, meningoencephalitis, myocarditis, cardiogenic shock, and renal failure. The case fatality rate of rickettsial myocarditis is 7%–11%.[1] Very few cases of rickettsial disease with Kawasaki syndrome (KS) have been reported. We present a case of a 4-year-old girl with rickettsial disease with acute renal failure, shock, and features of KS. This case was presented before the COVID-19 pandemic.

  Case Report Top

A 4-year-old girl presented with a history of fever for 2 days, associated with vomiting, and rash all over her body with decreased urine output. On examination, she was febrile (102°F), pulse rate of 152 beats/min (tachycardia), respiratory rate of 46 breaths/min (tachypnea), blood pressure of 102/68 mmHg in the right arm supine position, peripheral pulses of low volume, capillary refill time was prolonged, and extremities were cold. She was in a state of compensated septic shock. She had maculopapular rash over the trunk, eschar over the chin, significant unilateral cervical lymph node palpable, nonexudative conjunctivitis, and anasarca. She also had significant hepatomegaly (liver span: 12.5 cm) and no splenomegaly, and the chest was clear. Investigation showed hemoglobin – 8.3 mg/dl, total white cell count – 8090 cells/cumm (neutrophilic – 64%), platelet count – 83,000 cells/cumm, serum C-reactive protein – 254 mg/l, urea – 66.2 mg/dl, creatinine – 1.27 mg/dl, prothrombin time/international normalized ratio – 41.03 s/3.23, activated partial thromboplastin – 69.75 s, cardiac markers creatine phosphokinase-MB – 58 U/l, pro-B-type natriuretic peptide – 6183 pg/ml, troponin T – 27.5 ng/ml, serum ferritin – 225 ng/ml, liver function test normal, and dengue NS1 antigen and dengue IgG IgM – negative. Urine and stool examinations were normal, and blood and urine cultures had no growth. She needed respiratory support with a humidified high-flow nasal cannula, inotropic support, doxycycline, and ceftriaxone. Chest X-ray showed cardiomegaly. Given raised cardiac markers and persistent tachycardia, two-dimensional echocardiography (2D-ECHO) was done suggestive of mild left ventricular dysfunction with a left ventricular ejection fraction of 50% with dilated coronaries with a Z-score of more than 3. She was given intravenous immunoglobulin (IVIG) (2 g/kg). Rickettsial polymerase chain reaction was positive and Weil–Felix was positive for OXK (1:80). Scrub typhus IgM was negative. Slowly, she was stabilized her fever subsided, and inotropic support was weaned. Repeat 2D-ECHO after 1 month was normal study.

  Discussion Top

Rickettsia infection is an acute febrile, arthropod-borne disease caused by obligate intracellular bacteria. Myocarditis and pericarditis are rare complications of rickettsial infection that occur in disseminated conditions. The case fatality rate of rickettsial infection is 3%–7%.[2]

The major target of rickettsiae is the endothelium lining of the small- and medium-sized blood vessels. Invasion and injury to endothelial cells alter vascular permeability causing platelet and fibrinolytic system changes, release of acute phase response proteins, activation of innate and acquired immune system, all leading to vascular inflammation termed “as rickettsial vasculitis,” is reflected in the clinical features of all rickettsial diseases.[3],[4] KS is systemic inflammatory disorder manifesting as vasculitis with predilection of coronary arteries. Hence, as our case was fulfilling the criteria of it. Dionne and Dahdah reported that evidence of myocarditis can be found in all patients with KS on histology specimens, but only minority of patients are clinically symptomatic.[5]

Coronary artery (CA) aneurysms and myocarditis are the common cardiovascular complication of KS. From histopathology standpoint, KS-induced myocarditis is more prevalent than CA aneurysm and found in all KS patients during the acute phase irrespective of CA aneurysm. Myocarditis is contributed to the early cause of death in patient with KS.[5]

Yoshikawa et al. in 2006 from Japan reported four cases of KS complicated with myocarditis. Of these four cases, three were older than 6 years of age and two cases were complicated with encephalopathy. All four patients developed CA abnormality during convalescence. There was one case with left ventricular dysfunction because of severe inflammation before IVIG administration. These all cases needed IVIG and catecholamine treatment.[6]

A case similar to ours was reported by Cascio et al. in 2011 in Italy, A case of a 3-year-old boy with features of atypical KS with IgM and IgG titers against Rickettsia Conorii 1:320 and 1:640, respectively, with 2D-ECHO showing dilated right coronary with a diameter of 2.8 mm responded to IVIG. A repeat echocardiogram after 6 weeks was a normal study.[7] A case report was described by van Doorn et al. in 2006 of a 3-year-old girl with KS serologically suggestive of murine typhus, a flea-borne rickettsiosis, who responded to IVIG follow-up echocardiography 1 week after showed dilated left CA.[8]

Bal and Kairys in 2009 reported a case of a 4-year-old girl with KS and serology confirmed of Rocky Mountain spotted fever, who responded well to IVIG and aspirin. On follow-up, her echocardiogram was a normal study.[9] There are two cases of myocarditis due to R. conorii reported by Mansour et al.[10] Salvi et al. reported an adult case of myocarditis where endomyocardial biopsy was done for diagnosis of rickettsial infection.[11]

Our patient had left ventricular dysfunction with coronaries dilatation which responded well with doxycycline and IVIG. On follow-up, the patient doing well and a follow-up 2D-ECHO is suggestive of a normal study. The main aim to report this case is to make aware of possible cardiac involvement with coronary involvement in patients with rickettsia features suggestive of KS. Close monitoring and early intervention and appropriate treatment are essential to reduce mortality in such cases.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient's legal guardian has given the consent for images and other clinical information to be reported in the journal. The patient's legal guardian understands that name and initials 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

Colomba C, Siracusa L, Trizzino M, Gioè C, Giammanco A, Cascio A. Myocarditis in Mediterranean spotted fever: A case report and a review of the literature. JMM Case Rep 2016;3:e005039.  Back to cited text no. 1
Bellini C, Monti M, Potin M, Dalle Ave A, Bille J, Greub G. Cardiac involvement in a patient with clinical and serological evidence of African tick-bite fever. BMC Infect Dis 2005;5:90.  Back to cited text no. 2
Amaro M, Bacellar F, França A. Report of eight cases of fatal and severe Mediterranean spotted fever in Portugal. Ann N Y Acad Sci 2003;990:331-43.  Back to cited text no. 3
Paddock CD, Denison AM, Dryden MW, Noden BH, Lash RR, Abdelghani SS, et al. High prevalence of “Candidatus Rickettsia andeanae” and apparent exclusion of Rickettsia parkeri in adult Amblyomma maculatum (Acari: Ixodidae) from Kansas and Oklahoma. Ticks Tick Borne Dis 2015;6:297-302.  Back to cited text no. 4
Dionne A, Dahdah N. Myocarditis and Kawasaki disease. Int J Rheum Dis 2018;21:45-9. doi: 10.1111/1756-185X.13219. Epub 2017 Nov 3. PMID: 29105303.  Back to cited text no. 5
Yoshikawa H, Nomura Y, Masuda K, Hazeki D, Yotsumoto K, Arata M, et al. Four cases of Kawasaki syndrome complicated with myocarditis. Circ J 2006;70:202-5.  Back to cited text no. 6
Cascio A, Maggio MC, Cardella F, Zangara V, Accomando S, Costa A, et al. Coronary involvement in Mediterranean spotted fever. New Microbiol 2011;34:421-4.  Back to cited text no. 7
van Doorn HR, Lo-A-Njoe SM, Ottenkamp J, Pajkrt D. Widened coronary arteries in a feverish child. Pediatr Cardiol 2006;27:515-8.  Back to cited text no. 8
Bal AK, Kairys SW. Kawasaki disease following Rocky Mountain spotted fever: A case report. J Med Case Rep 2009;3:7320.  Back to cited text no. 9
Mansour NB, Barakett N, Hajlaoui N, Haggui A, Filali T, Dahmen R, et al. Acute myocarditis complicating Mediterranean spotted fever. A case report Ann Cardiol Angeiol (Paris) 2014;63:55-7. French. doi: 10.1016/j.ancard.2011.05.003. Epub 2011 May 25. PMID: 21664598.  Back to cited text no. 10
Salvi A, Della Grazia E, Silvestri F, Camerini F. Acute rickettsial myocarditis and advanced atrioventricular block: Diagnosis and treatment aided by endomyocardial biopsy. Int J Cardiol 1985;7:405-9.  Back to cited text no. 11


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