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NATIONAL HANSEN’S DISEASE PROGRAM

NATIONAL HANSEN’S DISEASE PROGRAM. REACTIONS IN HANSEN’S DISEASE. REACTIONS - What are they?. Increased immune response Not treatment failure, drug toxicity or drug allergy. Reversal Reaction (Type 1) Upgrading reaction in BT, BB, BL. Erythema Nodosum Leprosum (Type 2)

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NATIONAL HANSEN’S DISEASE PROGRAM

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  1. NATIONAL HANSEN’S DISEASE PROGRAM

  2. REACTIONS IN HANSEN’S DISEASE

  3. REACTIONS - What are they? • Increased immune response • Not treatment failure, drug toxicity or drug allergy

  4. Reversal Reaction (Type 1) • Upgrading reaction in BT, BB, BL • Erythema Nodosum Leprosum (Type 2) • Immune complex disorder in BL and LL • Lucio Phenomenon • Reaction in Lucio Leprosy TERMINOLOGY

  5. NEURITIS • 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

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

  8. 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

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

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

  11. PRECIPITATING FACTORS IN REACTIONS • Physical or mental stress • Pregnancy • Surgical procedures • Injuries • Intercurrent infections • Antibacterial treatment of HD

  12. 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

  13. REVERSAL REACTION • Usually occurs within first six months of treatment • Nerve damage may occur rapidly • Reversal reaction with acute neuritis is a medical emergency

  14. 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

  15. 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

  16. 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

  17. CLINICAL FEATURES OF REACTION REACTION: 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.

  18. REACTIONS MILDSEVERE Painful/tender nerves X Swelling/pain of the face X Edema of hands/feet X Painless skin lesions X

  19. 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 • 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

  20. ENL TREATMENT Mild • Symptomatic treatment Severe • 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

  21. 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 LENGTH OF STEROID TREATMENT No Evidence of Neuritis Reaction with Neuritis

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