Transcript LEPROSY - Dr. Raj Kumar Sharma

LEPROSY
EPIDEMIOLOGY
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8 MILLION PATIENTS
60,000 NEW CASES EACH YEAR
WORLD WIDE
LEPROSY IS ENDEMIC IN ASIA &
AFRICA
APPOX. 80% CASES IN THESE
AREAS IN THE WORLD
Global Leprosy Situation in 2005
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WHO Region
Africa
Americas
East Mediterranean
South East Asia
Western Pacific
World
Point Prevalence
47 596
36 977
5 398
186 182
10 010
286 063
PERVALENCE OF LEPROSY
IN
SEA REGION
WHO 2003
DEFINITION
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LEPROSY IS A NONFATAL ,CHRONIC INFECTIOUS DISEASE
CAUSED BY MYCOBACTRIUM LEPRAE (By G.A.Hansen 1873)
INVOLVING SKIN
PERIPHERAL NERVOUS SYSTEM
UPPER RESPIRATORY TRACT
EYES
TESTES
M.LEPRAE HAS UNIQE TROPISM FOR PEIPHRAL NERVES
REACTIONAL STATE RESPONSIBLE FOR MORBIDITY &
DISEASE IF NOT TREATED LEADS TO CHARECTERSTIC
DEFORMITY & PROFOUND SOCIAL STIGMA
DEFORMITY
M. LEPRAE
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NONCULTIVABLE IN MEDIUM
FACULTATIVE OBLIAGTE INTRACELLULAR ORGANISM
GRAM-POSITIVE
ACID-FAST BACILUS
PEPTIDOGLYCAN-BACK BONE
ARABINOGALACTAN & MYCOLIC ACID
PHENOLIC GLYCOLIPD-1 (PGL-1) ATTACHED TO LAMININ 2 OF
SCHWANN CELLS
MHC CLASS II FOR DISEASE EXPRESSION NOT FOR
SUSCEPTIBILITY OF DISEASE
SUSCEPTIBILTY GENE LOCUS ON CHROMOSOME-10P13
The route of transmission
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Not been definitively established,
Although human-to-human aerosol spread of nasal
secretions is the most likely mode of transmission in
most cases.
The disease is not spread by touch, since the
mycobacteria are incapable of crossing intact skin.
Living near people with leprosy is associated with
increased transmission. Among household contacts,
the relative risk for leprosy is increased 8- to 10-fold
in multibacillary and 2- to 4-fold in paucibacillary
forms.
Animal reservoirs do exist (armadillos, certain
nonhuman primates), and cases of suspected zoonotic
transmission have been reported.
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Two indices which depend on observation of
M. leprae in smears from skin or nasal smears
are useful in assessing
the amount of infection,
the viability of the organisms
the progress of the patient under treatment.
They are the morphological index (MI)
the bacteriological index (BI).
1. The bacteriological index (BI)
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This is an expression of the extent of bacterial
loads. It is calculated by counting six to eight
stained smears under the 100 x oil immersion
lens.
A smear stained by the Ziehl-Neelsen method
and decolorized (but not completely) which
1% acid alcohol.
The results are expressed on a logarithmic
scale.
BACTERIOLOGICAL INDEX
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LOGAITHMIC SCALE AS TO NUMBER OF
BACILLI PER OIL IMMERSION FIELD(OIF)
BI OF 6 IS 1000 OR MORE BACILLI/OIF
BI OF 5 IS 100 TO 1000/OIF
BI OF 4 IS 10 TO 100 BACILLI/OIF
BI OF 3 IS 1 TO 10 BACILLI/OIF
BI OF 2 IS 1 BACILLUS/1 TO 10 OIFS
BI OF 1 IS 1 BACILLUS/ 10 TO 100 OIFS
BI OF 0 IS NO BACILLUS PER 100 OIFS
MORPHOLOGICAL INDEX
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PERCENTAGE OF SOLIDLY STAINED
BACILLI IN STAINED SMEAR
Only the solid-staining bacilli are viable.
It is not unusual for solid-staining M. leprae to
reappear for short periods in patients being
successfully treated with drugs.
It is important to recognize that measurement
of MI is liable for observer variations and
therefore not always reliable.
IS LEPROSY HOST IMMUNE
DEPENDENT DISEASE?
YES….
CMI OR ANTIBODY
MEDIATED ?
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CLINICAL PRESENTATION
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LEPROSY HAS SPECTRUM OF DISEASE
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Ridley-Jopling classification system
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TT
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INDICIES FOR SPECTRUM
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BACTERIOLOGICAL
IMMUNOLOGICAL
CLINICAL
HISTOPATHOLOGICAL
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BT
BB
BL
LL
WHO Classification system:
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The WHO recommends classifying leprosy according
to the number of lesions and the presence of bacilli on
a skin smear.
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Paucibacillary (PB) leprosy is characterized by 5 or
fewer lesions with absence of organisms on smear.
Includes the tuberculoid and borderline tuberculoid
leprosy categories from the Ridley-Jopling system.
Multibacillary (MB) leprosy is marked by 6 or more
lesions with possible visualization of bacilli on smear.
Includes Lepromatous, borderline lepromatous, and
midborderline on the Ridley-Jopling scale .
The cardinal signs of leprosy
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Hypoesthesia,
skin lesions,
loss of hair and sweating & peripheral
neuropathy.
The first physical signs of leprosy are usually
cutaneous.
The subtype of leprosy often determines the
degree of skin involvement.
Physical examination
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Evaluation of skin lesions
Careful sensory and motor examination
Palpation of peripheral nerves for pain or
enlargement. Particular attention should be paid to
the following locations:
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Elbows - Ulnar nerve
Wrist - Superficial radial cutaneous and median
nerves
Popliteal fossa - Common peroneal nerve
Neck - Great auricular nerve
TUBERCULOID LEPROSY
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Single or few Lesions
Erythematous plaques
Hypopigmanted
Hypoasthatic
Macular lesions
Cutaneous nerve
thickened
TUBERCULOID LEPROSY
TUBERCULOID LEPROSY
TUBERCULOID LEPROSY
BORDERLINE LEPROSY
BORDERLINE LEPROSY
POSTERIOR AURICULAR
NERVE THICKNING
LEPROMATOUS LEPROSY
LEPROMATOUS LEPROSY
LEPROMATOUS LEPROSY
POSTERIOR AURICULR NERVE
NODULR LESION AT PINNA
NODULAR LESION AT PINNA
DIFFUSE INFILTRATION
IN
LEPROMATOUS LEPROSY
DIFFUSE INFILTRATION
IN
LEPROMATOUS LEPROSY
BORDERLINE LEPROMATOUS
LEPROSY
LEFT ULNER NERVE PALSY
ANESTHATIC HAND
SKIN SMEAR FROM LESION SHOWS AFB++
HISTPATHOLOGY
Immunologic tests
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Lepromin skin test
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Not diagnostic of exposure or infection with M
leprae
Assesses a patient's ability to mount a
granulomatous response against a skin
injection of killed M leprae.
Patients with tuberculoid or borderline
lepromatous leprosy typically have a positive
response (>5 mm).
Patients with lepromatous leprosy typically
have no response.
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Detection of antibodies to phenolic glycolipid-1 (PGL-1)
This is a specific serologic test.
This test has a sensitivity of 95% for the detection of
lepromatous disease but only 30% for tuberculoid disease.
PCR and recombinant DNA technology
development of gene probes with M leprae–specific
sequences.
This technology can be used to identify the mycobacterium
in biopsy samples, skin and nasal smears, and blood and
tissue sections.
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Lymphocyte migration inhibition test (LMIT):
As determined by a lymphocyte transformation and LMIT,
cell-mediated immunity to M leprae is absent in the
lepromatous form of disease but present in the tuberculoid
form of disease.
Contact or family screening for history of leprosy
REACTIONIONAL SATES
IN
LEPROSY
TYPE -1 REACTION
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OCCURS IN BORDERLINE CASES NOT IN POLAR
LEPROSY
ACTIVATION OF PREVIOUSLY INVOLVED SKIN
LESIONS
PANFUL & TENDER NERVES
DOWNGREADING REACTION - WHEN OCCURS
BEFORE INITIATON OF CHEMOTHERAPY
REVERSAL REACTION AFTER INITIATION OF
CHEMOTHERAPY- TH1 RESPONSES WITH IFNGAMMA,IL2
CORTICOSTEROID TREATMENT OF CHOICE
TYPE -1 REACTION
TYPE -2 REACTION
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OCCURS IN BL/LL FORM OF LEPROSY
IN 50% OF CASES
IN 90% OF CASES MAY BE PRESENTING SYMPTOM OF DISEASE
ENL- ERYTHEMA NODOSUM LEPROSUM
FEVER
ARTHRITIS
UVEITIS
ORCHITIS
GLOMERULONEPHRITIS
TNF- PLAYS CETERAL ROLE,
IMMUNE COMPLEX DEPOSITION
TH2 CYTOKINE PROFIL-IL6,IL8
THALIDOMIDE IS CHOICE OF TREATMENT
ERYTHEMA NODOSUM
LEPROSUM
Erythema Nodosum
TREATMENT-MDT
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MULTI
DRUG
THERAPY
What is WHO MDT?
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Multi drug therapy (MDT) is a key element of the
elimination strategy.
MDT is available free of charge from WHO
The drugs used in WHO-MDT are a combination of
Rifampicin,
clofazimine and Dapsone for MB leprosy patients and
Rifampicin and Dapsone for PB leprosy patients.
Treatment of leprosy with only one anti leprosy drug
will always result in development of drug resistance.
Treatment with Dapsone or any other anti leprosy
drug used as monotherapy should be considered as
unethical practice.
MULTI DRUG THERAPY
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Yes, it is the best combination available today,
as proved by its successful application in
leprosy control under varying conditions since
1982.
The combination not only cures leprosy but is
also highly cost-effective.
TREATMENT REGIMENS WHO
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TUBERCULOID- PAUCIBACILLARY
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DAPSONE 100 mg/D UNSUPERVISED +
RIFAMPIN 600mg/MONTH SUPERVISED FOR 6 MONTH
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MULTIBACILLARY DISEASE
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DAPSONE 100mg /D PLUS
CLOFAZIMINE 50mg/D UNSUPERVISED PLUS
RIFIAMPIN 600mg + CLOFAZIMNE 300mg MONTHLY SUPERVISED
FOR 1 YEAR
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Physiotherapy
 Reconstructive surgeries
 Rehabilitation programes
 Health education
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THANK
YOU…….
MDT
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Rifampicin: The drug is given once a month. No toxic effects
have been reported in the case of monthly administration. The
urine may be coloured slightly reddish for a few hours after its
intake, this should be explained to the patient while starting
MDT.
Clofazimine: It is most active when administered daily. The
drug is well tolerated and virtually non-toxic in the dosage
used for MDT. The drug causes brownish black discoloration
and dryness of skin. However, this disappears within few
months after stopping treatment. This should be explained to
patients starting MDT regimen for MB leprosy.
Dapsone: The drug is very safe in the dosage used in MDT
and side effects are rare. The main side effect is allergic
reaction, causing itchy skin rashes and exfoliative dermatitis.
Patients known to be allergic to any of the sulpha drugs should
not be given dapsone.