1. NATIONAL HANSEN’S DISEASE PROGRAM NATIONAL HANSEN’S DISEASE PROGRAM

2. REACTIONS IN HANSEN’S DISEASE REACTIONS IN HANSEN’S DISEASE

3. 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

4. TERMINOLOGY 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 Phenomenon Reaction in Lucio Leprosy Lucio Phenomenon Reaction in Lucio Leprosy

5. NEURITIS 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

6. 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

7. The Hansen’s Disease Spectrum LEPROMATOUS MANY BACTERIA ANTIBODY LEVELS HIGH LOW RESISTANCE TO M. LEPRAE TT BT BB BL LL HIGH RESISTANCE TO M. LEPRAE LOW ANTIBODY LEVELS BACTERIA FEW TUBERCULOID CMI BI ENL ANTIBODY LEVEL Reversal Reaction

10. ERYTHEMA 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

11. The Hansen’s Disease Spectrum LEPROMATOUS MANY BACTERIA ANTIBODY LEVELS HIGH LOW RESISTANCE TO M. LEPRAE TT BT BB BL LL HIGH RESISTANCE TO M. LEPRAE LOW ANTIBODY LEVELS BACTERIA FEW TUBERCULOID CMI BI ENL ANTIBODY LEVEL Reversal Reaction

14. The Hansen’s Disease Spectrum LEPROMATOUS MANY BACTERIA ANTIBODY LEVELS HIGH LOW RESISTANCE TO M. LEPRAE TT BT BB BL LL HIGH RESISTANCE TO M. LEPRAE LOW ANTIBODY LEVELS BACTERIA FEW TUBERCULOID CMI BI ENL ANTIBODY LEVEL Reversal Reaction

15. PRECIPITATING FACTORS IN REACTIONS PRECIPITATING FACTORS IN 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

16. DIAGNOSIS 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

17. REVERSAL 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

31. EDEMA 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

36. 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

45. CLINICAL FEATURES OF REACTION Reversal Reaction-Assymetric skin and nerve involvement ENL-Diffuse skin and nerve involvement Reversal Reaction-Nerve pain and acute nerve damage rapid ENL-Chronic nerve pain and nerve damage slow Reversal Reaction-Assymetric skin and nerve involvement ENL-Diffuse skin and nerve involvement Reversal Reaction-Nerve pain and acute nerve damage rapid ENL-Chronic nerve pain and nerve damage slow

46. CLINICAL FEATURES OF REACTION REACTION:REACTION: Reversal Reaction-BT, BB, BL ENL-BL and LL Reversal Reaction-BT, BB, BL ENL-BL and LL LESIONS IN REACTION : Reversal Reaction-Usually appears in previously existing skin lesions. ENL-Appears in normal skin. Reversal Reaction-Usually appears in previously existing skin lesions. ENL-Appears in normal skin.

47. REACTIONS MILD SEVERE Painful/tender nerves X Swelling/pain of the face X Edema of hands/feet X Painless skin lesions X Painful/tender nerves X Swelling/pain of the face X Edema of hands/feet X Painless skin lesions X

48. REVERSAL REACTION TREATMENT Mild Symptomatic treatment Severe 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 DOSES

49. ENL TREATMENT Mild Symptomatic treatment Severe Thalidomide 400 mg daily tapered to 100 mg daily Thalidomide 400 mg daily tapered to 100 mg daily Prednisone 40 - 80 mg daily Clofazimine 100 - 300 mg daily CONTINUE ANTIBACTERIAL TREATMENT AT FULL DOSES

50. LENGTH OF STEROID TREATMENT No Evidence of Neuritis Reaction 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

51. MONITORING 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

52. NATIONAL HANSEN’S DISEASE PROGRAMS Baton Rouge, Louisiana Phone: 1-800-642-2477 Baton Rouge, Louisiana Phone: 1-800-642-2477