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Rocky Mountain Spotted Fever (RMSF)

BASICS

  • Definition: Tick-borne disease caused by Rickettsia rickettsii; most severe/fatal US spotted fever rickettsiosis (SFR).
  • Key Symptoms: Fever, headache, and rash (typically wrists/ankles, spreading to palms/soles and trunk).
  • Systems affected: Cardiovascular, skin, CNS, renal, hepatic, pulmonary.

EPIDEMIOLOGY

  • Vectors: Ticks (American dog tick Dermacentor variabilis—most common; Rocky Mountain wood tick, brown dog tick).
  • Distribution: All US states except Hawaii/Alaska; most common in southeast/southcentral US (Arkansas, Missouri, NC, TN, VA).
  • Incidence: US SFR incidence: 1.7/million (2000) → 13.2/million (2016); 5,207 cases (2019).
  • Season: Peak May–August.
  • Risk Groups: Highest incidence >40 years, highest mortality in children <10 years.

ETIOLOGY & PATHOPHYSIOLOGY

  • Organism: R. rickettsii released from tick salivary glands after 6–10 hr feeding.
  • Pathology: Vascular endothelial infection → small/medium vessel injury, disseminated inflammation, ↑ vascular permeability → edema, hyponatremia, end-organ damage (e.g., pulmonary/cerebral edema, ARF, shock).
  • Transmission: Mostly via tick bite; rarely by direct inoculation into wounds/conjunctiva or inhalation.

RISK FACTORS

  • Tick bite (esp. engorged or >20 hr attachment)
  • Outdoor exposure/wooded areas
  • Contact with outdoor pets (dogs)/wild animals
  • Tick crushed during removal

PREVENTION

  • Avoid tick habitats; wear long sleeves/pants, light-colored clothing.
  • Use DEET-containing repellents (20–30%).
  • Permethrin spray for clothes.
  • Tick checks and prompt/proper removal.
  • Protect pets (ectoparasite control).

DIAGNOSIS

  • Clinical suspicion essential—do not wait for lab confirmation to start treatment!
  • Typical history: Acute febrile illness, rash (may be absent!), possible tick exposure, May–August.
  • Symptoms (First 1–4 days): Fever, severe headache, malaise, myalgia, N/V, photophobia.
  • Rash: 2–4 days after fever onset; starts as blanching pink macules on wrists/ankles, spreads to trunk, may become petechial. Palms/soles involved by days 5–6 (advanced disease). Rash spares face, not pruritic, may be absent (esp. adults).
  • Other findings: Conjunctival injection, mental status changes, arthralgia, periorbital/peripheral edema, calf pain, hearing loss.

Physical Exam

  • Fever >102°F
  • Erythematous/maculopapular rash (1–5 mm), can become petechial, purpuric, necrotic in severe cases.
  • May have AMS, focal neurologic deficits, lymphadenopathy, hepatosplenomegaly, edema.

Differential Diagnosis

  • Viral exanthems (hand-foot-and-mouth, measles, rubella)
  • Meningococcemia, TTP/ITP, toxic shock syndrome
  • Other tick-borne: ehrlichiosis, Lyme, babesiosis, typhus
  • Drug reaction, serum sickness, Kawasaki disease, vasculitides

Diagnostic Tests

  • Serology (IFA): Gold standard (4-fold rise in IgG). Sensitivity low in first 10–12 days, so treat empirically.
  • PCR: Blood or skin biopsy, more specific.
  • Labs: Thrombocytopenia (60%), hyponatremia, ↑ LFTs, anemia, ↑ BUN/creatinine, elevated PT/PTT.
  • CSF: Lymphocytic pleocytosis, ↑ protein, normal glucose.
  • Imaging: Rarely helpful.

TREATMENT

General Principles

  • Do not delay treatment for lab confirmation!
  • Doxycycline is drug of choice for all ages.

First Line

  • Doxycycline (adults and children, including <8 years):
  • 45 kg: 100 mg PO/IV q12h

  • <45 kg: 2.2 mg/kg PO/IV q12h
  • Continue at least 3 days after fever subsides (minimum 5–7 days).
  • Adverse effects: GI upset, photosensitivity; no dental staining risk in children <8 yrs.
  • Severe allergy: Desensitization may be considered in life-threatening illness.

Second Line

  • Chloramphenicol (only if severe tetracycline allergy):
  • 50–75 mg/kg IV divided q6h x 7 days
  • Avoid in 3rd trimester pregnancy (risk of gray baby syndrome)

Pregnancy

  • Short-term doxycycline is appropriate for RMSF, including in pregnancy/breastfeeding, if suspicion high.
  • Chloramphenicol only if absolutely necessary.

Admission Criteria

  • CNS dysfunction, vomiting, immunocompromised, organ failure, oral therapy not possible, severe allergy.

ONGOING CARE

  • Follow-up: Every 2–3 days until symptom resolution.
  • Hospitalize moderate/severe cases; mild disease can be outpatient with close follow-up.
  • Monitor: CBC, LFTs, electrolytes if needed.
  • Prognosis: Excellent if treated promptly; mortality ↑ with delayed therapy (>5 days).
  • No lifelong immunity—reinfection possible.

PATIENT EDUCATION

  • Tick prevention, outdoor precautions.
  • Early recognition and prompt treatment.
  • Do not wait for rash to appear before seeking care.

COMPLICATIONS

  • Encephalopathy, seizures, focal neurologic deficit, renal/hepatic failure, CHF, respiratory failure, DIC, tissue necrosis.
  • Highest risk: Children <10 years, adults >70, G6PD deficiency (fulminant, fatal cases possible in ≤5 days).

ICD-10 CODES

  • A77.0 Spotted fever due to Rickettsia rickettsii

CLINICAL PEARLS

  • Diagnosis requires a high index of suspicion.
  • Tick bite is often not noticed; rash may be absent.
  • Start doxycycline immediately if RMSF suspected—do not wait for lab confirmation.
  • No evidence for antibiotic prophylaxis after tick bite.