Various rheumatological diseases

1. Rheumatological diseases

2. Rheumatic Diseases
Common Rheumatic Diseases :
 Rheumatoid arthritis (RA)
 Osteoarthritis
 Systemic lupus erythematous (SLE)
 Sjogren syndrome
 Dermatomyositis/polymyositis
 Scleroderma/systemic sclerosis
 Ankylosing spondylitis.
 Psoriatic arthritis

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

7. Treatment
 Surgery
• Removal of inflamed synovium
• Arthroplasty
 Physical therapy
 Cortisone Therapy
 Anti-inflammatory Agents
• Aspirin
• Ibuprofen
• Naproxen
 Acetaminophen
• Tylenol

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

11. Possible Anatomic Sites Of Pain Generation in OA

12. Special Investigations
 Blood tests: Normal
 Radiological features:
1) Cartilage loss
2) Subchondral sclerosis
3) Cysts
4) Osteophytes

13. Treatment
 Education
 Physiotherapy
– Exercise program
– Pain relief modalities
 Aids and appliances
 Medical Treatment
 Surgical Treatment

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

31. Sclerosis: Pathogenesis

32. Forms of Systemic Sclerosis
 Limited Scleroderma
• Crest Syndrome
• Calcinosis
• Raynaud’s
• Esophageal Dysmotility
• Sclerodactyly
• Telangiectasisa
 Diffuse Scleroderma
• Organ Involved
• Skin
• Musculoskeletal
• Pulmonary
• Renal

33. Forms of Systemic Sclerosis
 Limited Scleroderma
• Crest Syndrome
• Calcinosis
• Raynaud’s
• Esophageal Dysmotility
• Sclerodactyly
• Telangiectasisa
 Diffuse Scleroderma
• Organ Involved
• Skin
• Musculoskeletal
• Pulmonary
• Renal

34. Treatment: Sclerosis
 Corticosteroids
 Immunosuppressive Therapy
 Antispasmodics (muscle relaxants)
 Nutritional therapy
 High-protein diet with supplementary vitamins

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

41. Main Features of Psoriatic Arthritis

42. Clinical Features in Psoriatic Arthritis
Dactylitis Enthesitis

43. Treatment
 NSAIDS
 DMARDS
• MTX
• Leflunomide
• Sulfasalazine
• Cyclosporine
• TNF α inhibitor
 Coordinate b/w Rheumatology and Dermatology