Complications of streptococcal tonsillopharyngitis

INTRODUCTION — Pharyngitis caused by infection with Streptococcus pyogenes (also known as group A Streptococcus [GAS]) is usually a self-limited condition; symptoms in untreated patients typically last two to five days. Antimicrobial therapy reduces the duration and severity of symptoms by one to two days (when begun within 48 hours of illness) and prevents spread of infection [1-3]. (See "Treatment and prevention of streptococcal pharyngitis in adults and children".)

The other major goal of therapy is to reduce the risk of suppurative and nonsuppurative complications. The potential complications of GAS tonsillopharyngitis will be briefly reviewed here.

NONSUPPURATIVE COMPLICATIONS — The nonsuppurative complications of group A streptococcal (GAS) tonsillopharyngitis include:

●Acute rheumatic fever (ARF)

●Poststreptococcal reactive arthritis (PSRA)

●Scarlet fever

●Streptococcal toxic shock syndrome

●Acute glomerulonephritis

●Pediatric autoimmune neuropsychiatric disorder associated with group A streptococci (PANDAS)

Acute rheumatic fever — ARF is a delayed, nonsuppurative sequela of a pharyngeal infection with GAS. Following the initial pharyngitis, there is a latent period of two to three weeks before the first signs or symptoms of ARF appear [4]. The disease presents with various manifestations that may include arthritis, carditis, chorea, subcutaneous nodules, and erythema marginatum. (See "Acute rheumatic fever: Clinical manifestations and diagnosis".)

While there has been a dramatic decline in both the severity and mortality of ARF since the beginning of the 20^th century [5,6], there have been reports of its resurgence in the United States [7], reminding us that the disease remains a public health problem even in developed countries [8]. In addition, the disease continues essentially unabated in many of the resource-limited countries; estimates suggest there will be 10 to 20 million new cases per year in those countries in which two-thirds of the world's population lives. (See "Acute rheumatic fever: Epidemiology and pathogenesis".)

Poststreptococcal reactive arthritis — Poststreptococcal arthritis is a reactive arthritis with inflammation involving one or more joints that develops within a month following a pharyngeal GAS infection. It is discussed further separately. (See "Acute rheumatic fever: Clinical manifestations and diagnosis", section on 'Poststreptococcal reactive arthritis'.)

Scarlet fever — Scarlet fever (also known as "scarlatina") is a diffuse erythematous eruption that generally occurs in association with pharyngitis. Development of the scarlet fever rash requires prior exposure to S. pyogenes and occurs as a result of delayed-type skin reactivity to pyrogenic exotoxin (erythrogenic toxin, usually types A, B, or C) produced by the organism.

The rash of scarlet fever is a diffuse erythema that blanches with pressure, with numerous small (1 to 2 mm) papular elevations, giving a "sandpaper" quality to the skin (picture 1). It usually starts in the groin and armpits and is accompanied by circumoral pallor and a strawberry tongue (picture 2). Subsequently, the rash expands rapidly to cover the trunk, followed by the extremities (picture 3), and, ultimately, desquamates (picture 4); the palms and soles are usually spared. The rash is most marked in the skin folds of the inguinal, axillary, antecubital, and abdominal areas and about pressure points. It often exhibits a linear petechial character in the antecubital fossae and axillary folds, known as Pastia's lines (picture 5).

The diagnosis is established based on clinical manifestations. Apart from rapid strep testing and throat culture, there is no role for additional testing. (See "Group A streptococcal tonsillopharyngitis in children and adolescents: Clinical features and diagnosis", section on 'Diagnosis'.)

Scarlet fever with pharyngitis can predispose to acute rheumatic fever. The approach to treatment of scarlet fever is the same as that of streptococcal pharyngitis; no additional treatment is warranted for the skin rash. Children may return to school or daycare 24 hours after initiation of antibiotics. No additional monitoring for such patients is required. (See "Treatment and prevention of streptococcal pharyngitis in adults and children".)

Streptococcal toxic shock syndrome — Streptococcal toxic shock syndrome is a rare complication of streptococcal pharyngitis. Severe GAS infections associated with shock and organ failure have been reported with increasing frequency, predominantly from North America and Europe [9-11]. These infections have been termed streptococcal toxic shock syndrome (table 1).

The most common portals of entry for streptococcal infections are the skin, vagina, pharynx, and mucosa. However, the portal cannot be ascertained in 45 percent of cases [12]. (See "Invasive group A streptococcal infection and toxic shock syndrome: Epidemiology, clinical manifestations, and diagnosis".)

Acute glomerulonephritis — Poststreptococcal glomerulonephritis is induced by infection with specific nephritogenic strains of GAS (such as type 12 and type 49) [13-16]. This can occur in sporadic cases or during an epidemic. The incidence of clinically detectable glomerulonephritis in children infected during an epidemic is about 5 to 10 percent with pharyngitis and 25 percent with skin infections [13,14]. Younger children below the age of seven appear to be at highest risk.

The clinical presentation can vary from asymptomatic, microscopic hematuria to full-blown acute nephritic syndrome, characterized by red to brown urine, proteinuria (which can reach the nephrotic range), edema, hypertension, and acute renal failure [15,17,18]. The prognosis is generally favorable. (See "Poststreptococcal glomerulonephritis".)

PANDAS syndrome — Pediatric autoimmune neuropsychiatric disorder associated with group A streptococci (PANDAS) is a term used to describe a subset of children whose symptoms of obsessive compulsive disorder or tic disorders are exacerbated by GAS infection. This is discussed in detail separately. (See "PANDAS: Pediatric autoimmune neuropsychiatric disorder associated with group A streptococci".)

SUPPURATIVE COMPLICATIONS — The suppurative complications of group A streptococcal (GAS) tonsillopharyngitis include:

●Tonsillopharyngeal cellulitis or abscess

●Otitis media

●Sinusitis

●Necrotizing fasciitis (see "Necrotizing soft tissue infections")

●Streptococcal bacteremia, which is rare (see "Invasive group A streptococcal infections in children" and "Invasive group A streptococcal infection and toxic shock syndrome: Epidemiology, clinical manifestations, and diagnosis", section on 'Bacteremia')

●Meningitis or brain abscess, a rare complication resulting from direct extension of an ear or sinus infection or from bacteremic spread

●Jugular vein septic thrombophlebitis (see "Lemierre syndrome: Septic thrombophlebitis of the internal jugular vein", section on 'Microbiology')

Tonsillopharyngeal cellulitis or abscess — Cellulitis or an abscess can arise in the peritonsillar or retropharyngeal spaces secondary to streptococcal tonsillopharyngitis [19]. GAS are frequently recovered from such patients, although GAS are usually present in association with other oral flora rather than as the sole pathogen [20].

Otitis media — Otitis media is one of the two most common suppurative complications of streptococcal tonsillopharyngitis and occurs via direct extension of the organisms from the pharynx to the ear via the Eustachian tube. However, GAS accounts for less than 5 percent of all cases of acute otitis media (AOM) and occurs predominantly in children age five years and older. (See "Acute otitis media in children: Epidemiology, microbiology, and complications", section on 'Bacterial pathogens'.)

The peak age of otitis media is 6 to 24 months, whereas the peak age for streptococcal tonsillopharyngitis is 5 to 12 years old [21]. The clinical manifestations of AOM do not differ based upon the etiologic organism. Children usually present with fever, otalgia, irritability, anorexia, loose stools, and vomiting, whereas the most common symptom in adults is ear pain. Examination of the tympanic membrane reveals an erythematous, bulging membrane secondary to purulent fluid in the middle ear and loss of the usual landmarks.

Sinusitis — Sinusitis is also a common complication of streptococcal tonsillopharyngitis; it occurs via extension of organisms from the nasopharynx up the ostiomeatal complex and into the sinuses. The most common presenting symptoms of acute sinusitis are persistent coryza and postnasal drip. Headache and fever are also frequently present. Examination of the sinuses will often show tenderness over the affected sinus(es). (See "Acute sinusitis and rhinosinusitis in adults: Clinical manifestations and diagnosis" and "Acute bacterial rhinosinusitis in children: Clinical features and diagnosis", section on 'Acute bacterial rhinosinusitis'.)

Skin and soft tissue infections — Skin and soft tissue infections associated with streptococcal infection include necrotizing fasciitis and pyomyositis.

Necrotizing fasciitis caused by GAS may occur following a breach in the skin (due to acute varicella infection, trauma, or other cause). It can also occur in association with transmission of GAS from an individual with acute pharyngitis [22]. When a portal of entry is not clear, the pathogenesis may be attributable to hematogenous translocation of GAS from the throat (in the presence or absence of symptomatic pharyngitis) to a site of blunt trauma or muscle strain [23]. (See "Necrotizing soft tissue infections".)

Necrotizing fasciitis typically presents with unexplained pain that increases rapidly over time. Local or diffuse erythema may be present; however, in some patients, severe pain in the absence of cutaneous findings is the only clue to infection. Within 24 to 48 hours, erythema may develop or darken, frequently with associated blisters and bullae; bullae can also develop in normal-appearing skin. Fever, tachycardia, malaise, myalgia, diarrhea, and anorexia may also be present during the first 24 hours. Hypotension may be present initially or develop over time. Surgical exploration is critical for diagnosis and management of necrotizing fasciitis. (See "Necrotizing soft tissue infections".)

Issues related to pyomyositis are discussed separately. (See "Primary pyomyositis".)

Other complications — Streptococcal bacteremia and metastatic infection to the meninges, brain, or distant sites are rare suppurative complications of streptococcal tonsillopharyngitis. (See "Invasive group A streptococcal infections in children" and "Invasive group A streptococcal infection and toxic shock syndrome: Epidemiology, clinical manifestations, and diagnosis".)

Foodborne outbreaks of streptococcal pharyngitis have been described [24,25].

Postoperative GAS infections have occurred in association with intraoperative exposure to health care workers with cutaneous, pharyngeal, vaginal, or rectal GAS carriage [26]. In addition, transmission of GAS can occur from surgical wounds and cause pharyngitis in health care workers [27].

PREVENTING COMPLICATIONS — Treatment of streptococcal tonsillopharyngitis with antibiotic therapy is important for reducing complications [28]. (See "Treatment and prevention of streptococcal pharyngitis in adults and children".)

SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Streptococcal tonsillopharyngitis".)

INFORMATION FOR PATIENTS — UpToDate offers two types of patient education materials, "The Basics" and "Beyond the Basics." The Basics patient education pieces are written in plain language, at the 5^th to 6^th grade reading level, and they answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials. Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are written at the 10^th to 12^th grade reading level and are best for patients who want in-depth information and are comfortable with some medical jargon.

Here are the patient education articles that are relevant to this topic. We encourage you to print or email these topics to your patients. (You can also locate patient education articles on a variety of subjects by searching on "patient info" and the keyword(s) of interest.)

●Basics topics (see "Patient education: Strep throat in adults (The Basics)" and "Patient education: Strep throat in children (The Basics)" and "Patient education: Scarlet fever (The Basics)")

SUMMARY

●Preventing complications of tonsillopharyngitis – Pharyngitis due to infection with group A Streptococcus (GAS) is usually a self-limited condition, with symptoms lasting two to five days in untreated patients. Treatment of streptococcal tonsillopharyngitis with antibiotic therapy is important for reducing complications. (See 'Introduction' above and "Treatment and prevention of streptococcal pharyngitis in adults and children".)

●Nonsuppurative complications – The nonsuppurative complications of GAS tonsillopharyngitis include acute rheumatic fever (ARF), scarlet fever, streptococcal toxic shock syndrome, acute glomerulonephritis, and pediatric autoimmune neuropsychiatric disorder associated with group A streptococci (PANDAS). (See 'Nonsuppurative complications' above.)

•Acute rheumatic fever – Acute rheumatic fever is a delayed sequela of a pharyngeal infection with GAS. Following the initial pharyngitis, there is a latent period of two to three weeks before the first signs or symptoms of ARF appear. The disease presents with various manifestations that may include arthritis, carditis, chorea, subcutaneous nodules, and erythema marginatum. (See 'Acute rheumatic fever' above.)

•Scarlet fever – Scarlet fever is a diffuse erythematous eruption that generally occurs in association with pharyngitis. Development of scarlet fever requires prior exposure to Streptococcus pyogenes and occurs as a result of delayed-type skin reactivity to pyrogenic exotoxin (erythrogenic toxin, usually types A, B, or C) produced by the organism. The rash of scarlet fever is a diffuse erythema that blanches with pressure, with numerous small (1 to 2 mm) papular elevations, giving a "sandpaper" quality to the skin (picture 1). (See 'Scarlet fever' above.)

•Streptococcal toxic shock syndrome – Severe GAS infections associated with shock and organ failure have been reported with increasing frequency, predominantly from North America and Europe. (See 'Streptococcal toxic shock syndrome' above.)

•Poststreptococcal glomerulonephritis – Poststreptococcal glomerulonephritis is induced by infection with specific nephritogenic strains of GAS (such as type 12 and type 49). This can occur in sporadic cases or during an epidemic. The clinical presentation can vary from asymptomatic, microscopic hematuria to full-blown acute nephritic syndrome, characterized by red to brown urine, proteinuria (which can reach the nephrotic range), edema, hypertension, and acute renal failure. (See 'Acute glomerulonephritis' above.)

•PANDAS syndrome – Pediatric autoimmune neuropsychiatric disorder associated with group A streptococci (PANDAS) is a term used to describe a subset of children whose symptoms of obsessive compulsive disorder or tic disorders are exacerbated by GAS infection. This is discussed in detail separately. (See "PANDAS: Pediatric autoimmune neuropsychiatric disorder associated with group A streptococci".)

●Suppurative complications – The suppurative complications of GAS tonsillopharyngitis include tonsillopharyngeal cellulitis or abscess, otitis media, sinusitis, and necrotizing fasciitis; streptococcal bacteremia, meningitis, and brain abscess are rare complications of GAS infection. (See 'Suppurative complications' above.)