Pertussis Syndrome

ETIOLOGY
• The pertussis syndrome includes disease caused by Bordetella pertussis
and certain other infectious agents.
• Classic pertussis, the whooping cough syndrome, usually is caused by
B. pertussis, a gram-negative pleomorphic bacillus with fastidious
growth requirements. Some cases of pertussis syndrome are caused by
other organisms, including Bordetella parapertussis, which causes a
similar but milder illness that is not affected by B. pertussis vaccination.
• B. pertussis and B. parapertussis infect only humans and are
transmitted person to person by coughing.
• Asymptomatic carriage is rare.
 Adenoviruses have been associated with the pertussis syndrome.
• Dual infection with B. pertussis and adenovirus occurs more frequently
than expected.
 EPIDEMIOLOGY

• The mean incubation period is 6 days.

• Patients are most contagious during the earliest stage.
• The annual rate of pertussis was approximately 100 to 200 cases
per 100,000 population in the prevaccination era, and presently
it may be higher in developing countries.
• In the U.S., the incidence of pertussis has increased steadily
since the 1980s, with 5000 to 10,000 cases reported each year.
The peak age incidence of pertussis in the U.S. is younger than 4
months of age-among infants too young to be completely
immunized and most likely to have the complications of
pneumonia and severe infection that are associated with a high
mortality.
• If vaccination rates are not high, pertussis rates
increase. In the United Kingdom, there was a
steady decline in the incidence of pertussis until
the late 1970s, when the incidence increased
dramatically as the rate of vaccination declined.
These data, a similar episode in Japan at about
the same time, and the decline in the incidence
of cases when vaccines were first tested are
major events indicating the efficacy of
vaccination.
 CLINICAL MANIFESTATIONS

• Classic pertussis is the syndrome seen in most infants beyond the

neonatal period through school age.
• The progression of the disease is divided into
 catarrhal,
 paroxysmal,
 and convalescent stages.
 The catarrhal stage is marked by nonspecific signs (injection,
 increased nasal secretions,
 and low-grade fever) that last 1 to 2 weeks.
 The paroxysmal stage
 is the most distinctive stage of pertussis.
 Coughing occurs in paroxysms during expiration, causing young
children to lose their breath.
 This pattern of coughing is due to the need to dislodge plugs
of necrotic bronchial epithelial tissues and thick mucus.
 It lasts approximately 2 to 4 weeks.
 The forceful inhalation against a narrowed glottis that follows
this paroxysm of cough produces the characteristic whoop.
 Post-tussive emesis is common.
 The convalescent stage
o is marked by gradual resolution of symptoms over 1 to 2
weeks.
o Coughing becomes less severe, and the paroxysms and
whoops slowly disappear.
o Although the disease typically lasts 6 to 8 weeks, residual
cough may persist for months, especially with physical stress
or respiratory irritants
 Young infants
 may not display the classic pertussis syndrome;
 the first signs may be episodes of apnea.
 Young infants are unlikely to have the classic whoop,
 are more likely to have CNS damage as a result of hypoxia,
 and are more likely to have secondary bacterial pneumonia.
 Adolescents and adults with pertussis usually present with a
prolonged bronchitic illness that often begins as a nonspecific
upper respiratory tract infection. This illness is not much
different from pertussis in children, but generally adolescents
and adults do not have a whoop with the cough, although
they may have severe paroxysms. The cough may persist many
weeks to months
 LABORATORY AND IMAGING STUDIES

• The diagnosis depends on isolation of B. pertussis, which is

usually accomplished during the early phases of illness by
culture of nasopharyngeal swabs on Regan-Lowe medium or
classically on glycerin-potato-blood agar medium (Bordet-
Gengou) to which penicillin has been added to inhibit growth
of other organisms.
• Direct fluorescent antibody staining to detect the organism is
technically difficult, dependent on the skills of the
technologist, and has low specificity.
• PCR is useful.
• Available serologic tests are not useful for diagnosis of acute
infection.
• A characteristic feature of pertussis in patients beyond the
neonatal age is an abnormally high absolute number and
relative percentage of lymphocytes in the peripheral blood.
• In classic B. pertussis-associated pertussis, lymphocytosis is
found in 75% to 85% of patients, although in young infants the
rate is much less.
• The WBC count may increase from 20,000 cells/mm 3 to more
than 50,000 cells/mm3, consisting mostly of mature lymphocytes.
• It is not unusual for physical and radiographic signs of segmental
lung atelectasis to develop during pertussis, especially during the
paroxysmal stage.
• Perihilar infiltrates are common and are similar to what is seen in
viral pneumonia.
 DIFFERENTIAL DIAGNOSIS

• For a young child with classic pertussis syndrome, the

diagnosis based on recognition of the pattern of
illness is quite accurate. The paroxysmal stage is the
most distinctive part of the syndrome.
• Respiratory viruses such as RSV, parainfluenza virus,
and C. pneumoniae can produce bronchitic illnesses
among infants.
• In older children and young adults, M. pneumoniae
may produce a prolonged bronchitic illness that is not
distinguished easily from pertussis in this age group
 TREATMENT
• Erythromycin, given early in the course of illness, eradicates
nasopharyngeal carriage of organisms within 3 to 4 days and
ameliorates the effects of the infection.
• Treatment is not effective in the paroxysmal stage.
• When given to neonates younger than 4 weeks old, erythromycin
has been associated rarely with pyloric stenosis, but treatment is still
recommended because of the seriousness of pertussis at this age.
• Azithromycin and clarithromycin can be given for a shorter duration
and are associated with fewer gastrointestinal adverse effects.
• TMP-SMZ may be beneficial as an alternative, but this remains
unproven.
• Pertussis-specific immunoglobulin may be effective in reducing the
symptoms of the paroxysmal stage.
 COMPLICATIONS
• Major complications are most common among infants and
young children and include
 hypoxia,
 apnea,
 pneumonia,
 seizures,
 encephalopathy,
 and malnutrition.
 The most frequent complication is pneumonia caused by B.
pertussis itself or resulting from secondary bacterial
infection from S. pneumoniae, Hib, and S. aureus.
 Atelectasis may develop secondary to mucous plugs.
• The force of the paroxysm may rupture alveoli and
produce:
 pneumomediastinum,
 pneumothorax,
 or interstitial or subcutaneous emphysema;
 epistaxis;
 hernias;
 and retinal and subconjunctival hemorrhages.
 Otitis media and sinusitis may occur.
 PROGNOSIS
• Most children do well with complete healing
of the respiratory epithelium and have normal
pulmonary function after recovery. Young
children can die from pertussis; 13 children
died in the U.S. from pertussis in 2003. Most
deaths occurred in unvaccinated children or
infants too young to be vaccinated.
• Most permanent disability is a result of
encephalopathy.
 PREVENTION
• Active immunity can be induced with acellular pertussis
vaccine, given in combination with the toxoids of tetanus
and diphtheria (DTaP). Pertussis vaccine has an efficacy of
70% to 90%; efficacy declines with fewer vaccinations. The
acellular vaccines contain one or more antigens isolated
from B. pertussis, such as pertussis toxin, pertactin, or
filamentous hemagglutinins. Each preparation currently
licensed seems to provide equivalent protection. DTaP
vaccine is available for adolescents. Compared with older,
whole cell pertussis vaccines, acellular vaccines have
fewer adverse effects and local reactions
• Erythromycin and other macrolides are effective in
preventing disease in contacts exposed to
pertussis. Close contacts younger than 7 years old
who have received four doses of vaccine should
receive a booster dose of DTaP, unless a booster
dose has been given within the preceding 3 years.
They also should be given a macrolide antibiotic.
Close contacts older than age 7 should receive
prophylactic macrolide antibiotic for 10 to 14 days,
but not the vaccine.