Revision Checklist Topics Flashcards
Rheumatoid arthritis - what antibodies are associated with this condition? What other blood tests may be raised?
What genetic mutations are associated with this condition?
Anti-Rheumatoid Factor and Anti-CCP antibodies. CRP, ESR and pV may also be raised. (NOTE - patients may still have RA and not have anti-CCP antibodies)
HLA-DR1 and HLA-DR4
Name some Rheumatoid deformities of the hands
Ulnar drift
Knuckle and wrist subluxation
Swan-necking
Boutonniere’s
Z-thumbing
Rheumatoid arthritis - what joints are typically affected and what time of day do they seem to be worst? How does activity affect these joints?
Hands - not DIPs
Feet
Sacral spine
C1/C2 - causing atlanto-axial subluxation
Less commonly, the shoulder and hip
Rheumatoid arthritis - other than pathology in the joints, where else might RA present?
Skin - rheumatoid nodules are commonly seen in areas of bony prominence e.g. eblows, heels, knuckles. Also may rarely see pyoderma gangrenosum, erythema nodosum, palmar erythema and atrophy of finger skin
Lungs - pulmonary fibrosis and pleural effusions
Heart and blood vessels - atherosclerosis, various forms of vasculitis, pericarditis, endocarditis, left ventricular failure etc.
Blood - anaemia
Kidneys
Eyes - episcleritis, scleritis
Liver - PBC, autoimmune hepatitis
Rheumatoid arthritis - investigations
Imaging
- Xray
- USS
- MRI (gold standard but expensive)
Serology (remember, may be negative for antibodies but still have RA!)
- Anti-rheumatoid factor, anti-CCP antibodies (best marker, co-relates with disease severity)
- CRP, ESR and pV
Rheumatoid arthritis - what scoring tools exist to grade extent and progression of disease?
EULAR Rheumatoid Arthritis Scoring Criteria - grades amount of joint involvement in small and large joints
DAS28 - monitors progression of disease
- >5.1 = active disease
- 3.2-5.1 = moderate disease
- 2.6-3.2 = mild disease
- <2.6 = remission (target for rheumatologists)
Rheumatoid arthritis - treatment
MTD review required to agree treatment plan
DMARDs within 3 months to prevent deformity
-
methotrexate is first line - 15mg/week initially, scale up to 25mg/week
- also need to give folic acid
- risk of pneumonitis and teratogenicity
- sulfasalazine
- Hydroxychloroquine (HCL) - doesn’t prevent erosions!
- Leflunomide
- Gold injections, azathioprine and others
If Methotrexate is insufficient alone, combine with other DMARDs. If no response after MTX and 2 or more other DMARDs, can use steroids as an adjucnt, and then Biologics
- prior to commencing Biologics, patients must be screened for TB!!!
Osteoarthritis - hallmark signs to look for on Xray
L - loss of joint space
O - osteophyte formation
S - subchondral sclerosis
S - subchondral cyst formation
Osteoarthritis - how do the symptoms of OA differ to those of RA?
What OA deformities may be seen on the hands, and how does this compare to the swelling seen in RA?
OA is gradual onset, taking months-years to develop
OA pain is mechanical, being worse on activity and at the end of the day, unlike RA which improves on activity.
OA also affects the DIPs
OA also causes morning stiffness (like RA), but this lasts less than an hour
Where swelling in RA is soft and boggy, in OA there is usually no joint swelling and instead hard bony swellings are seen
Deformities seen in OA
- Bouchard’s nodes - bony lumps seen at the PIPs
- Heberden’s nodes - bony lumps seen at the DIPs
Osteoarthritis - investigations
History and examination to exclude primary inflammatory arthritis
FBC
Xrays (remember, LOSS)
Osteoarthritis - treatment options
Non-pharmacological
- education
- physiotherapy
- weight loss
- appropriate footwear and walking aids
Pharmacological
- analgesia
- paracetamol
- topical analgesics, compound analgesics
- NSAIDs
- pain modulators
- tricyclics (amitriptyline), anti-convulsants (gabapentin)
- Intra-articular steroids - ONLY for short term relief of symptoms
Surgery
- arthroscopic joint washouts, soft tissue trimming and loose body removal
- partial/total joint replacements
Paget’s Disease - most commonly affected areas
Pelvis
Femur
Lumbar vertebrae
Skull
(may rarely transform into a malignant bone cancer, however Paget’s itself does not spread from bone to bone)
Paget’s Disease - suspected causes and genetic associations
Viral triggers? RSV, Canine Distemper Virus, Measles
Genetic factors? SQSTM1, RANK, specific regions on chromosomes 5 and 6 implicated
Paget’s Disease - signs and symptoms
35% of patients are asymptomatic at time of diagnosis
Most common presentation is bone pain. Rarely, there may also be hearing and vision loss due to nerve compression
Associated conditions: osteoarthritis, heart failure (rare), kidney stones, raised ICP
Paget’s disease - what is usually the first clinical manifestation of the disease seen in bloods?
How is diagnosis confirmed?
Usually first clinical manifestation is raised Alk Phos, while calcium, phosphate, PTH and aminotransferase are typically normal
Imaging is done to confirm diagnosis - Pagetic bone has a characteristic appearance on Xray, and bone scans may be useful in determing severity and extent of the disease
Paget’s Disease - treatment
MTD required
Medication (purpose is to relieve bone pain and prevent progression of disease)
-
Bisphosphonates
- Alendronic acid
- Calcitonin
Surgery may be required
Diet and exercise also seem to play a role
PMR - what condition is commonly associated with Polymyalgia Rheumatica?
GCA (temporal arteritis) is often seen alongside PMR
PMR - signs and symptoms
Wide ranging, typically seen in older patients
Proximal myalgia and stiffness of the hip and shoulder girdles, especially in the morning, lasts for more than an hour and tends to improve as the day goes on
Constant fatigue, weakness and sometimes exhaustion
Lack of appetite, anaemia and a general, non-specifc flu-like illness may be seen
PMR - investigations
No specific test to diagnose PMR, need to exclude other conditions
CRP, ESR and pV may be raised
PMR - treatment
Patients typically respond very dramatically to low-dose steroids (e.g. prednisolone, 15mg/day), suddenly patients feel much better
This can be used as a diagnostic test!
Gradually reduce pred. dose over 18 months
Systemic sclerosis - what are the two classifications of this disease and what antibody is each associated with?
Limited - skin involvement tends to be confined to face, hands, forearms and feet. Organ involvement tends to occur later on
Diffuse Cutaneous- skin changes occur more rapidly and tend to involve the trunk. There is early significant organ involvement
Limited - anti-centromere antibody
Diffuse - anti-Scl-70 antibody, anti-topoisomerase, anti-RNA III polymerase
Diffuse Cutaneous Systemic sclerosis sees much earlier organ involvement than Limited, how might this present?
ILD
Renal crisis due to pulmonary hypertension
GI problems - dysphagia, malabsorption and bacterial overgrowth of small bowel
Inflammatory arthritis and myositis
What did Limited Systemic Sclerosis used to be known as?
What is a common complication of Limited SSc?
Used to be called CREST - calcinosis, Raynaud’s (seen in 90% of patients), Oesophageal dysmotility, Sclerodactyly, Telangiectasia
Pulmonary hypertension is a common complication
Systemic Sclerosis - treatment
No one overall treatment, need to manage the symptoms and complications
Raynaud’s/digital ulcers - CCBs (amlodipine, diltiazem, verapamil), iloprost, bosentan
If renal involvement - ACE inhibitors (-prils)
If GI involvement - PPIs (e.g. omeprazole), H2 receptor antagonists
If ILD - immunosuppression, usually w/ cyclophosphamide.
Need tight control of BP and regular monitoring and screening of organ function
SLE - what is it and who tends to get it more?
Systemic autoimmune disease, can affect any part of the body. Immune system attacks the body’s cells and tissues, antibody-immune complexes form and precipitate, causing further immune response
Who gets it?
- women more than men - 9:1
- higher prevalence in asians, afro-americans, afro-caribbeans and hispanic-americans
- uncommon in african blacks
- genetic factors
- increased incidence amongst relatives
- hormonal factors
- higher incidence in those with increased oestrogen exposure - early menarche, oestrogen-containing contraceptives and HRT
- environmental
- viruses e.g. EBV
- UV light
- Silica dust (cigarettes, cleaning powders, cement)
What important complication needs to be screened for in people with SLE?
Renal disease
SLE has a very varied clinical presentation. What are some of the features that may be seen, and what classification criteria exists to help diagnose SLE?
SLICC Classification Criteria for SLE - require 4 or more of the following (at least one clinical and one laboratory)
Clinical
- acute cutaneous lupus
- chronic cutaneous lupus
- oral or nasal ulcers
- non-scarring alopecia
- arthritis
- serositis
- renal complications
- neurological complications
- haemolytic anaemia
- leukopenia
- thrombocytopenia
Laboratory
- ANA
- Anti-DNA
- Anti-Sm
- Antiphospholipid antibody
- low complement
- Direct Coombs’ test
SLE - what are some of the general constitutional symptoms that may be experienced?
Fever
Malaise
Weight loss and poor appetite
Fatigue
SLE - what are some of the mucocutaneous and musculoskeletal signs associated with SLE?
Mucocutaneous
- Malar rash
- Alopecia
- photosensitivity
- discoid/subcutaneous lupus
- oral/nasal ulceration
- Raynaud’s
Musculoskeletal
- arthralgia
- myalgia
- inflammatory arthritis
- AVN of femoral head (related to steroid use?)
