CLINICAL BRIEF

Streptococcal Toxic Shock Syndrome

Vidya Krishna & Shuba Sankaranarayan &

Rajakumar Padur Sivaraman & Krithika Prabaharan

Received: 8 July 2013 /Accepted: 10 October 2013# Dr. K C Chaudhuri Foundation 2013

Abstract Streptococcal Toxic Shock syndrome (STSS) is aserious complication caused by exotoxins of Group A Strep-tococcus (GAS). It presents with fulminant shock and rash, israpidly progressive with Multi-Organ Dysfunction Syndrome(MODS) and requires aggressive therapy with fluids, antibi-otics and source control.

Keywords Streptococcal Toxic Shock Syndrome .

Group A Streptoccus .Multi Organ Dysfunction Syndrome .

Renal Replacement Therapy

Introduction

GAS infections can range from cellulitis to necrotizing fasci-itis, myositis, empyema, bacteremia and endocarditis. A fatalcomplication is Toxic- shock syndrome, an exotoxin mediatedillness presenting with rash, shock and multi-organ dysfunc-tion. Incidence is especially higher in North America andEurope [1]. The authors report two cases of StreptococcalToxic Shock syndrome (STSS) encountered in their settingand discuss their presentations, management and outcomes.

Case Reports

Case 1

A 5-y-old boy presented with fever for 3 d, cough, respiratorydistress and loose stools for 1 d. He was in compensated shock

with irritability, diffuse erythematous rash all over the bodyand severe mottling. He required large volume of fluid forresuscitation and inotropes and ventilation. Chest radiographshowed left sided pneumonia with effusion and inter-costaldrainage was done. Laboratory examination showed de-creased total leucocyte counts (1,750 cells/cu mm), elevatedrenal and liver parameters and altered coagulation profile.Toxic Shock syndrome was suspected due to the rapid course,rash and multi-organ dysfunction. Antibiotics and intravenousimmunoglobulin (IVIG) were given. Pleural fluid and bloodcultures grew Streptococcus pyogenes. He developed Rhab-domyolysis with myoglobinuria and grossly elevated CPK(2,38,400 u/L). Acute Kidney Injury (AKI) worsened andRenal Replacement Therapy (RRT) was started. He eventuallysuccumbed to the Multi-Organ Dysfunction syndrome(MODS) and coagulopathy.

Case 2

A 16-y-old girl, a known case of Juvenile Idiopathic Arthritison Etanercept, got admitted with fever and joint pain for 4 wk.She had vomiting and swelling of the right thigh since 2 dprior to admission. She had undergone branding for arthritis.She was in compensated shock with diffuse erythema over thebody, cellulitis of right thigh and arthritis right knee. Shedeveloped hypotension within 4 h and was resuscitated ap-propriately. She had elevated liver enzymes and renal param-eters. Fasciotomy done showed subcutaneous tissue necrosis.Blood cultures grew Streptococcus mitis. As she developedbrainstem dysfunction, IVIG and RRT were deferred. Shesuccumbed to the illness after 2 d.

Discussion

Toxic shock syndrome (TSS) is a multisystem disease causedby toxin producing strains of Staphylococcus/Streptococcus.

V. Krishna : S. Sankaranarayan (*) : R. P. Sivaraman :K. PrabaharanDepartment of Pediatrics, Sri Ramachandra Medical College andHospital, Porur, Chennai 600116, Indiae-mail: drshuba2005@yahoo.com

Indian J PediatrDOI 10.1007/s12098-013-1272-8

Streptococcal M types 1, 3, 12, and 28 have been the mostcommon isolates from patients with shock and multiorganfailure [1].

Group A streptococcus (e.g., Streptococcus pyogenes), anaerobic gram-positive coccus, enters the body via skin, usu-ally following trivial trauma or varicella [2]. GAS can causepharyngitis, soft tissue infections and bacteremia. It is morecommon in children under 10 y [3]. TSS usually presents withfever, chills, vomiting, loose stools, myalgia and diffuse mac-ular erythroderma. The patient has hypotension at presentationor develops it within 4– 8 h. Some degree of renal dysfunctionis known to precede shock [1].

Incidence may vary from 0.7 to 4.16/1,00,000 population[3]. Specific diagnostic criteria are listed below.

Diagnostic Criteria for Streptococcal TSS (CDC 2010) [4]

& Hypotension - Systolic blood pressure less than the fifthpercentile by age for children aged less than 16 y.

& Multi-organ involvement characterized by two or more ofthe following:

– Renal impairment: Creatinine greater than or equal totwice the upper limit of normal for age.

– Coagulopathy: Platelets less than or equal to 100,000/mm3

(less than or equal to 100×106/L) or disseminatedintravascular coagulation.

– Liver involvement: Alanine aminotransferase, Aspar-tate amino-transferase or total bilirubin levels greaterthan or equal to twice the upper limit of normal.

– Acute respiratory distress syndrome– A generalized erythematous macular rash that may

desquamate.– Soft-tissue necrosis, including necrotizing fasciitis or

myositis, or gangrene.

& Laboratory criteria for diagnosis– Isolation of group A Streptococcus from sterile site

Case Classification

Probable A case that meets the clinical case definition in theabsence of another identified etiology for the illness and withisolation of group A Streptococcus from a non-sterile site.

Confirmed A case that meets the clinical case definition andwith isolation of group A Streptococcus from a normallysterile site (e.g., blood or cerebrospinal fluid or less commonly,joint, pleural, or pericardial fluid).

Streptococcal TSS is caused by super-antigen toxin medi-ated injury [1]. Super antigens do not require processing byantigen dependent cells and cause non-specific binding ofMajor Histo-compatibility Complex (MHC) class II to T-cellreceptors resulting in polyclonal T-cell activation. The

resultant cytokine storm causes the multi-system involvement[1].

Treatment includes rapid resuscitation with fluids, vasoac-tive agents and treatment for MODS, including dialysis forrenal failure. Source control should be done early. Antibiotictherapy should include Clindamycin in addition to beta-lactams. Clindamycin’s action does not depend on inoculumsize, is a potent suppressor of bacterial toxin synthesis, facil-itates phagocytosis and has a longer post-antibiotic effect thanbeta-lactams. Penicillin can fail when microbiological load islarge [1, 3]. Although IVIG has been used early in Strepto-coccal TSS its role has not been firmly established [5]. RRThas been found to be useful by removing streptococcal exo-toxins and inflammatory mediators [6].

Mortality rates are 30–70 % despite adequate intensive care[7]. Differential diagnoses include Staphylococcal Toxic Shocksyndrome, Gram-negative septic shock and Severe Dengue.

In a multi-centric, retrospective study of children in Spainall patients developed shock and variable organ dysfunction.The median PICU stay was 7 d (0–41). 65.8 % of patientssurvived, 26.8 % with sequelae. The cause of death wasrefractory shock and multi-organ failure [8].

Toxic Shock syndrome requires early identification, aggres-sive management with fluids, antibiotics and source control.

Contributions All the authors were involved in case management. VKwrote the initial draft and shall act as the guarantor. KP helped in draftwriting. SS and RPS gave intellectual inputs in case management andedited the draft. All authors approved the final draft.

Conflict of Interest None.

Role of Funding Source None.

References

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4. Streptococcal Toxic Shock Syndrome (STSS). CDC Case definition2010. Available from:http://wwwn.cdc.gov/NNDSS/script/casedef.aspx?CondYrID=858&DatePub=1/1/2010%2012:00:00%20AM.Accessed on 06/08/2013.

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6. Wiles 3rd CE, Reynolds HN, Bar-Lavie Y. Flush resuscitation forgroup A streptococcus toxic shock : A possible role for continuousrenal resuscitation therapy and plasmapheresis. Md Med J. 1998;47:188–90.

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7. Demers B, Simor AE, Velland H, Schlievert PM, Byrne S, Jamieson F,et al. Severe invasive group A streptococcal infections in Ontario,Canada: 1987–1991. Clin Infect Dis. 1993;16:792–800. discussion801–2.

8. Rodríguez-Nuñez A, Dosil-Gallardo S, Jordan I. Clinical characteris-tics of children with group A streptococcal toxic shock syndromeadmitted to pediatric intensive care units. Eur J Pediatr. 2011;170:639–44.

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