Infectious Disease · PANCE / PANRE

Rabies Post-Exposure Prophylaxis

Lyssavirus zoonosis transmitted via mammalian bites; near-100% fatal once symptomatic — post-exposure prophylaxis with vaccine and HRIG is highly effective.

Also known as: rabies, rabies PEP, post-exposure prophylaxis, hydrophobia, lyssavirus

Overview

Acute viral encephalomyelitis caused by Lyssavirus (Rabies virus), an enveloped negative-sense RNA virus of the Rhabdoviridae family, transmitted by infected mammalian saliva (bite, less often mucous membrane exposure). Once symptomatic, mortality is virtually 100%.

Epidemiology

US: 1-3 human cases annually, mostly bat-related. Worldwide ~59,000 deaths/year, predominantly dog bites in Asia and Africa. In the US, wildlife reservoirs are bats, raccoons, skunks, foxes; small rodents (squirrels, mice) are virtually never infected.

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Risk factors

  • Bite from bat, raccoon, skunk, fox, coyote, or unvaccinated dog/cat (especially abroad)
  • Sleeping in a room where a bat is found (potential unrecognized exposure)
  • Travel to dog-rabies endemic areas (India, China, Africa)
  • Spelunking, wildlife handling, veterinary work
  • Failure to seek timely PEP after exposure

Pathophysiology

Virus replicates locally in muscle, then enters peripheral nerves at neuromuscular junctions and travels retrograde axonally to the CNS at ~12-100 mm/day. Replicates massively in CNS neurons (Negri bodies on histology) → encephalitis. Then spreads centrifugally to salivary glands, skin, cornea, and other organs. Long incubation (typically 1-3 months, range days to years) reflects time required for axonal transport from the bite site.

Clinical presentation

Symptoms

  • Prodrome (1-7 days): fever, malaise, headache, anorexia; pain or paresthesias at bite site (pathognomonic)
  • Furious form (~80%): hydrophobia (painful pharyngeal spasms triggered by attempts to drink), aerophobia, agitation, hyperactivity, hallucinations, autonomic instability (hypersalivation, sweating, lacrimation), seizures
  • Paralytic form (~20%): ascending flaccid paralysis resembling Guillain-Barre
  • Coma, multi-organ failure, death within 1-2 weeks of symptom onset

Signs / physical exam

  • Hypersalivation, lacrimation, sweating
  • Pharyngeal/laryngeal spasm with drinking
  • Hyperactivity alternating with lucid intervals
  • Cranial nerve palsies
  • Negri bodies (eosinophilic cytoplasmic inclusions in neurons) on autopsy — historical hallmark

Classic findings

Hydrophobia and aerophobia in an encephalopathic patient with a remote animal bite — virtually pathognomonic for clinical rabies.

Differential diagnosis

  • Viral encephalitis (HSV, arboviral) — Fever, altered mental status; CSF, PCR; treat empiric acyclovir
  • Guillain-Barre syndrome — Ascending flaccid paralysis, areflexia, albuminocytologic dissociation; can resemble paralytic rabies
  • Tetanus — Rigidity with preserved consciousness; unattended wound; differs from rabies encephalitis
  • Acute psychosis or substance intoxication — May mimic furious rabies; toxicology screen, history
  • Other encephalitides (autoimmune, anti-NMDA) — Autoantibodies, immunotherapy response

Diagnostic workup

Diagnostic criteria

Clinical syndrome with exposure history and laboratory confirmation. Diagnosis is most often confirmed in the animal source.

Labs

  • Pre-mortem diagnosis difficult: skin biopsy (nuchal area) with direct fluorescent antibody for viral antigen, CSF/saliva PCR, serum/CSF antibody (after 7-10 days of illness)
  • Animal testing: brain tissue with direct fluorescent antibody is gold standard (requires euthanasia)
  • Post-mortem: brain biopsy with Negri bodies and direct fluorescent antibody

Imaging

  • MRI may show abnormal signal in brainstem, hippocampus, hypothalamus — non-specific

Diagnostic algorithm

flowchart TD
  A[Potential rabies exposure<br/>bite or saliva to wound/mucosa] --> B{Animal type}
  B -->|Small rodent / rabbit| C[PEP rarely indicated]
  B -->|Healthy dog/cat available| D[Quarantine animal 10 days]
  D -->|Healthy at 10 d| E[No PEP]
  D -->|Develops illness| F[Initiate PEP]
  B -->|Wild reservoir<br/>bat, raccoon, skunk, fox| G[Initiate PEP immediately<br/>test animal if available]
  G --> H[Step 1: Wash wound<br/>15 min soap and water]
  H --> I[Step 2: HRIG 20 IU/kg<br/>infiltrate around wound]
  I --> J[Step 3: Vaccine days 0, 3, 7, 14<br/>5 doses if immunocompromised]
Rabies post-exposure prophylaxis decision pathway (US/ACIP recommendations).

Treatment

First-line

  • Post-exposure prophylaxis (PEP) is highly effective if started promptly:
  • 1. Wound care: thorough washing with soap and water for 15 minutes, then povidone-iodine or virucidal agent — reduces local viral load and is the single most important step
  • 2. Human rabies immune globulin (HRIG) 20 IU/kg — infiltrate as much as possible AROUND the wound; remainder IM at a distant site. Given only ONCE, ideally on day 0
  • 3. Rabies vaccine series (HDCV or PCECV) IM in the deltoid (or anterolateral thigh in young children) on days 0, 3, 7, and 14 (4 doses for immunocompetent — ACIP 2010)
  • 4. Immunocompromised patients: 5-dose series on days 0, 3, 7, 14, and 28 PLUS HRIG
  • 5. Pre-existing immunization (pre-exposure prophylaxis received): 2 booster doses on days 0 and 3; do NOT give HRIG

Second-line / adjunct

  • Pre-exposure prophylaxis (PrEP): vaccine series for high-risk workers (veterinarians, lab workers, spelunkers, travelers to endemic areas) — 2-dose series on days 0 and 7 per ACIP 2022 update
  • Milwaukee protocol (induced coma, antivirals) — experimental, virtually no proven survival benefit; not standard
  • Once symptomatic, treatment is supportive comfort care; survival reports remain anecdotal

Complications

  • Death from rabies encephalitis (virtually 100% once symptomatic)
  • PEP side effects: mild local reactions, low-grade fever; serum sickness rare with modern HDCV/PCECV (vs. older nerve tissue vaccines)

PANCE pearls

  • Bat exposures: any potential contact with a bat (e.g., waking with bat in room, child sleeping in room with bat) is grounds for PEP unless the bat tests negative — bites can be too small to detect.
  • Small rodents (squirrels, hamsters, mice, rats) and rabbits virtually never transmit rabies in the US — PEP rarely indicated.
  • Healthy dog/cat that bit a human can be quarantined and observed for 10 days; if remains healthy, PEP can be deferred.
  • Always wash the wound first — physical removal of virus from the wound is a critical part of PEP.
  • Do NOT give HRIG in the same syringe or site as the vaccine — antibody can neutralize antigen and blunt response.

References

  • ACIP 2010 — Rupprecht et al., Use of a reduced (4-dose) vaccine schedule for postexposure prophylaxis to prevent human rabies (MMWR Recommendations and Reports)
  • ACIP 2022 — Updated recommendations for pre-exposure prophylaxis against rabies (MMWR)
  • WHO 2018 — WHO Expert Consultation on Rabies, third report (Technical Report Series 1012)

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