3. The Most Common Autoimmune Disease
1% population or 3 per 10,000
3:1 female to male
Highest in 3rd and 4th decades
Two peaks incidence
Morbid/mortal disease
Description dates to the 17th century -A New World Disease
4. Rheumatoid arthritis (RA)
Rheumatoid Arthritis (RA) is a chronic inflammatory disorder that may affect
many tissues and organs, but mainly attacks the joints producing an
inflammatory synovitis.
RA mainly affects the joints.
May also produce inflammation in the lungs, pericardium, pleura, and sclera.
Rheumatoid Arthritis is a chronic disease who’s pain intensity and deterioration
of joint structures progress over time often leading to deformations and
disability.
5. Signs and Symptoms of Rheumatoid Arthritis
Tender, warm, swollen joints
Joint stiffness that is usually worse in
the mornings and after inactivity.
Fatigue, fever and weight loss
6. RA Diagnosis & Tests
X Rays of hands and feet are generally performed in people with RA.
Magnetic Resonance Imaging (MRI)
Ultrasounds
Blood Tests: Rheumatoid Factor
8. Osteoarthritis
Most common rheumatic disease and is characterized
by progressive loss of cartilage and reactive changes
at the margins of the joint and in the subchondral bone
Primarily affects weight-bearing joints
such as the knees, hips and lumbosacral spine
In early disease, pain occurs only after joint use
and is relieved by rest
As the disease progresses, pain occurs with
minimal motion or even at rest
9. Classification of OA
Primary OA Secondary OA
Etiology is unknown Etiology is known
• More common than secondary OA Due to a predisposing cause such as:
1) Injury to the joint
• Common-in elders where there is no 2) Previous infection
previous pathology 3) RA
4) CDH
• Its mainly due to wear and tear changes 5) Deformity
occurring in old ages mainly in weight 6)Obesity
bearing joints
10. Clinical features of OA
Pain
Stiffness
Muscle spasm
Restricted movement
Deformity
Muscle weakness or wasting
Joint enlargement and instability
Crepitus
Joint Effusion
14. Systemic Lupus Erythematous (SLE)
A multi-system inflammatory, autoimmune connective
tissue disease that occurs most commonly in women
during their reproductive age.
The hallmark of SLE is its variety of presentation &
autoantibodies.
Essentially any organ system can be affected,
particularly the skin, joints, kidneys, and CNS.
15. Epidemiology of SLE
More common in urban than in rural areas
4 -5 cases per 10,000
Female : male= 9：1 (adult)
Onset age= 65% between 15-25 ys(late onset ?40-55)
Identical twin：30%
First degree relative：5%
Child of SLE mother has risk of SLE
(with positive anti –Ro/SSA antibody) ≈2%
16. Pathophysiology of SLE
Systemic lupus erythematous (SLE) is characterized by a global loss of self-
tolerance with activation of auto reactive T and B cells leading to production of
pathogenic autoantibodies and tissue injury.
Autoimmune reactions directed against constituents of cell nucleus, DNA
17. Clinical Features and Clinical Manifestations
Clinical Features:
• Musculoskeletal disease
• Mucocutaneous involvement
• Renal Disease
• Central nervous system disease
• Cardiopulmonary disease
• Hematologic abnormalities
• Gastrointestinal involvement
Clinical Manifestation:
• Severe fatigue
• Fever
• Weight loss
• Anorexia
• Lymphadenopathy
18. Treatment of SLE
Arthritis, Arthralgias, Myalgias: Glomerulonephritis
NSAIDS, Anti-Malarials (eg. Plaquenil), Steroids
steroids-injections, oral methotrexate Pulse cytotoxic
Mycophenolate mofetil
Photosensitivity, Dermatitis
Avoid Sun exposure CNS disease
Topical steroids, Plaquenil Anti-coagulants for thrombosis
Steroids and cytotoxic for
vasculitis
Weight loss and fatigue Infarction
Steroids Steroids
Cytotoxic
Abortion, Fetal loss Prostacyclin
ASA, Immunosuppression
Thrombosis
Anti-coagulants
19. Sjogren syndrome
A chronic, slowly progressive autoimmune
disease characterized by lymphocytic
infiltration of the exocrine glands resulting in
xerostomia and dry eyes
1/3 have systemic manifestations
Few develop lymphoma
Female-to-male ratio, 9:1
20. 2 Forms: Sjogren syndrome
Primary Sjogren's syndrome
The disease presents alone
Secondary Sjogren's :
Associated with other autoimmune diseases
• RA
• SLE
• Scleroderma
• Mixed CT disease
• Primary biliary cirrhosis
• Vasculitis
• Chronic active hepatitis
21. Sjogren syndrome: Etiopathology
Etiology -not well understood
Findings suggest an on-going interaction between
The innate and acquired immune systems
Lymphocytic (T,B) infiltration of exocrine glands
+
B lymphocyte hyper-reactivity
Inherited susceptibility+ exo /endogenous antigens
Trigger a self-perpetuating inflammatory response
22. Sjogren’s: Clinical Manifestation
Medications that cause similar symptoms
• Antidepressants
• Anticholinergic
• Beta blockers
• Diuretics
• Antihistamines
• Women on HRT
• Anxiety
Glandular: Extra glandular:
• Xerophthalmia • Arthralgias/arthritis
• Xerostomia • Raynaud's phenomenon
• Bilateral parotid swelling • Lymphadenopathy
23. Investigations
Routine:
• Mild normochromic, normocytic anaemia
• ESR rise- in 70%
Mouth:
• Sialometry
• Sialography
• Imaging: Ultrasound, MRI or MR sialography of salivary glands.
• Salivary gland Biopsy
Eye:
• Schirmer test
24. Sjogren’s: Treatment
Artificial tears, Rx -corneal ulcerations
Avoid drugs that secretions (Diuretics, anti HTs,anticholinergic &
antidepressants)
Xerostomia: Best replacement - water
Vaginal dryness: Propionic acid gels
Secretagogues: Oral Pilocarpine / Cevimeline
Arthralgias : HCQ
RTA: Oral Soda bicarb
Systemic vasculitis: Steroids,
immunosuppressives, M Abs
High-grade lymphomas: Chemo (CHOP) + M Abs
25. Dermatomyositis
Dermatomyositis is a disease of the connective-tissue which is defined by
swelling of the skin and muscles.
Dermatomyositis affects adults and children however it is more common in
females than males.
It mostly affects the skin and muscles, but it may also affect the lungs and
oesophagus
26. Dermatomyositis: Symptoms
Skin rash
Symmetric proximal muscle weakness
Muscle pain
Temporary paralysis
Difficulty in swallowing
Scaly skin eruption
Flat, erythematous lesion on the shoulders and chest
Reddish-purple eruption on the upper eyelid
Erythema
Psoriasiform scalp changes
Gastrointestinal infection
Lung problems
27. Diagnostic Criteria
Bohan and Peter Criteria: Symmetric proximal muscle weakness
Typical rash
Elevated serum muscle enzymes
Myopathic changes on EMG
Characteristic muscle biopsy abnormalities and absence of histopathologic signs
of other myopathies
28. Treatment
Improve muscle strength and avoid development of extra muscular
complications
Glucocorticoids are the cornerstone of initial therapy
Typically initiate prednisone at 1 mg/kg to a maximum dose of 80 mg
Initial treatment with high doses for the first several months to establish
disease control
Slow taper to the lowest effective dose for total duration of 9-12 months
First line agents include Azathioprine or methotrexate
More than 80% of patients will improve with glucocorticoids alone
29. Sclerosis
Multisystem disorder
Unknown Etiology
Thickening of skin caused by accumulation of
connective tissue (collagen types I and III)
Involvement of visceral organs
30. Sclerosis: Etiology
Unknown
Environmental Exposures
• Silica exposure in men conferred increased risk
• Silicone breast implants: no definite risk identified
• Aniline laced Contaminated rapseed oil in Spain
• Vinyl chloride exposure increased risk of SSc like disorder: Eosinophilic
Fasciitis
• Bleomycin
• L-tryptophan: Eosinophilia Myalgia syndrome
Genetic Factor
Familial Clustering: 1.5-2.5% of those with 1st degree relative
35. Ankylosing Spondylitis
A systemic rheumatic disease and is one of
the seronegative spondyloarthropathies
The typical patient is young, aged 18-30
Men are affected more than women by
a ratio about of 3:1
36. Signs and Symptoms
Mild to severe back and buttock pain that is
often worse in the early morning hours
Continued inflammation of the:
• Ligaments
• Tendons
• Joint capsules
• joints of the spine
Common symptom is generalized fatigue.
37. Diagnosis
A blood test for the HLA-B27 gene
X-ray
Tomography and magnetic resonance imaging of the sacroiliac joints
Schober's test
38. Treatment
Anti-inflammatory drugs
• NSAIDs such as Aspirin, Ibuprofen, Phenylbutazone, Indomethacin, Naproxen
and COX-2 inhibitors.
DMARDs
• Such as Cyclosporine, Methotrexate, Sulfasalazine, and Corticosteroids
TNFα blockers (antagonists)
• Etanercept, infliximab and Adalimumab
Surgical Management
• Osteotomy for marked deformities of the hip/spine.
• Occasionally, hip or knee Arthroplasty is used
39. Psoriatic Arthritis
Inflammatory arthritis associated with psoriasis
One of the spondyloarthropathies.
Males and females are equally affected.
Psoriatic Arthritis is chronic, progressive
inflammatory disorder affecting the joints and skin
characterized by osteolysis and bony proliferation.
40. Signs and Symptoms
Morning stiffness lasting >30 min in 50% of patients
Patients may present with less joint tenderness than is usually seen in RA
Dactylitis may be noted in >40% of patients2,4
Ridging, pitting of nails, onycholysis – up 90% of patients vs nail changes in only
40% of psoriasis cases2,3
