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DR.SRINATH.CHANDRAMANI
ASST PROF & ICU-INCHARGE
K.J.SOMAIYA HOSPITAL
MUMBAI
IMMUNO-DEFICIENCY DISORDERS
Deficiencies of the Innate Immune System
Phagocytic cells:
- Impaired production: severe congenital neutropenia (SCN)
- Asplenia
- Impaired adhesion: leukocyte adhesion deficiency (LAD)
- Impaired killing: chronic granulomatous disease (CGD)
Innate immunity receptors and signal transduction:
- Defects in Toll-like receptor signaling
- Mendelian susceptibility to mycobacterial disease
Complement deficiencies:
- Classical, alternative, and lectin pathways
- Lytic phase
IMMUNO-DEFICIENCY DISORDERS
Deficiencies of the Adaptive Immune System
T lymphocytes:
- Impaired development Severe combined immune deficiencies (SCIDs) DiGeorge syndrome
- Impaired survival,
migration, function
B lymphocytes:
- Impaired development XL and AR agammaglobulinemia
- Impaired function
Hyper-IgM syndrome
Common variable immunodeficiency (CVID)
IgA deficiency
AUTO-IMMUNITY
Major Criteria
1. Presence of autoantibodies or evidence of cellular reactivity to self
2. Documentation of relevant autoantibody or lymphocytic infiltrate in the pathologic
lesion
3. Demonstration that relevant autoantibody or T cells can cause tissue pathology
a. Transplacental transmission
b. Adaptive transfer into animals
c. In vitro impact on cellular function Supportive Evidence
Supportive Evidence
1. Reasonable animal model
2. Beneficial effect from immunosuppressive agents
3. Association with other evidence of autoimmunity
4. No evidence of infection or other obvious cause
Organ Specific
Graves' disease
Hashimoto's thyroiditis
Autoimmune polyglandular syndrome
Type 1 diabetes mellitus
Insulin-resistant diabetes mellitus
Immune-mediated infertility
Autoimmune Addison's disease
Pemphigus vulgaris
Pemphigus foliaceus
Dermatitis herpetiformis
Autoimmune alopecia
Organ Nonspecific (Systemic)
Systemic lupus erythematosus
Rheumatoid arthritis
Systemic necrotizing vasculitis
Vitiligo
Autoimmune hemolytic anemia
Autoimmune thrombocytopenic purpura
Pernicious anemia
Myasthenia gravis
Multiple sclerosis
Guillain-Barr syndrome
Stiff-man syndrome
Acute rheumatic fever
Sympathetic ophthalmia
Goodpasture's syndrome
Granulomatosis with polyangiitis (Wegener's)
Antiphospholipid syndrome
Sjgren's syndrome
HYPERSENSITIVITY REACTIONS
SLE
ANTIPHOSPHOLIPID ANTIBODY SYNDROME
SYSTEMIC SCLEROSIS / SCLERODERMA
RA
SJOGRENS SYNDROME / SICCA SYNDROME
SPONDYLOARTHROPATHY
VASCULITIS
BEHCETS
MIXED CONNECTIVE TISSUE DISORDER
HYPERSENSITIVITY REACTIONS
TYPE 1 - ANAPHYLAXIS
TYPE 2 ANTIBODY MEDIATED REACTION
TYPE 3 SERUM SICKNESS
TYPE 4 - DELAYED HYPERSENSITIVITY
SLE
PROTOTYPE OF AUTO-IMMUNE DISORDERS
EPIDEMIOLOGY
DIAGNOSTIC CRITERIA
ANTIBODIES
TREATMENT
ANTIPHOSPHOLIPID AB SYNDROME
EPIDEMIOLOGY
ANTIBODIES
CLINICAL FEATURES
DIAGNOSIS
TREATMENT
IN PREGNANCY
Rheumatoid arthritis
Definition
Epidemiology
Diagnostic Criteria 4/7 : arthritis > 6 weeks
symmetric joint involvement
> 3 small joint involvement - MCP, Wrist, PIP
morning stiffness > 1 hour
Subcutaneous nodules
RA factor
X-ray suggestive of joint erosions
SCLERODERMA
Epidemiology
Diagnostic criteria ( next slide )
SCLERODERMA
LOCALSISED
SCLERODERMA
DISTAL TO ELBOWS, FACE
DIFFUSE SCLERODERMA
RAYNAUDS
PHENOMENON
PULMONARY
INVOLVEMENT
RENAL
PRECEDES SKIN,
PROMINENET
LATE/NEVER
LATE
EARLY
RARE
EARLY/FLORID
CALCINOSIS CUTIS
EARLY
LATE
ANTIBODY
ANTI CENTROMERE
ANTI TOPOISO 1
SKIN INVOLVEMENT
GENERALISED, TRUNK
Patients who have lcSSc coexisting with features of SLE, polymyositis, and rheumatoid arthritis
may have mixed connective tissue disease (MCTD).
This overlap syndrome is generally associated with the presence of high titers of autoantibodies
to U1-RNP.
The characteristic initial presentation is Raynaud's phenomenon associated with puffy fingers
and myalgia. Gradually, lcSSc features of sclerodactyly, calcinosis, and cutaneous telangiectasia
develop. Skin rashes suggestive of systemic lupus erythematosus (malar rash, photosensitivity)
or of dermatomyositis (heliotrope rash on the eyelids, erythematous rash on the knuckles) occur.
Arthralgia is common, and some patients develop erosive polyarthritis. Pulmonary fibrosis and
isolated or secondary PAH may develop.
While anti-U1RNP antibodies are detected in the serum in high titers, SSc-specific autoantibodies
are not found. In contrast to SSc, patients with MCTD often show a good response to treatment
with glucocorticoids, and the long-term prognosis is better than that of SSc. Whether MCTD is a
truly distinct entity or is, rather, a subset of SLE or SSc remains controversial.
Sjogrens syndrome
Also called Sicca syndrome
Lymphocytic infiltration of exocrine
glands leading to its manifestatikons.
SPONDYLOARTHROPATHY
ANKYLOSING SPONDYLOSIS
REACTIVE ARTHRITIS
INFLAMMATORY BOWEL RELATED
ARHTRITIS
GOUT
PSOARITIC ARTHROPATHY
The Caspar (CLassification Criteria for Psoriatic Arthritis) Criteriaa
To meet the CASPAR criteria, a patient must have inflammatory articular
disease (joint, spine, or entheseal) with 3 points from any of the following five
categories:
1.Evidence of current psoriasis,b, c a personal history of psoriasis, or a family
history of psoriasisd
2.Typical psoriatic nail dystrophye observed on current physical examination
3.A negative test result for rheumatoid factor
4.Either current dactylitisf or a history of dactylitis recorded by a
rheumatologist
5.Radiographic evidence of juxtaarticular new bone formationg in the hand or
foot
GOUT
Gout Mono Sodium Urate crystals
Pseudo-Gout CPPD
Calium apatite crystals.
VASCULITIS
BEHCETS SYNDROME
Behet's syndrome is a clinicopathologic entity
characterized by recurrent episodes of oral and
genital ulcers, iritis, and cutaneous lesions. The
underlying pathologic process is a
leukocytoclastic venulitis, although vessels of
any size and in any organ can be involved.
Miscellaneous Disorders
Fibromyalgia
Periarthritis Frozen shoulder