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RHEUMAT & IMMUNOLOGY

DR.SRINATH.CHANDRAMANI
ASST PROF & ICU-INCHARGE
K.J.SOMAIYA HOSPITAL
MUMBAI

INTRODUCTION TO IMMUNE SYSTEM


Immunity is broadly divided into :
- Innate immunity
- Adaptive immunity
Individual constituents are the MonocyteMacrophage system, Dendritic cells, NK cells and the
Granulocytes (N, E and Basophils)
T-Cells and B-cells respectively.

INTRODUCTION TO IMMUNE SYSTEM

Other Mediators of immunity are :


- Immunoglobulins
- Interferons
- Complement cascade

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

Severe combined immunodeficiencies


Hyper-IgE syndrome (autosomal dominant)
CD40 ligand deficiency
Wiskott-Aldrich syndrome
Ataxia-telangiectasia and other DNA repair deficiencies

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

FINALLY THE FINAL TEN

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

Mixed Connective Tissue Disease

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.

The diagnostic criteria are as follows :

ACR Classification Criteria for Sjogren syndrome 2012

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

Classification based on caliber of blood vessel involved:


I. Large vessel vasculitis:
Giant cell arteritis
Takayasus arteritis
II. Medium vessel vasculitis:
Polyarteritis nodosa
Kawasaki disease
III. Small vessel vasculitis:
Microscopic polyangiitis
Leukocytoclastic vasculitis
Wegeners granulomatosis
Churg-Strauss disease

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

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