NATIONALHANSEN’S DISEASE

PROGRAM

NATIONALHANSEN’S DISEASE

PROGRAM

REACTIONS INHANSEN’S DISEASE

REACTIONS INHANSEN’S DISEASE

REACTIONS - What are they?REACTIONS - What are they?

• Increased immune response

• Not treatment failure, drug toxicity or drug allergy

• Increased immune response

• Not treatment failure, drug toxicity or drug allergy

TERMINOLOGYTERMINOLOGY

•Reversal Reaction (Type 1)Upgrading reaction in BT, BB, BL

•Reversal Reaction (Type 1)Upgrading reaction in BT, BB, BL

•Erythema Nodosum Leprosum (Type 2)Immune complex disorder in BL and LL

•Erythema Nodosum Leprosum (Type 2)Immune complex disorder in BL and LL

•Lucio PhenomenonReaction in Lucio Leprosy

•Lucio PhenomenonReaction in Lucio Leprosy

NEURITISNEURITIS

• Tender or painful nerves with or without other symptoms of reaction

• Silent neuritis – deterioration of nerve function without other symptoms of reaction

• Tender or painful nerves with or without other symptoms of reaction

• Silent neuritis – deterioration of nerve function without other symptoms of reaction

REVERSAL REACTION REVERSAL REACTION

• Coombs and Gell type IV hypersensitivity

• Shift of cell mediated immunity toward tuberculoid disease

• Occurs in BT, BB and BL disease

• Coombs and Gell type IV hypersensitivity

• Shift of cell mediated immunity toward tuberculoid disease

• Occurs in BT, BB and BL disease

The Hansen’s Disease SpectrumThe Hansen’s Disease Spectrum

LEPROMATOUSLEPROMATOUS

MANYMANYBACTERIABACTERIA

ANTIBODYANTIBODYLEVELSLEVELS

HIGHHIGH

LOW RESISTANCELOW RESISTANCETO M. LEPRAETO M. LEPRAE

TT BT BB BL LLTT BT BB BL LL

HIGH RESISTANCEHIGH RESISTANCETO M. LEPRAETO M. LEPRAE

LOWLOWANTIBODYANTIBODY

LEVELSLEVELS

BACTERIABACTERIAFEWFEW

TUBERCULOIDTUBERCULOID

CMICMI BIBI

ENLENL

ANTIBODY

ANTIBODY

LEVEL

LEVEL

Reversal Reaction

Reversal Reaction

ERYTHEMA NODOSUM LEPROSUMERYTHEMA NODOSUM LEPROSUM

• Immune complex disorder

• Coombs and Gell type III hypersensitivity

• TNF levels increase during ENL

• Neutrophil infiltration

• Occurs in BL and LL disease

• Immune complex disorder

• Coombs and Gell type III hypersensitivity

• TNF levels increase during ENL

• Neutrophil infiltration

• Occurs in BL and LL disease

The Hansen’s Disease SpectrumThe Hansen’s Disease Spectrum

LEPROMATOUSLEPROMATOUS

MANYMANYBACTERIABACTERIA

ANTIBODYANTIBODYLEVELSLEVELS

HIGHHIGH

LOW RESISTANCELOW RESISTANCETO M. LEPRAETO M. LEPRAE

TT BT BB BL LLTT BT BB BL LL

HIGH RESISTANCEHIGH RESISTANCETO M. LEPRAETO M. LEPRAE

LOWLOWANTIBODYANTIBODY

LEVELSLEVELS

BACTERIABACTERIAFEWFEW

TUBERCULOIDTUBERCULOID

CMICMI BIBI

ENLENL

ANTIBODY

ANTIBODY

LEVEL

LEVEL

Reversal Reaction

Reversal Reaction

The Hansen’s Disease SpectrumThe Hansen’s Disease Spectrum

LEPROMATOUSLEPROMATOUS

MANYMANYBACTERIABACTERIA

ANTIBODYANTIBODYLEVELSLEVELS

HIGHHIGH

LOW RESISTANCELOW RESISTANCETO M. LEPRAETO M. LEPRAE

TT BT BB BL LLTT BT BB BL LL

HIGH RESISTANCEHIGH RESISTANCETO M. LEPRAETO M. LEPRAE

LOWLOWANTIBODYANTIBODY

LEVELSLEVELS

BACTERIABACTERIAFEWFEW

TUBERCULOIDTUBERCULOID

CMICMI BIBI

ENLENL

ANTIBODY

ANTIBODY

LEVEL

LEVEL

Reversal Reaction

Reversal Reaction

PRECIPITATING FACTORSIN REACTIONS

PRECIPITATING FACTORSIN REACTIONS

• Physical or mental stress

• Pregnancy

• Surgical procedures

• Injuries

• Intercurrent infections

• Antibacterial treatment of HD

• Physical or mental stress

• Pregnancy

• Surgical procedures

• Injuries

• Intercurrent infections

• Antibacterial treatment of HD

DIAGNOSIS OF REACTIONDIAGNOSIS OF REACTION

• Worsening of existing skin lesions with increased edema erythema

• New erythematous skin lesions

• Painful or tender nerves

• Pain and/or swelling of hands and feet

• Worsening of existing skin lesions with increased edema erythema

• New erythematous skin lesions

• Painful or tender nerves

• Pain and/or swelling of hands and feet

REVERSAL REACTIONREVERSAL REACTION

• Usually occurs within first six months of treatment

• Nerve damage may occur rapidly

• Reversal reaction with acute neuritis is a medical emergency

• Usually occurs within first six months of treatment

• Nerve damage may occur rapidly

• Reversal reaction with acute neuritis is a medical emergency

EDEMA DURING REACTIONSEDEMA DURING REACTIONS

• Edema of hands and feet is a common symptom

• Swelling of hands can cause permanent stiffness

• Edema of hands and feet should always be considered a symptom of severe reaction

• Edema of hands and feet is a common symptom

• Swelling of hands can cause permanent stiffness

• Edema of hands and feet should always be considered a symptom of severe reaction

ENL ENL

• Usually 1 – 3 years after beginning treatment

• Involves skin, nerves, eyes and other organs

• May present as a febrile illness

• Nerve damage usually slow

• Usually 1 – 3 years after beginning treatment

• Involves skin, nerves, eyes and other organs

• May present as a febrile illness

• Nerve damage usually slow

CLINICAL FEATURES OF REACTIONCLINICAL FEATURES OF REACTION

Reversal ReactionReversal Reaction -- Assymetric skin andAssymetric skin andnerve involvementnerve involvement

ENLENL -- Diffuse skin and nerveDiffuse skin and nerveinvolvementinvolvement

Reversal ReactionReversal Reaction -- Nerve pain and acuteNerve pain and acutenerve damage rapidnerve damage rapid

ENLENL -- Chronic nerve pain andChronic nerve pain andnerve damage slownerve damage slow

Reversal ReactionReversal Reaction -- Assymetric skin andAssymetric skin andnerve involvementnerve involvement

ENLENL -- Diffuse skin and nerveDiffuse skin and nerveinvolvementinvolvement

Reversal ReactionReversal Reaction -- Nerve pain and acuteNerve pain and acutenerve damage rapidnerve damage rapid

ENLENL -- Chronic nerve pain andChronic nerve pain andnerve damage slownerve damage slow

CLINICAL FEATURES OF REACTIONCLINICAL FEATURES OF REACTION

REACTION:REACTION:REACTION:REACTION:

Reversal ReactionReversal Reaction -- BT, BB, BLBT, BB, BLENLENL -- BL and LLBL and LLReversal ReactionReversal Reaction -- BT, BB, BLBT, BB, BLENLENL -- BL and LLBL and LL

LESIONS IN REACTIONLESIONS IN REACTION::LESIONS IN REACTIONLESIONS IN REACTION::

Reversal ReactionReversal Reaction -- Usually appears in Usually appears in previously existing skinpreviously existing skinlesions.lesions.

ENLENL -- Appears in normal skinAppears in normal skin..

Reversal ReactionReversal Reaction -- Usually appears in Usually appears in previously existing skinpreviously existing skinlesions.lesions.

ENLENL -- Appears in normal skinAppears in normal skin..

REACTIONSREACTIONS

MILDMILD SEVERESEVEREMILDMILD SEVERESEVERE

Painful/tender nervesPainful/tender nerves X X

Swelling/pain of the faceSwelling/pain of the face X X

Edema of hands/feetEdema of hands/feet X X

Painless skin lesionsPainless skin lesions X X

Painful/tender nervesPainful/tender nerves X X

Swelling/pain of the faceSwelling/pain of the face X X

Edema of hands/feetEdema of hands/feet X X

Painless skin lesionsPainless skin lesions X X

REVERSAL REACTION TREATMENTREVERSAL REACTION TREATMENT

MildMild•Symptomatic treatment•Symptomatic treatment

SevereSevere•Prednisone 40 - 80 mg single dose daily for 5 - 7 days; taper to elimination in 2 - 6 months

•Prednisone 40 - 80 mg single dose daily for 5 - 7 days; taper to elimination in 2 - 6 months

•Clofazimine in selected cases 300 mg daily for 6 weeks; then 100 - 200 mg daily for 6 - 12 months

•Clofazimine in selected cases 300 mg daily for 6 weeks; then 100 - 200 mg daily for 6 - 12 months

CONTINUE ANTIBACTERIAL TREATMENT AT FULL DOSESCONTINUE ANTIBACTERIAL TREATMENT AT FULL DOSES

ENL TREATMENTENL TREATMENT

MildMild

•Symptomatic treatment•Symptomatic treatment

SevereSevere

•Thalidomide 400 mg daily tapered to 100 mg daily•Thalidomide 400 mg daily tapered to 100 mg daily

•Prednisone 40 - 80 mg daily•Prednisone 40 - 80 mg daily

•Clofazimine 100 - 300 mg daily•Clofazimine 100 - 300 mg daily

CONTINUE ANTIBACTERIAL TREATMENT AT FULL DOSES CONTINUE ANTIBACTERIAL TREATMENT AT FULL DOSES

LENGTH OF STEROID TREATMENTLENGTH OF STEROID TREATMENT

No Evidence of NeuritisNo Evidence of Neuritis

Reaction with NeuritisReaction with Neuritis

• Treat according to symptoms

• ENL may require several years treatment

• Treat according to symptoms

• ENL may require several years treatment

• Minimum 3 months steroid treatment

• Longer periods for nerve pain or loss of nerve function

• Minimum 3 months steroid treatment

• Longer periods for nerve pain or loss of nerve function

MONITORING PATIENTS WITH REACTIONSMONITORING PATIENTS WITH REACTIONS

• Primary concern is preventing permanent nerve damage

• Monitor regularly for sensory or motor changes in hands and feet or eye problems

• Note pain or tenderness of nerves

• Note edema of hands and feet

• Primary concern is preventing permanent nerve damage

• Monitor regularly for sensory or motor changes in hands and feet or eye problems

• Note pain or tenderness of nerves

• Note edema of hands and feet

NATIONAL HANSEN’S DISEASE

PROGRAMS

NATIONAL HANSEN’S DISEASE

PROGRAMS

Baton Rouge, Louisiana

Phone: 1-800-642-2477

Baton Rouge, Louisiana

Phone: 1-800-642-2477