the principles of rheumatology
TRANSCRIPT
Jiang Lindi , Dai Xiaomin
Department of Rheumatology
Zhongshan Hospital, Fudan University
The Principles of Rheumatology
“Rheumatology”
rheuma
^ first appeared in “Hippocratic” in 3rd Century B.C.
^ flows as a river or stream
^ hypothesis of humor
tology
the study of
History of Rheumatology
3rd
Century B.C. ~18th
Century：vague
18th
Century ~ 1980s :
systemic diseases
1949, Joseph L. Hollander, first use the word“rheumatology”
treatments: antibiotics, antimalarials, antineoplastics, and
anti-immune-rejection drugs
1980s ~
rapidly developing discipline
advancements owing largely to new
scientific discoveries related to immunology
of these disorders.
Pathogenesis of many rheumatic diseases are
related to immunology and molecular biology.
The discovery of new biomarkers greatly improve
diagnostic efficiency.
Rheumatology
Most new treatment modalities are also based on
clinical research in immunology :
NSAIDs, GC, DMARDs, biologic agents
Future treatment may include gene therapy as well.
At present evidence-based medical treatment has
helped patients with rheumatic disease lead a near-
normal life
Rheumatology
Rheumatic Disease
any painful disorder affecting joints, musle ,
connective tissue, soft tissue around the joints
and bones.
connective tissue disease (CTD) is a major
focus of rheumatic diseases
The Classification Of Rheumatic Diseases
CTD
Rheumatoid arthritis
Lupus erythematosus
Sjögren's syndrome
Scleroderma
Polymyositis
Dermatomyositis
Vasculitis
Adult onset still disease
Mixed connective tissue disease
Degenerative：Osteoarthritis
Crystal-induced：Gout
Septic/infectious
Congenital and familial disorders affecting Joints
Marfan syndrome
Ehlers-Danlos syndrome
SPA（seronegative spondyloarthropathies）：
ankylosing spondylitis(AS), reactive arthritis(ReA), psoriatic arthritis(PsA), arthropathy of inflammatory bowel disease, undifferential SPA
What are connective tissues ?
Connective tissues are the structural portions of our body that
essentially hold the cells of the body together.
These tissues form a framework or matrix for the body.
Examples of connective tissue are
tendons, ligaments, cartilage, blood, bone,
and the dermis of the skin.
Their functions include cushioning, protecting, supporting,
insulating and strengthening the body tissues and organs.
Connective tissue diseases （CTD）
Most CTD are autoimmune diseases where
auto-bodies attack the normal cells and lead
to damage of organ and progressive
deterioration of function for the patients
What causes connective tissue disease?
The specific causes of most CTD are
unknown.
There are genetic patterns that are
considered to increase the risk for
developing CTD.
A HLA type and associated conditions
Infections, drugs, physicochemical
factors,as well as family history, have been
postulated
It is likely that a combination of genetic
risks and environmental factors are
necessary for the development of CTD.
What causes connective tissue disease?
How causes connective tissue diseases?
At the heart of the immune response is the ability to
distinguish between self and non-self, or foreign.
Every body cell carries distinctive molecules that
distinguish it as "self."
Lymphocytes in patients with CTD lost the ability
and cell-mediated immunity (involving T-cells), and
humoral immunity (involving antibody-producing B-
cells) are abnormally activated.
CTD are characterized as a group by the presence of
overactivity of the immune system that results in
the production of extra antibodies into the
circulation.
rheumatoid factors (RF), antinuclear autobodies (ANA),
anti-SSA，anti-SSB, anti-Smith , anti-Jo1, anti-RNP,
ani-CCP (Cyclic Citrullinated Peptide),
ANCA (anti-neutrophil cytoplasmic antibody ), and so on.
How causes connective tissue diseases?
What are the symptoms of connective tissue disorders?
The manifestations of skin, joint, muscle, mucosa are
the most common in patients with CTD
The symptoms of CTD vary depending on the tissue
involved. The severity and type of symptoms vary
among individuals.
RA symptoms and signs :
Joint pain and swelling
Involvement of multiple joints
Joint stiffness, especially in the
morning
Fatigue
Rheumatoid nodules
Lupus symptoms and signs Joint pain
Fatigue
Joint stiffness
Rashes, including
the"butterfly rash" across
the cheeks
Sun sensitivity
Hair loss
Raynaud's phenomenon
Internal organ involvement, such
as the kidneys
Blood disorders
Seizures or strokes
Sjogren's symptoms and signs
Dry eyes
Eye irritation and burning
Dry mouth
Dental decay, gum disease
Swelling of the parotid
glands on the sides of the
face
Joint pain and stiffness
(rarely)
Internal organ diseases
(rarely)
Accessory Examination:
1. Autoantibodies
(1) anti-nuclear antibodies (ANA)
directed against contents of the cell nucleus
The normal titer of ANA is 1:40 or less.
ANA are present in low titers in some of the general population.
Higher titers are indicative of an autoimmune disease:
prevalence of ANA(+)
SLE (80%-90%)
Sjögren’s syndrome (60%)
RA (30%-40%)
SSc, PM, and DM (30%)
MCTD
(2) anti-ENA (Extractable nuclear antigen)
Anti-Ro (SS-A)
Anti-La (SS-B)
Anti-Sm (Smith antigen)
Anti-nRNP (nuclear ribonucleoproteins)
Anti Scl-70 (topoisomerase I)
Anti-Jo -1
Anti-centromere
Accessory Examination:
1. Autoantibodies
X-rays
Ultrasound
CT
MRI
CTA and MRA
Accessory Examination:
2. Imaging examination
Accessory Examination:
3. Pathology
Pathology aspirated or make biopsy from
affected tissues
How to make the diagnosis of
CTD ?
Each of these diseases has a “classic” presentation
with typical findings of physical examination.
Each also has various typical blood test abnormalities
and a variety of abnormal antibodies that are
commonly found in blood.
SLE：1984 ACR classfication criterion
RA: 1987 ACR classfication criterion
Treatment
Most rheumatic diseases are treated with
NSAIDs (Non-Steroid Anti-Inflammatory Drugs)
glucocorticoids (in serious cases)
DMARDs (Disease-Modifying Anti-Rheumatic Drugs)
Biologic agent: monoclonal antibodies, such as infliximab and
adalimumab, and the soluble TNF receptor etanercept
Rituximab (Anti-B-Cell Therapy）
Patients with rheumatic diseases often need a long
term, coordinated and a multidisciplinary team
approach towards management of individual patients.
Treatment is often tailored according the individual
needs of the individual patient which is also
dependent on the response and the tolerability of
medications.
Treatment
Key Points
Rheumatology has developed rapidly with great advancements in
immunology and molecular biology, new discoveries in new biomarkers,
and new treatment modalities.
Connective tissue disease (CTD) is a major focus of rheumatic diseases,
often affecting joints, musle , connective tissue, soft tissue around the joints
and bones.
It is likely that a combination of genetic risks and environmental factors are
necessary for the development of CTD.
Key points
Rheumatic diseases are characterized with the overactivity of the immune
system that results in the production of extra antibodies into the circulation.
Treatments of rheumatic diseases need a multidisciplinary and individual
management.
Practices
Please state the definition of rheumatic
diseases.
Please state the etiology of rheumatic
diseases.
