Rheumatoid arthritis

Definition:

- It is a chronic inflammatory progressive autoimmune disease

leading to:
• Articular manifestations : symmetrical inflammatory erosive
polyarthritis affecting mainly peripheral joints.
• Extra-articular manifestations: multi organ affected
Etiology:

The etiology isn’t exactly known, but the following factors may play a role
1. Genetic predisposition:
❑ May be +ve family history of the disease.
❑ Commonly associated with HLA-DR4
2. Environmental:
❑ Infections agents Especially with EB virus.
❑Smoking increase risk for RA
3. Immunological mechanisms:
❑Presence of autoantibodies e.g. RF, ACPA
❑Associated with other auto immune disease e.g. hashimotos thyroiditis.
❑Responds to corticosteroid and other immunosuppressive drugs
Pathogenesis

❑Initiating event unknown (may be viral infection) stimulates susceptible T cells.

❑The stimulated T released lymphokines which activate B and T cell proliferation
(IL1, IL6 & TNF)
❑The B cells released IgG (usually disfigured)
❑The immune system released IgM (RF) against the formed IgG the antibody
bonded together to form immune complex.
❑The formed immune complex fixed the complement and engulfed by PNL
cells and lysozymes which initiate synovitis.
❑With chronicity the following changes were developed :
• Angiogenesis
• Accumulation of inflammatory cells in the synovium
• Synovial cell proliferation (rapidly growing pannus)
Pathology:
1- Arthritis:
• Synovitis with joint effusion at the onset.
• Proliferation of the synovial membrane with out growth of granulation tissue ( pannus
formation)
• cartilage destruction with bone erosion.
• Later on, fibrosis occurs leading to ankylosis, deformity, subluxation.
2. Subcutaneous nodules(granuloma):
• formed of central fibrinoid necrosis surrounded by lymphocytes, macrophages & fibroblasts.
3. Arteritis (vasculitis) usually in small arteries.
Clinical picture:-
❑Incidence:
▪ Females are affected 3 times more than males.
▪ The mean age of onset is 30 -40 years.
▪ More in smokers
❑Clinical course:
▪ It is usually life-long with intermittent remissions and exacerbations
❑Manifestations:
1) General manifestations
2 ) Articular manifestations
3) Extra - articular manifestations
1) General manifestations :
Fever, malaise, anorexia, weight loss
2 ) Articular manifestations(3 D):

a. Distribution of joint affection:

• Polyarticular (more than 4 joints), symmetrical, peripheral arthropathy
• It starts in the proximal interphalangeal joints (PIP), metacarpophalangeal joints (MCP),
wrists & ankles.
• The distal interphalangeal joints (DIP) are usually spared.
• Later on proximal big joints may be affected (centripetal)
• The temporo-mandibular, crico-arytenoid, stemo-clavicular & atlanto-axial joints may be
involved.
b. Description:

• The affected joint is painful, tender, red, hot Swollen with limitation of
movement(sign of inflammation).
• Morning stiffness is characteristic for > 1 hour improve with movement(
why)
• Soft tissue tender swelling indicates thickened synovial
c.Deformity:

Due to erosion and destruction of the joint surface, which impairs range of
movement and deformity
• Fusiform finger: swelling of proximal inter phalangeal joint( PIP) (early
sign)
• Ulnar deviation: fingers deviated at MCP joint ( toward the little finger) .
• Boutonniere deformity: (flexion of proximal inter phalangeal joint ( PIP)
and hyperextension of distal inter phalangeal joint ( DIP) ).
Fusiform finger Ulnar deviation
Boutonniere deformity
• Swan neck deformity: hyperextension at PIP joint, hyperflexion at DIP.
• Z-Thumb deformity: fixed flexion and subluxation at the MP joint and
hyperextension at the IP joint
• Hammer toes: subluxation of heads of MTP, shortening of extensor tend
Z-Thumb deformity
 d. Complications:

1. Joint deformities disability and disuse atrophy ( Ms wasting)

2. Atlanto-axial and subaxial subluxation spinal cord compression (occipital headache)
3. Tenosynovitis may cause rupture of tendons
4. Compression of carpal tunnel thenar atrophy, tingling of thumb, index finger,
and middle finger
5. Posterior rupture of knee joint with herniation of synovial membrane tender
swelling of popliteal fossa ( Baker’s cyst) rupture in the calf severe pain
similar to DVT.
Baker’s cyst
g. Subcutaneous nodules :
• Occur in 20 - 25 % o f cases ( only in sero +ve cases ).
• They appear in pressure areas ( e.g. elbows, extensor surface of forearms ) &
in relation to tendon sheaths ( e.g. Achilles tendon & flexor tendons in the
palms causing Triggering o f fingers )
3- Extra-articular manifestations:

1- C.N.S
▪ Psychosis - Chorea
▪ Depression - Epilepsy
▪ Neuropathy - Myopathy
▪ Cerebral vasculitis ----- stroke
▪ Carpal tunnel syndrome: compression of the Median nerve due to hypertrophied
synovium, it may be the first symptom of R.A.
▪ Atlanto-axial syndrome:- spinal cord compression ----- paraplegia
2- Eye:
▪ Sjogren’s syndrome ( dry eye , dry mouth).
▪ Conjunctivitis
▪ Scleritis ----- repeated attack ----- scleromalacia (blue sclera)
3- Chest
❑Hoarseness of voice
❑ pleura: Pleurisy , pleural effusion
❑ Interstitial pulmonary fibrosis
❑ Pulmonary infarction - Pulmonary hypertension
❑ Caplan's syndrome:- rheumatoid nodules with pneumoconiosis (very rare)
4- C.V.S
▪ Pericardium: Pericarditis (effusion)
▪ Myocardium: Myocarditis , cardiomyopathy
▪ Endocardium: Endocarditis
▪ HTN – IHDs (coronary atherosclerosis, vasculitis)
▪ AR occurs due to vasculitis (not endocarditis)
5- Abdomen:
▪ Nausea & vomiting
▪ Esophagitis,
▪ Abdominal pain due to vasculitis
▪ Pancreatitis
▪ Hepatosplenomegaly
▪ Renal (Lupus nephritis)
5- Skin:
▪ Subcutaneous nodule(on pressure site ,extensor tendon) (In RF +VE)
▪ Palmer erythema
▪ Vasculitis: digital gangrene, cutaneous ulcer, purpura
▪ Raynaud's phenomenon
Palmar erythema
7- Blood & L.N:
▪ Anemia:-
- Normocytic normochromic anemia (autoimmune hemolytic anemia)
- Microcytic hypochromic anemia (iron deficiency anemia)
- Macrocytic anemia (Methotrexate)
▪ Neutropenia
▪ Thrompocytosis
8- Renal:
▪ Amyloidosis nephrotic syndrome & may progress to renal failure.
▪ Renal affection 2ry to drugs: NSAIDs, Gold & penicillamine
▪ proteinuria & nephrotic syndrome.
▪ interstitial nephritis.
Investigations:

]) Haematological :
▪ Anaemia ( hypochromic or normochromic).
▪ Pancytopenia in hypersplensim of felly’s syndrome.
▪ Markedly elevated ESR & C-re active protein in disease activity .
2-Serological tests:
a- Rheumatoid factor:
▪ It is an IgM against IgG which is +ve in 85 % of cases
▪ Non specific for RA but usually used for follow up & severity evaluation
▪ High values indicate disease activity and bad prognosis
False positive:
▪ 5% of healthy people 10-20% of people over age 65
▪ Other collagen diseases e.g. SLE, scleroderma,
▪ Bacterial and viral infections.
b- ANA(anti nucler antibodies) : +ve in 20% of cases (not sensitive, not
specific)

c- Recently: anti CCP (cyclic citrullinated peptide) (high specific)

3- X-ray:
▪ Early cases: periarticular destruction and soft tissue shadow
▪ Late findings: obliterated joint space + deformities
4- Aspiration of synovial fluid:
• positive RF
• Increase Proteins & PN Ls ( but culture is sterile ).
• Decrease glucose & complement.
5-X-ray chest:
pleural effusion, ILD