Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ACUTE

patients without CNS involvement

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initial antibiotic therapy

Standard recommendations are initial treatment with either ceftriaxone or benzylpenicillin.[11][77][78][79][80]​​

Trimethoprim/sulfamethoxazole in combination with intramuscular streptomycin is recommended as an alternative. It can be used in penicillin-allergic patients.

Oral doxycycline plus hydroxychloroquine can be considered as a second-line option in patients who are allergic to ceftriaxone, benzylpenicillin, or trimethoprim/sulfamethoxazole, or who are unable to tolerate parenteral therapy.

Initial antibiotic therapy is for 14 days.

Primary options

ceftriaxone: 2 g intravenously every 24 hours

OR

procaine benzylpenicillin: 1.2 g intramuscularly every 24 hours

OR

benzylpenicillin sodium: 1.2 g intravenously every 4 hours

OR

trimethoprim/sulfamethoxazole: 160/800 mg orally three times daily

and

streptomycin: 500 mg intramuscularly every 12 hours

Secondary options

doxycycline: 100 mg orally twice daily

and

hydroxychloroquine: 200 mg orally three times daily

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Plus – 

maintenance antibiotic therapy

Treatment recommended for ALL patients in selected patient group

Standard maintenance therapy is oral trimethoprim/sulfamethoxazole for 1 year, or until bacterial DNA on duodenal biopsies can no longer be seen.[79]

Oral doxycycline plus hydroxychloroquine can be used as alternative maintenance therapy in sulfa-allergic patients.

Primary options

trimethoprim/sulfamethoxazole: 160/800 mg orally twice daily

Secondary options

doxycycline: 100 mg orally twice daily

and

hydroxychloroquine: 200 mg orally three times daily

patients with CNS involvement

Back
1st line – 

initial antibiotic therapy

Standard recommendations are initial treatment with either ceftriaxone or benzylpenicillin.[11][77][78][79][80]​​

Trimethoprim/sulfamethoxazole in combination with intramuscular streptomycin is recommended as an alternative. It can be used in penicillin-allergic patients.

Oral doxycycline plus hydroxychloroquine can be considered as a second-line option in patients who are allergic to ceftriaxone, benzylpenicillin, or trimethoprim/sulfamethoxazole, or who are unable to tolerate parenteral therapy. However, in patients with CNS involvement, trimethoprim/sulfamethoxazole should be used in addition to the oral doxycycline plus hydroxychloroquine regimen.

Initial antibiotic therapy is for 14 days.

Primary options

ceftriaxone: 2 g intravenously every 24 hours

OR

procaine benzylpenicillin: 1.2 g intramuscularly every 24 hours

OR

benzylpenicillin sodium: 1.2 g intravenously every 4 hours

OR

trimethoprim/sulfamethoxazole: 160/800 mg orally three times daily

and

streptomycin: 500 mg intramuscularly every 12 hours

Secondary options

doxycycline: 100 mg orally twice daily

and

hydroxychloroquine: 200 mg orally three times daily

and

trimethoprim/sulfamethoxazole: 160/800 mg orally three times daily

Back
Plus – 

maintenance antibiotic therapy

Treatment recommended for ALL patients in selected patient group

In patients with CNS involvement, maintenance therapy consists of trimethoprim/sulfamethoxazole for 1 year (or until bacterial DNA on duodenal biopsies can no longer be seen), plus doxycycline and hydroxychloroquine.[79]

Primary options

doxycycline: 100 mg orally twice daily

and

hydroxychloroquine: 200 mg orally three times daily

and

trimethoprim/sulfamethoxazole: 160/800 mg orally five times daily until PCR of cerebrospinal fluid turns negative, then 160/800 mg twice daily thereafter until end of treatment

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Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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