rheumatoid arthritis (ra)
DESCRIPTION
RHEUMATOID ARTHRITIS (RA). RHEUMATOID ARTHRITIS ( RA ) Gergely Péter dr. Definition: Chro ni c destru ctive diseases characterized by joint inflammation with pain and swelling. In a considerable proportion of patients, the arthritis is progressive, resulting in joint destruction and - PowerPoint PPT PresentationTRANSCRIPT
RHEUMATOID ARTHRITIS (RA)
Gergely Péter dr
RHEUMATOID ARTHRITIS (RA)
Gergely Péter dr
Definition: Chronic destructive diseases characterized by joint inflammation with pain and swelling. In a considerable proportion of patients, the arthritis is progressive, resulting in joint destruction andultimately incapacitation and increased mortality. Relatively common, prevalence: 0.3-1.5 %, the male:female ratio cca. 1:3. Typical case: woman aged 30-40 years with polyarthritis and early joint deformities.
Endogenous factors Exogenous factorsMHC genes, hormon milieu) cross-reacting antigenes,
bacteria, viruses
Synovial vasculitis
Adhesion molecule expression cellular infiltration
Macrophages, T cells, B cells, granulocytes
Cytokines (TNF-, IL-1, IL-6), RF, free-radicals, enzymes
Synovial proliferation, angiogenesis, chondrocyte-, osteoclast-activation
Pannus, cartilage destruction, bone resorption
Pathogenesis of RA
CytokinekinterakcióiCytokineinteractions
Classification criteria of RA (ARA, 1987)
1. Morning stiffness – for at least 1 hr and present for atleast 6 weeks
2. Swelling of 3 or more joints for at least 6 weeks3. Swelling of wrist, metacarpophalangeal (MCP) or
proximal interphalangeal (PIP) joints for at least 6weeks
4. Symmetric joint swelling5. Typical radiologic changes in hands (erosions or
unequivocal bony decalcification)6. Rheumatoid nodules7. Serum rheumatoid factor (RF) positivity
Diagnosis is made by the presence of 4 or more criteria
Differential diagnosis of polyarthritis RA should be differentiated from:
- Other autoimmune diseases (SLE, primary Sjögren’s syndrome, MCTD, PM/DM, PSS, PAN, gian cell vasculitis, polymyalgia rheumatica, adult onset Still’s disease) - Viral diseases (parvovirus B19 infection, rubella, hepatitis B & Cinfection)- Bacterial infections (tbc, rheumatic fever, Jaccoud’s arthritis, septic endocarditis, mycoplasma arthritis)- Seronegative spondylarthritides (erosive psoriatic arthitis, reactive arthritis, enteropathic arthritis)- Paraneoplastic arthritis- Other diseases (e.g. hyperthrophic osteoarthropathy, erythema nodosum, agammaglobulinemia, acromegaly, diabetes mellitus)- Other rheumatic diseases (chronic gout, inflamed erosive osteoarthritis)
Signs of early RA (=undifferentiated arthritis)
Signs of early RA (=undifferentiated arthritis)
In the early stage (within the first 3-6 months) (ARA) classification criteria cannot be used.
The patient should be referred to a rheumatologist, if
• the patient has 3 or more swollen joints• the metacarpophalangeal (MCP) and/or
metatarsophalangeal (MTP) joints are involved; the squeeze test is positive• morning stiffness is 30 min or more.
Squeeze test
Joint involvement in RA
The most specific sign of RA is arthritis.
It is progressive and deforming in the majority (2/3) of cases (= erosive polyarthritis)
RA early stage
Early assymmetric RA
PIP joint involvement in RA
RA: swan neck deformity
RA: ulnar deviation
Ulnar deviation in RA with severe atrophy of interossealmuscles
RA: Boutonnière deformity
RA: arthritis mutilans
Involvement of joints of feet in RA
Severe destruction of ankles in RA
Periarticular osteoporosis (decalcification)
Erosions and sclerosis (in late stage)
Erosion in RA
Early erosions (MRI)
Scinti-graphy ofthe hands
Baker’s cyst
Bursitis in the shoulder
Bursitis and rheumatoid nodule
Rheumatoid nodules
Atlantoaxialsubluxation
RA – end stage
Extraarticular manifestations of RA
• rheumatoid nodules – subcutaneous
- in internal organs (lung, aortic valve)
• pleuritis/pericarditis• fibrotizing alveolitis• Felty’s syndrome• vasculitis• amyloidosis
Systemic manifestations ofRA:pulmonary fibrosis
Interstitial pneumonitis in RA
Systemic manifestations ofRA:Caplan’s syndrome
Rheumatoid nodules in the lungs
Episcleritis in RA
Scleritis in RA
Scleromalacia perforans
Vasculitis in RA
Vasculitis in RA
Leg ulcers in Felty’s syndrome
Large granular lymphocytes in Felty’s syndrome
Disease modifying antirheumatic drugs (DMARD)Disease modifying antirheumatic drugs (DMARD): Drug Adverse effects Dosegold (i.m.) dermatitis, stomatitis, 25-50 mg /2-4
proteinuria, enterocolitis, weeksthrombocytopenia
gold (p.o.) less frequently used, brecause of lower tolerability
chloroquine (hydroxy- retinopathia, pigment- 250 mg/daychloroquine) anomalies
Regular ophthalmology check is requiredd-penicillamine proteinuria, myasthenia, 125-750 mg/day
stomatitisOwing to low tolerability it is not used any more
azathioprine hepatitis, bone marrow depression 50-150 mg/day
Scarcely given in RAmethotrexate hepatotoxicity, pulmonary fibrosis, 7,5-25 (MTX) bone marrow depression mg/week
most frequently used therapy
sulfasalazine nausea, vomiting 1,5-2 g/daydiarrhea, bone marrow depression
cyclosporine A nephrotoxicity, tremor 1,5-4 mg/kg/daycreatinine and blood pressure should bechecked regularly
leflunomide hepatotoxicity, GI 10-20 mg/daycomplaints
TNF- blockers: local reaction, autoimmune disease (SLE, SM)(etanercept, infection (tbc)infliximab, and abatacept)
etanercept: 25 mg 2x weekly s.c.infliximab: 3 mg/kg every 8 week i.v.
Other:anakinra (IL-1 blocker)rituximab (anti-CD20 antibody)abatacept (T cell activation blocker antibody)
Diseases related to RA:
1) Juvenile forms (= juvenile RA, juvenile idiopathicarthritis (JIA)
Subgroups:a) systemic (Still’s disease)b) pauciarticular (<4 joints)c) polyarticular (similar to adult RA)
2) Seronegative (RF negative) forms (seronegative spondarthropathies = SNSA)a) Ankylosing spondylarthritis (Mo Bechterew) b) Psoriatic arthritisc) Reiter’s disease - postinfectious arthritisd) Enteropathic arthritis
Classification criteria of JIA (ARA, 1982)
1. Persistent arthritis of at least 6 weeks duration in one or more joints
2. Exclusion of other causes of arthritis (in particular):a. other systemic autoimmune diseses (SLE, rheumatic fevers, vasculitis, PSS, SS, MCTD, Behçet’s syndrome, PM/DM, SPA, Reiter’s syndroma, psoriatic arthritis)b. Infectious arthritisc. Inflammatory bowel diseasesd. Neoplasms (e.g. leukaemia)e. Nonrhematic conditionsf. Hematologic diseasesg. Psychogenic arthralgiah. Other (sarcoidosis, hyperthrophic osteoarthropathy, villonodular synovitis, chronic aktive hepatitis, familial Mediterranean fever)
Child with advanced polyarticular JIA
Micrognathia in JIA
Typical skin rash inStill’s disease
Inflamed joints with diffuse edema in SNSA (‘sausage-like’ fingers)
Involvement of DIP joint in SNSA
Asymmetric (MTP) arthritis in SNSA
Skin and nails in psoriasis
Exanthema in the rare adult onset Still’s disease