rheum Flashcards
(25 cards)
RA
systemic cx: constrictive pericarditis IPF benign nail fold vasculitis Felty's syndrome (splenomegaly, neutropenia, leg ulcers) pyoderma gangrenous peripheral neuropathy
drug induced lupus
- eg TNF-a
- ix; anti-histone ab
Up to 95% of patients have antihistone antibodies, which also are present in more than 50% of patients with SLE.
Antibodies to anti-double-stranded DNA, anti-Smith, antiribonucleoprotein, or anti-Ro/SSA or anti-La/SSB antibodies are typically absent
abs - disease
Microsopic POlyangiitis is associated with anti-MPO antibodies.(pANCA)
Wegener’s: with anti PR3.(cANCA)
Renal limited vasculitis: 70-80% with anti-MPO (pANCA)
Anti Jo-1 + ILD = polymyositis (anti-synthetase syndrome)
symmetric, progressive muscle weakness
Interstitial lung disease may precede myopathy or occur early in the disease, often in association with the presence of antibodies to t-RNA synthetases
limited scleroderma
Anti-centromere Ab
-Centromere antibody protective effect against severe ILD
Cardiac and renal disease is RARE
- Lung disease 30%/ Pulm HT 10%
Telangiectasia is the most common feature
Diffuse scleroderma
Anti-topoisomerase(Scl-70)
-Anti-RNA polymerase III is associated with diffuse cutaneous disease and is the most common immunological marker for renal crisis
Tendon friction rubs is the hallmark feature
systemic sclerosis
NSIP
scleroderma
- common Gi - gord
- ILD –> cyclophosphamide
RA
NO DIP ivlved for RA and SLE
Synovial fluid
RA: WCC 2000-10,000 , PMNs >50%.
septic arthritis: WCC >50,000 and PMNs >75%
Clinical triad of Felty’s syndrome
RA,
Neutropenia (<2.0 x 109/L) and
Splenomegaly.
Hydroxychloroquine
No evidence for slowing of radiographic progression with the use of hydroxychloroquine.
major side effect affecting the eyes and this includes:
1) Maculopathy-reduced visual acuity(dose related)
2) Vortex keratopathy(non dose related)
dermatomyositis
Gottron’s sign heliotrope rash Shawl sign Periungal changes Mechanics hands
3 mandatory fx of PMR
Age >50.
Bilateral shoulder ache.
Abnormal CRP +/- ESR
autoab
Microsopic POlyangiitis is associated with anti-MPO antibodies.(pANCA)
Wegener’s: with anti PR3.(cANCA)
Renal limited vasculitis: 70-80% with anti-MPO (pANCA)
R. factor +ve
Rheumatic disorders:
● Rheumatoid arthritis – 26 to 90 percent
● Sjögren’s syndrome – 75 to 95 percent
● Mixed connective tissue disease – 50 to 60 percent
● Mixed cryoglobulinemia (types II and III) – 40 to 100 percent
● Systemic lupus erythematosus – 15 to 35 percent
● Polymyositis or dermatomyositis – 5 to 10 percent
(high titres in RA, Sjogrens, cryoglobulinaemia)
Nonrheumatic disorders :
● Indolent or chronic infection, as with SBE or with hepatitis B or C virus infection. RF production typically ceases with resolution of the infection in these disorders.
● Inflammatory or fibrosing pulmonary disorders, such as sarcoidosis
● Malignancy, particularly B-cell neoplasms
● Primary biliary cholangitis (previously referred to as primary biliary cirrhosis).
Healthy individuals — Rheumatoid factors have been found in up to 4 percent of young, healthy individuals
seronegative spondyloarthropathies
- ankylosing spondylitis
- psoriatic arthritis
- reactive arthritis
- enteropathic spondylitis
- undifferentiated spondyloarthropathy
biological DMARDs
- Tumor Necrosis Factor: Infliximab Etanercept Adalimumab Golimumab Certolizumab
- T cell activation:
Abatacept - Anti CD-20:
Rituximab - Interleukin-6:
Tocilizumab - Interleukin-1:
Anakinra
Abatacept is a fusion protein of CTLA4 and Fc portion of IgG1
It interferes with T cell activation by preferentially binding to CD80/86 on the APC therefore preventing CD80/86 binding to CD28 on T cells.
In short,
Without Abatacept:
CD28 binds to CD80/86 = T cell activation.
But with Abatacept:
CTLA4 binds to CD80/86 = No T cell activation.
CTLA generates a NEGATIVE signal
genes are associated with risk of developing RA
PADI
STAT4
DR4
PTPN22
predictors of severity (erosive damage) of RA
- High titre of RF
- Presence of anti-CCP ab
- Erosions
- ESR and CRP at presentation
- Swollen joint count
- high disability HAQ score
- Rheumatoid nodules
- HLA DR4
Among all of these, erosion is the best predictor of severity.
Risk factors for RA development:
– Positive anti-CCP
– Smoking
– STAT 4 polymorphism.
Wegeners
renal + pulmonary (URT +/- LRT) involvement
Features
- upper respiratory tract: epistaxis, sinusitis, nasal crusting
- lower respiratory tract: dyspnoea, haemoptysis
- glomerulonephritis (‘pauci-immune’, 80% of patients)
- saddle-shape nose deformity
- also: vasculitic rash, eye involvement (e.g. proptosis), cranial nerve lesions
Investigations
- cANCA positive and presence of antiproteinase-3 antibodies > 90%, pANCA positive in 25%
- chest x-ray: wide variety of presentations, including cavitating lesions
- renal biopsy: crescentic glomerulonephritis
Management
- steroids
- cyclophosphamide (90% response)
- plasma exchange
- median survival = 8-9 years
Adult Still’s disease
- typically affects 16-35 year olds
Features
- arthralgia
- elevated serum ferritin
- rash: salmon-pink, maculopapular
- pyrexia
- lymphadenopathy
- rheumatoid factor (RF) and anti-nuclear antibody (ANA) negative
drugs - serum rate
Drugs that raise serum urate concentrations – Diuretics – Tacrolimus – Cyclosporin – Ethambutol – Pyrazinamide – Cytotoxic chemotherapy – Ethanol – Salicylates (low dose) – Levodopa – Ribavirin and interferon – Teriparatide
Drugs that lower serum urate concentrations – Ascorbic acid – Benzbromarone – Calcitonin – Citrate – Oestrogens – Fenofibrate – Losartan – Probenecid – Salicylates (high dose) – Sulfinpyrazone
Basically the diuretics, anti-TB drugs,anti-viral, immunosuppressives and chemo drugs may give rise to increased serum uric acid.
