2014 — Streptococcal toxic shock syndrome, ICD-10 code A48.3 (emerging disease) — 12

–12  CDC. Compressed Mortality, 1999-2016. CDC Wonder Online Database, June 2017.

 

Streptococcal Toxic Shock Syndrome (STSS) (Streptococcus pyogenes): “Streptococcal toxic shock syndrome (STSS) is a severe illness associated with invasive or noninvasive group A streptococcal (Streptococcus pyogenes) infection. STSS may occur with infection at any site but most often occurs in association with infection of a cutaneous lesion [skin injury]. Signs of toxicity and a rapidly progressive clinical course are characteristic, and the case fatality rate may exceed 50%.” (CDC. Streptococcal Toxic Shock Syndrome (STSS) (Streptococcus pyogenes) 2020 Case Definition.)

 

Stevens on STSS: “Since the 1980s there has been a marked increase in the recognition and reporting of highly invasive group A streptococcal infections with or without necrotizing fasciitis[1] associated with shock and organ failure. Such dramatic cases have been defined as streptococcal toxic-shock syndrome. Strains of group A streptococci isolated from patients with invasive disease have been predominantly M types 1 and 3 that produce pyrogenic[2] exotoxin A or B or both….

 

“…many old pathogens have become major clinical problems because of increased virulence or antibiotic resistance (e.g., penicillin-resistant pneumococcus, multidrug-resistant Mycobacterium tuberculosis, methicillin-resistant Staphylococcus aureus, and vancomycin-resistant Enterococcus faecium). Arguably, group A Streptococcus (GAS) is the quintessence of an old organism that has become more virulent….

 

“Recently, severe invasive GAS [Group A Streptococcal] infections associated with shock and organ failure have been reported with increasing frequency, predominantly from North America and Europe…These infections have been termed streptococcal toxic-shock syndrome (TSS…)

 

“Persons of all ages are affected; most do not have predisposing underlying diseases. This is in sharp contrast to previous reports of GAS bacteremia, in which patients were either under 10 or over 60 years of age, and most had underlying conditions such as cancer, renal failure, leukemia, or severe burns or were receiving corticosteroids or other immunosuppressing drugs. The complications of current GAS infections are severe; bacteremia associated with aggressive soft tissue infection, shock, adult respiratory distress syndrome and renal failure are common; 30% to 70% of patients die in spite of aggressive modern treatments…

 

“The portal of entry of streptococci cannot be proven in at least half the cases and can only be presumed in many others. Patients with symptomatic pharyngitis rarely develop streptococcal TSS, though such cases have been reported, especially in the last year. Procedures such as suctionlipectomy, hysterectomy, vaginal delivery, bunionectomy [bunion surgery] and bone pinning[3] have provided a portal of entry in many cases (author’s unpublished observations). Most commonly, infection begins at a site of minor local trauma, which frequently does not result in a break in the skin. Numerous cases have developed within 24 to 72 hours of minor nonpenetrating trauma, resulting in hematoma, deep bruise to the calf, or even muscle strain. Virus infections, such as varicella [chickenpox] and influenza, have provided a portal in other cases. In some cases the use of nonsteroidal anti-inflammatory agents may have either masked the early symptoms or predisposed the patient to more severe streptococcal infection and shock. For the most part, these infections have occurred sporadically and have not been associated with clusters of cases or minor epidemics, though outbreaks of severe GAS-infections have occurred in closed environments such as nursing homes.

 

“Pain the most common initial symptom of streptococcal TSS is abrupt in onset and severe, and usually precedes tenderness or physical findings. The pain usually involves an extremity but may also mimic peritonitis,[4] pelvic inflammatory disease, pneumonia, acute myocardial infarction, or pericarditis.[5] Twenty percent of patients have an influenza-like syndrome characterized by fever, chills, myalgia [muscle pain], nausea, vomiting, and diarrhea. Fever is the most common early sign, although hypothermia may be present in patients with shock. Confusion is present in 55% of patients, and in some, coma or combativeness is manifest. Eighty percent of patients have clinical signs of soft tissue infection, such as localized swelling and erythema,[6] which in 70% of patients progressed to necrotizing fasciitis or myositis[7] and required surgical debridement,[8] fasciotomy[9] or amputation. An ominous sign is the progression of soft tissue swelling to the formation of vesicles, then bullae,[10] which appear violaceous[11] or bluish….” (Stevens. “Streptococcal Toxic-Shock Syndrome: Spectrum of Disease, Pathogenesis, and New Concepts in Treatment.” Emerging Infectious Diseases, Vol. 1, No. 3, July 1995.)

 

Sources

 

Centers for Disease Control and Prevention, National Center for Health Statistics. Compressed Mortality File 1999-2016. CDC Wonder Online Database, released June 2017. ICD-10 code A48.3, Toxic shock syndrome (2014). Accessed 11-1-2018 at: https://wonder.cdc.gov

 

Centers for Disease Control and Prevention. Streptococcal Toxic Shock Syndrome (STSS) (Streptococcus pyogenes) 2020 Case Definition. Accessed 11-1-2018 at: https://wwwn.cdc.gov/nndss/conditions/streptococcal-toxic-shock-syndrome/case-definition/2010/

 

Stevens, Dennis L. “Streptococcal Toxic-Shock Syndrome: Spectrum of Disease, Pathogenesis, and New Concepts in Treatment.” Emerging Infectious Diseases, Vol. 1, No. 3, July 1995. Accessed 11-1-2018 at: https://wwwnc.cdc.gov/eid/article/1/3/95-0301_article

[1] Rare bacterial infection that spreads quickly in the body and can cause death. (CDC)

[2] Producing or produced by fever.

[3] Medical procedure wherein metal screws, pins, rods or plates are used to hold a bone or bones in place.

[4] Inflammation of the peritoneum — a silk-like membrane that lines the abdominal wall and covers the organs within the abdomen. (Mayo Clinic. “Peritonitis.”

[5] Inflammation of the tissue surrounding the heart.

[6] Superficial reddening of the skin, usually in patches, as a result in injury or irritation causing dilation of the blood capillaries.

[7] Inflammation of the muscles one uses to move the body.

[8] Removal of damaged tissue or foreign objects from a wound.

[9] Surgical procedure where the fascia (thin sheath of fibrous tissue enclosing a muscle or organ.

[10] Bubble-like cavity filled with air or fluid.

[11] Of a violet color.