Rheumatology Flashcards
Osteoarthritis note:
Osteoarthritis is not an inflammatory condition like RA. It occurs in the synovial joints and is a result of a combination of genetic factors, overuse and injury.
RF’s for osetoarthritis ?
-Obesity
-Age
-Occupation
-Trauma
-Being female
-Family history
It is thought that osteoarthritis is the result of an imbalance between the cartilage being worn down and the chondrocytes repairing it, leading to structural issues in the joint. These abnormalities can be seen on an xray, what are they ?
Four key Xray changes (LOSS):
-L - Loss of joint space
-O - Osteophytes
-S - Subarticular sclerosis (increased density of the bone along the joint line)
-S - Subchondral cysts (fluid filled holes in the bone)
Note: Xray changes do no necessarily correlate with symptoms. Significant xray changes might be found incidentally in someone without symptoms. Equally, someone with severe symptoms of osteoarthritis may have only mild changes on an xray.
Presentation of osteoarthritis ?
It presents with joint pain and stiffness. This pain and stiffness tends to be worsened by activity in contrast to inflammatory arthritis where activity improves symptoms. It also leads to deformity, instability and reduced function in the joint.
What joints are commonly affected in osteoarthritis ?
-Hips
-Kness
-Sacro-iliac joints
-Distal interphalangeal joints (DIPs)
-The MCP joint at the base of the thumb
-Wrist
-Cervical spine
Signs of osteoarthritis in the hands ?
-Heberdens nodes (in the DIP joints)
-Bouchards nodes (in the PIP joints)
-Squaring at the base of the thumb at the carpo-metacarpal joint
-Weak grip
-Reduced range of motion
Note: The carpo-metacarpal joint at the base of the thumb is a saddle joint with the metacarpal bone of the thumb sat on the trapezius bone, using it like a saddle. It gets a lot of use from everyday activities. This makes it prone to wear when used for complex movements.
Diagnosis of osteoarthritis ?
NICE (2014) suggest that a diagnosis can be made without any investigations if the pt is over 45, has typical activity related pain and has no morning stiffness or stiffness lasting less than 30 minutes.
Management of osteoarthritis ?
Start with patient education about the condition and advise on lifestyle changes such as weight loss if overweight to reduce the load on the joint, physiotherapy to improve strength to support the joint and occupational therapy and orthotics to support activities and function.
Stepwise use of analgesia to control the symptoms:
1. Oral paracetamol and topical NSAIDs or topical capsaicin (chilli pepper extract)
2. Add oral NSAIDs and consider also prescribing a PPI to protect their stomach such as omeprazole. They are better used intermittently rather than continuously.
3. Consider opiates such as codeine and morphine. These should be used cautiously as they can have significant side effects and pts can develop dependence and withdrawal. They also don’t work for chronic pain and result in pts becoming dependent without benefitting from pain relief
Intra-articular steroid injections provide a temporary reduction in inflammation and improve symptoms.
Joint replacement can be used in severe cases. The hip and knee are the most commonly replaced joints.
What is rheumatoid arthritis ?
It is an autoimmune condition that causes chronic inflammation of the synovial lining (synovitis) of the joints, tendon sheaths and bursa. It is a symmetrical polyarthritis. Inflammation of the tendons increases the risk of tendon rupture.
Is RA more common in men or women, at what age does it usually present and is family history relevant ?
It is three times more common in women than men.
It most often develops in middle age but can present at any age. Family history is relevant and increases the risk of RA.
Genetic associations of RA ?
HLA DR4 (a gene often present in RF positive pts)
HLA DR1 (a gene occasionally present in RA pts)
What is rheumatoid factor, what does it target,what does this lead to and what class of immunoglobulin is RF normally ?
It is an autoantibody presenting in around 70% of RA pts. It targets the Fc portion of the IgG antibody. This causes activation of the immune system against the pts own IgG resulting in systemic inflammation. RF is most often IgM, however they can be any class of immunoglobulin.
What are anti-citrullinated cyclic antibodies (anti-CCP antibodies) ?
Autoantibodies that are more sensitive and specific to RA than RF. Anti-CCP antibodies often pre-date the development of the RA and give an indication that a pt will go on to develop RA at some point.
Presentation of RA ?
It typically presents with a symmetrical distal polyarthropathy. The key symptoms are joint:
-Pain
-Swelling
-Stiffness
Pts usually attend complaining of pain and stiffness in the small joints of the hands and feet, typically the wrist, ankle, MCP and PIP joints in the hands. They can also present with larger joints affected such as the knees, shoulders and elbows. The onset can be very rapid (i.e. overnight) or over months to years.
There are also associated systemic symptoms:
-Fatigue
-Weight loss
-Flu like illness
-Muscle aches and weakness
TOM TIP:
Pain from an inflammatory arthritis is worse after rest but improves with activity. Pain from a mechanical problem such as osteoarthritis is worse with activity and improves with rest.
What is palindromic rheumatism, how long does it last and what may indicate that it will progress to full RA ?
This involves self limiting short episodes of inflammatory arthritis with joint pain, stiffness and swelling typically affecting only a few joints. The episodes typically only last 1-2 days and then completely resolve. Having positive antibodies (RF and anti-CCP) may indicate that it will progress to full RA.
Common joints affected by RA ?
-PIP joints
-MCP joints
-Wrist and ankle
-MTP joints
-Cervical spine
-Large joints can also be affected such as the knee, hips and shoulders
TOM TIP
The DIP joints are almost never affected by RA. If you come across enlarged painful DIP joints this is most likely to be Heberden’s nodes due to osteoarthritis.
What is atlantoaxial subluxation ?
It occurs in the cervical spine of RA pts. The axis (C2) and the odontoid peg shift within the atlas (C1). This is caused by local synovitis and damage to the ligaments and bursa around the odontoid peg of the axis. Subluxation can cause spinal cord compression and is an emergency. This is particularly important if the pt is having a general anaesthetic and requiring intubation. MRI scans can visualise changes in these areas as part of pre-operative assessment.
Signs in the hands of RA ?
- Z shaped deformity to the thumb
- Swan neck deformity (hyperextended PIP with flexed DIP)
- Boutonnieres deformity (hyperextended DIP with flexed PIP)
-Ulnar deviation of the fingers from the MCP joints
Extra-articular manifestations of RA ?
- Pulmonary fibrosis with pulmonary nodules (Caplan’s syndrome)
-Bronchiolitis obliterans (inflammation causing small airway destruction)
-Felty’s syndrome (RA, neutropenia and splenomegaly)
-Secondary Sjogren’s Syndrome (AKA sicca syndrome)
-Anaemia of chronic disease
-CVD
-Eye manifestations
-Rheumatoid nodules
-Lymphadenopathy
-Carpal tunnel syndrome
-Amyloidosis
Name some eye related complications of RA ?
-Scleritis
-Episcleritis
-Keratitis
-Keratoconjunctivitis sicca
-Cataracts (secondary to steroids)
-Retinopathy (secondary to chloroquine)
The diagnosis of RA is clinical in pts with features of RA (i.e. symmetrical polyarthropathy affecting small joints). Which extra investigations are required at diagnosis ?
-Check RF
-If RF negative, check anti-CCP antibodies
-Inflammatory markers such as CRP and ESR
-X-ray of hands and feet
US scan of the joints can be used to evaluate and confirm synovitis. It is particularly useful where the findings of the clinical examination are unclear.
Xray changes seen in RA (4) ?
-Joint destruction and deformity
-Soft tissue swelling
-Periarticular osteopenia
-Bony erosions
When do NICE recommend a referral of suspected RA and when would it be urgent ?
Any adult with persistent synovitis, even if they have negative RF, anti-CCP antibodies and inflammatory markers.
The referral should be urgent if it involves the small joints of the hands or feet, multiple joints or symptoms have been present for more than 3 months.
Diagnosis of RA note:
Diagnostic criteria come from the American College of Rheumatology (ACR) / European League Against Rheumatism (ELAR) from 2010. Pts are scored based on:
-The joints that are involved (more and smaller joints score higher)
-Serology (RF and anti-CCP)
-Inflammatory markers (ESR and CRP)
-Duration of symptoms (more or less than 6 weeks)
Scores are added up and a score greater than or equal to 6 indicates a diagnosis of RA.
What is the DAS28 score + what is it used for ?
It is the disease activity score. It is based on the assessment of 28 joints and points are given for:
-Swollen joints
-Tender joints
-ESR / CRP result
It is useful in monitoring disease activity and response to treatment in RA.
What is the health assessment questionnaire (HAQ) ?
The questionnaire measures functional ability. NICE recommend using this at diagnosis of RA to check the response to treatment.
Prognosis of RA ?
Prognosis varies between pts from mild and remitting to severe and progressive. There is a worse prognosis with:
-Younger onset
-Male
-More joints and organs affected
-Presence of RF and anti-CCP antibodies
-Erosions seen on xray.
Management of RA ?
Starting treatment early is associated with better outcomes. It is important to have fully involvement of MDT including specialist nurses, physiotheraphy, OT, psychology and podiatry.
A short term of steroids can be used at first presentation and during flare ups to quickly settle the disease. NSAIDs are often effective but risk upper GI bleeding so are often avoided or co-prescribed with PPIs.
The aim is to induce remission or get as close to remission as possible. CRP and DAS28 is used to monitor the success of treatment. Aim to reduce the dose to the “minimal effective dose” that controls the disease.
NICE guidelines for DMARDS:
- First line is monotherapy with methotrexate, leflunomide or sulfasalazine. Hydroxychloroquine can be considered in mild disease and is considered the “mildest” anti rheumatic drug.
- Second line is 2 of these used in combination
- Third line is methotrexate plus a biological therapy, usually a TNF inhibitor.
- Fourth line is methotrexate plus rituximab.
Note: Pregnant women tend to have an improvement in symptoms during pregnancy, probably due to the higher natural production of steroid hormones. Sulfasalazine and hydroxychloroquine are considered as DMARDs in pregnancy.
Biology therapies used in RA treatment with examples:
Anti - TNF (adalimumab, infliximab, etanercept)
Anti - CD20 (rituximab)
Anti - IL6 (sarilumab)
Anti - IL6 receptor (tocilizumab)
JAK inhibitors (tofacitinib and baricitinib)
TOM TIP:
The most important biologics to remember are the TNF inhibitors adalimumab, infliximab and etanercept and it is also worth remembering rituximab. The others are very unlikely to come up in your exams but are worth being aware of. Just remember they all lead to immunosuppression so pts are prone to serious infections. They can also lead to reactivation of dormant infections such as TB and hepatitis B.
Note:
Orthopaedic surgery used to be an important part of managing RA where joint deformities caused significant problems with function, however the DMARDs and biologics mean that now pts are less likely to progress to that stage.
Discuss how methotrexate is used in the management of RA (include notable side effects) ?
It works by interfering with the metabolism of folate and suppressing certain components of the immune system. It is taken by injection or tablet once a week. Folic acid 5mg is also prescribed once a week to be taken on a different day to the methotrexate.
Notable side effects:
-Mouth ulcers and mucositis
-Liver toxicity
-Pulmonary fibrosis
-Bone marrow suppression and leukopenia (low white blood cells)
-It is teratogenic so needs to be avoided prior to conception in mothers and fathers.
What is leflunomide and how is it used in the management of RA (include notable side effects) ?
It is an immunosuppressant medication that works by interfering with the production of pyrimidine. Pyrimidine is an important component of RNA and DNA.
Notable side effects:
-Mouth ulcers and mucositis
-Increased blood pressure
-Rashes
-Peripheral neuropathy
-Liver toxicity
-Bone marrow suppression and leukopenia (low white blood cells)
-It is teratogenic so needs to be avoided prior to conception in mothers and fathers.
What is sulfasalazine (include notable side effects) ?
It works as an immunosuppressive and anti-inflammatory medication. It appears to be safe in pregnancy however women need adequate folic acid supplementation.
Notable side effects:
-Temporary male infertility (reduced sperm count)
-Bone marrow suppression
What is hydroxychloroquine and how is it used in the management of RA (include notable side effects) ?
It is traditionally an anti-malarial medication. It acts as an immunosuppressive medication by interfering with Toll-like receptors, disrupting antigen presentation and increasing the pH in the lysosomes of immune cells. It is thought to be safe in pregnancy.
Notable side effects:
-Nightmares
-Reduced visual acuity (macular toxicity)
-Liver toxicity
-Skin pigmentation
How do anti-TNF drugs work and name some examples (include side effects)?
Tumour necrosis factor is a cytokine involved in stimulating inflammation. Blocking TNF reduces inflammation. Some examples of anti-TNF drugs are:
-Adalimumab
-Infliximab
-Golimumab
-Certolizumab pegol
-Entanercept
Notable side efects:
-Vulnerability to severe infections and sepsis
-Reactivation of TB and hep B
Note: Adalimumab, infliximab, golimumab and certolizumab pegol are monoclonal antibodies to TNF. Entanercept is a protein that binds TNF to the Fc portion of IgG and thereby reduces its activity.
What is rituximab, what is it used for and name some notable side effects ?
It’s a monoclonal antibody that targets the CD20 protein on the surface of B cells. This causes destruction of B cells. It is used for immunosuppression in autoimmune conditions such as RA and cancers relating to B cells.
Notable side effects:
-Vulnerability to severe infections and sepsis
-Night sweats
-Thrombocytopenia
-Peripheral neuropathy
-Liver and lung toxicity
(RA management ) TOM TIP: There are a lot of side effects to remember for your exams. Many of them are shared between medications. Try to remember the unique ones as these are more likely to be tested
Methotrexate: pulmonary fibrosis
Leflunomide: HTN and peripheral neuropathy
Sulfasalazine: Male infertility (reduces sperm count)
Hydroxychloroquine: Nightmares and reduced visual acuity
Anti-TNF medications: Reactivation of TB or hep B
Rituximab: Night sweats and thrombocytopenia
What is psoriatic arthritis ?
An inflammatory arthritis associated with psoriasis.
Psoriatic arthritis can vary in severity. Pts may have a mild stiffening and soreness in the joint or the joint can be completely destroyed in a condition called what ?
Arthritis mutilans
How common is psoriatic arthritis in pts that have psoriais, when does it usually occur / at what age ?
It occurs in 10-20% of pts with psoriasis and usually occurs within 10 years of developing the skin changes. It typically affects people in middle age but can occur at any age.
Psoriatic arthritis is part of what group of conditions ?
The seronegative arthropathies
Patterns of presentation of psoriatic arthritis ?
The condition does not have a single pattern of affected joints in the same way as osteoarthritis or rheumatoid. There are several recognised patterns:
Symmetrical polyarthritis - presents similarly to RA and is more common in women. The hands, wrists, ankles and DIP joints are affected. The MCP joints are less commonly affected (unlike rheumatoid).
Asymmetrical pauciarthritis - affecting mainly the digits and feet. Pauciarthritis describes when the arthritis only affects a few joints.
Spondylitic pattern - is more common in men. It presents with:
-Back stiffness
-Sacroiliitis
-Atlanto-axial joint involvement
Other areas can be affected:
-Spine
-Achilles tendon
-Plantar fascia
Signs of psoriatic arthritis ?
-Plaques of psoriasis on the skin
-Nail pitting
-Onycholysis (separation of the nail from the nail bed)
-Dactylitis (inflammation of the full finger)
-Enthesitis (inflammation of the entheses, which are the points of insertion of tendons into bone)
Other associations of psoriatic arthritis ?
-Eye disease (conjunctivitis and anterior uveitis)
-Aortitis
-Amyloidosis
Screening tool for pts with psoriasis to screen for psoriatic arthritis + what would a high score trigger ?
Psoriasis epidemiological screening tool (PEST)
A high score triggers a referral to a rheumatologist
Xray changes seen in psoriatic arthritis ?
-Periostitis - causes a thickened and irregular outline of bone
-Ankylosis - is where bones join together causing joint stiffening
-Osteolysis - is destruction
-Dactylitis
-Pencil in cup appearance
Note: The classic xray change to the digits is the “pencil-in cup appearance”. This is where there are central erosions of the bone beside the joint. This causes the appearance of one bone in the joint being hollow and looking like a cup whilst the other is narrow and sits in the cup.
What is arthritis mutilans, where does it occurs and what happens ?
The most severe form of psoriatic arthritis. It occurs in the phalanxes. There is osteolysis of the bones around the joints in the digits. This leads to progressive shortening of the digit. The skin then folds as the digit shortens giving an appearance that is often called a “telescopic finger”
Management of psoriatic arthritis ?
Similar to the management of RA. There is a crossover between the systemic treatments of psoriasis and treatment of psoriatic arthritis. Treatment is often coordinated between dermatologists and rheumatologists.
Depending on the severity the pt might require:
-NSAIDs for pain
-DMARDS (methotrexate, leflunomide or sulfasalazine)
-Anti-TNF medications (etanercept, infliximab or adalimumab)
-Ustekinumab is last line (after anti-TNF medications) and is a monoclonal antibody that targets interleukin 12 and 23
What is reactive arthritis ?
Where synovitis occurs in the joints as a reaction to a recent infective trigger.
It used to be known as Reiter syndrome
Presentation of reactive arthritis ?
It typically causes an acute monoarthritis, affecting a single joint in the lower limb (most often the knee) presenting with a warm, swollen and painful joint.
Obvious differential diagnosis to reactive arthritis + difference between the two ?
Septic arthritis (infection in the joint). In reactive arthritis there is no joint infection.
Common infections that trigger reactive arthritis ?
Gastroenteritis and sexually transmitted infections. Chlamydia is the most common sexually transmittted cause of reactive arthritis.
Reactive arthritis is part of what group of conditions + what gene is it linked with ?
The seronegative spondyloarthopathies.
The HLA B27 gene
Associations of reactive arthritis ?
-Bilateral conjunctivitis (non-infective)
-Anterior uveitis
-Circinate balanitis - is dermatitis of the head of the penis
TOM TIP:
These features of reactive arthritis (eye problems, balanitis and arthritis) lead to the saying “cant see, pee or climb a tree”.
Management of reactive arthritis ?
Pts presenting with an acute warm, swollen, painful joint need to be treated according to the local “hot joint” policy. This will involve giving antibiotics until the possibility of septic arthritis is excluded. Aspirate the joint and send a sample for gram staining, culture and sensitivity testing to exclude septic arthritis.
Aspirated fluid can also be sent for crystal examination to look for gout and pseudogout.
Management of reactive arthritis when septic arthritis is exclude:
-NSAIDs
-Steroid injections into the affected joints
-Systemic steroids may be required, particularly where multiple joints are affected.
Most cases resolve within 6 months and don’t recur. Recurrent cases may require DMARDs or anti-TNF medications.
What is ankylosing spondylitis ?
An inflammatory condition mainly affecting the spine that causes progressive stiffness and pain
AS is part of which group of conditions + is linked to what gene
The seronegative spondyloarthropathies
The HLA B27 gene
Key joints affected by AS ?
The sacroiliac joints and the joints of the vertebral column.
AS can progress and cause what of the spine and sacroiliac column ?
Fusion
Fusion of the spinal bodies gives what classical xray finding ?
“Bamboo spine”
Presentation of AS ?
Typically a young male in their late teens or 20s. It affects males three times more often than females. Symptoms gradually develop over more than 3 months.
The main presenting features are lower back pain and stiffness and sacroiliac pain in the buttock region. The pain and stiffness is worse with rest and improves with movement. The pain is worse at night and in the morning and may wake them from sleep. It takes at least 30 minutes for the stiffness to improve in the morning and it gets progressively better with activity through the day.
Symptoms can fluctuate with flares of worsening symptoms and other periods where symptoms improve.
Vertebral fractures are a key complication of AS.
Associations of AS
-Systemic symptoms - such as weight loss and fatigue
-Chest pain - related to costovertebral and costosternal joints
-Enthesitis - inflammation of the entheses. This is where tendons or ligaments insert in to bone. This can cause problems such as plantar fasciitis and achilles tendonitis.
-Dactylitis - inflammation in a finger or toe
-Anaemia
-Anterior uveitis
-Aortitis
-Heart block - can be caused by fibrosis of the heart’s conduction system
-Restrictive lung disease - can be caused by restricted chest wall movement
-Pulmonary fibrosis
-IBD
What is schober’s test and what disease is it used in supporting a diagnosis ?
A test used as part of a general examination of the spine.
Have the pt stand straight. Find the L5 vertebrae. Mark a point 10cm above and 5cm below this point. Then ask the pt to bend forward as far as they can and measure the distance between the points. If the distance with them bending forwards is less than 20cm, this indicates a restriction in lumbar movement and will help support a diagnosis of spondylitis.
Investigations for AS ?
-Inflammatory markers (CRP and ESR) may rise with disease activity
-HLA B27 genetic test
-Xray of the spine and sacrum
-MRI of the spine can show bone marrow oedema early in the disease before there are any xray changes
Xray changes seen in AS ?
“Bamboo spine” is the typical exam description of the xray appearance of the spine in the later stages of AS.
Xray images in AS can show:
-Squaring of the vertebral bodies
-Subchondral sclerosis and erosions
-Syndesmophytes - are areas of bone growth where the ligaments insert into the bone. They occur related to the liagments supporting the intervertebral joints.
-Ossification of the ligaments, discs and joints
-Fusion of the facet, sacroiliac and costovertebral joints
Management of ankylosing spondylitis ?
Medication:
-NSAIDs - can be used to help with pain. If the improvement is not adequate after 2 to 4 weeks of a maximum dose consider switching to another NSAID
-Steroids - can be used during flares to control symptoms. This could be oral, intramuscular slow release injections or joint injections.
-Anti-TNF medications such as etanercept or a monoclonal antibody against TNF such as infliximab, adalimumab or certolizumab pegol.
-Secukinumab is a monoclonal antibody against IL-17. It is recommended by NICE if the response to NSAIDs and TNF inhibitors is inadequate
Additional management for AS ?
-Physiotherapy
-Exercise and mobilisation
-Avoid smoking
-Biphosphonates to treat osteoporosis
-Treatment of complications
-Surgery is occasionally required for deformities to the spine or other joints.
What is systemic lupus erythematosus ?
An autoimmune inflammatory connective tissue disorder. It is “systemic” because it affects multiple organs and systems, and “erythematosus” refers to the typical red malar rash that occurs across the face.
What course does SLE usually take ?
A relapsing-remitting course, with flares and periods where symptoms are improved
The result of chronic inflammation means that pts with lupus often have a shortened life expectancy. What are the leading causes of death ?
CVD and infection
Pathophysiology of lupus ?
SLE is characterised by anti-nuclear antibodies. These are antibodies to proteins within the persons own cell nucleus. This causes the immune system to target these proteins. When the immune system is activated by these antibodies targeting proteins in the cell nucleus it generates an inflammatory response. Inflammation in the body leads to the symptoms of the condition. Usually inflammation is a helpful response when fighting off an infection, however it creates numerous problems when it occurs chronically and against the tissues of the body.
Presentation of SLE ?
SLE presents with non-specific symptoms:
-Fatigue
-Weight loss
-Arthralgia and non-erosive arthritis
-Myalgia
-Fever
-Photosensitive malar rash - This is a butterfly shaped rash across the nose and checck bones that gets worse with sunlight
-Lymphadenopathy and splenomegaly
-Shortness of breath
-Pleuritic chest pain
-Mouth ulcers
-Hair loss
-Raynaud’s phenomenon
Investigations for SLE ? (7 bullet points)
-Autoantibodies (see more on different flashcard)
-FBC (normocytic anaemia of chronic disease)
-C3 and C4 levels (decreased in active disease)
-CRP and ESR
-Immunoglobulins (raised due to activation of B cells with inflammation)
-Urinalysis and urine protein: creatinine ratio for proteinuria found in lupus nephritis
-Renal biopsy can be used to investigate for lupus nephritis
Autoantibodies associated with SLE ?
-Anti-nuclear antibodies (ANA) - performing an ANA blood test is the initial step in testing for SLE in someone with symptoms of the condition. They can be positive in healthy pts and those with other autoimmune conditions thus a positive result needs to be interpreted in the context of their symptoms.
-Anti-double stranded DNA (anti-dsDNA) is specific to SLE. The levels vary with disease activity so they are useful in monitoring the disease activity and response to treatment.
-If you send a test for antibodies to extractable nuclear antigens (anti-ENA antibodies) the lab will check for antibodies to specific proteins in the cell nucleus. These are all types of antinuclear antibody:
-Anti-Smith (highly specific to SLE but not very sensitive)
-Anti-centromere antibodies (most associated with limited cutaneous systemic sclerosis)
-Anti-Ro and anti-La (most associated with Sjogren’s sndyrome)
-Anti-Scl-70 (most associated with systemic sclerosis)
-Anti-Jo-1 (most associated with dermatomyositis)
Antiphospholipid antibodies and antiphospholipid syndrome can occur secondary to SLE. They can occur in up to 40% of pts with SLE and are associated with an increased risk of VTE.
Diagnosis of lupus note:
You can use the SLICC criteria or the ACR criteria for establishing a diagnosis. This involves confirming the presence of antinuclear antibodies and establishing a certain number of clinical features suggestive of SLE.
Complications of SLE ?
-CVD - is a leading cause of death. Chronic inflammation in blood vessels leads to HTN and coronary artery disease
-INFECTION - is more common in pts with SLE as part of the disease process and secondary to immunosuppressants
-ANAEMIA OF CHRONIC DISEASE -is common in SLE. It affects the bone marrow causing a chronic normocytic anaemia. Pts can also get leucopenia, neutropenia and thrombocytopenia
-PERICARDITIS
-PLEURITIS
-ILD - caused by inflammation in the lung tissue. This leads to pulmonary fibrosis
-LUPUS NEPHRITIS - occurs due to inflammation in the kidney. It can progress to end stage renal failure. It is assessed with a urine protein:creatinine ratio and renal biopsy. The renal biopsy is often repeated to assess response to treatment.
-NEUROPSYCHIATRIC SLE - is caused by inflammation in the CNS. It can present with optic neuritis, transverse myelitis (inflam of the spinal cord) or psychosis.
-RECURRENT MISCARRIAGE - SLE is associated with other pregnancy complications such as IUGR, pre-eclampsia and pre-term labour
-VTE - is particularly associated with antiphospholipid syndrome occurring secondary to SLE.
Treatment of SLE ?
Anti-inflammatory medication and immunosuppression is the mainstay of treatment. There is no cure and the aim is to reduce symptoms and complications. Treatment is usually titrated upwards to find the minimal medication with the least side effects required to control the symptoms
First line treatments are:
-NSAIDs
-Steroids (prednisolone)
-Hydroxychloroquine (first line for mild SLE)
-Suncream and sun avoidance for the photosensitive malar rash
Other commonly used immunosuppressants in resistant or more severe lupus:
-Methotrexate
-Mycophenolate mofetil
-Azathioprine
-Tacrolimus
-Leflunomide
-Ciclosporin
Biological therapies are considered for pts with severe disease or where pts have not responded to other treatments. The main options in SLE:
-Rituximab is a monoclonal antibody that targets the CD20 protein on the surface of B cells
-Belimumab is a monoclonal antibody that targets B-cell activating factor.
What is discoid lupus erythematosus + who is it more common in/ when does it tend to present ?
A non-cancerous chronic skin condition.
It is more common in women and usually presents in young adulthood between ages 20 and 40. It is also more common in darker skinned pts and smokers.
What is discoid lupus erythematosus associated with ?
An increased risk of developing SLE, however this risk is still below 5%. Rarely the lesions can progress to SCC of the skin.
Presentation of discoid lupus erythematosus ?
Lesions typically occurr on the face, ears and scalp. they are photosensitive. They are associated with scarring alopecia (hair loss in affected areas that does not grow back) and hyper or hypo pigmented scars.
The appearance of the lesions are:
-Inflamed
-Dry
-Erythematous
-Patchy
-Crusty and scaling
Management of discoid lupus erythematosus (include diagnosis) ?
Skin biopsy can be used to confirm the diagnosis.
Treatment is with:
-Sun protection
-Topical steroids
-Intralesional steroids
-Hydroxychloroquine
Note:
The terms systemic sclerosis and scleroderma are often used interchangeably. Most pts with scleroderma have systemic sclerosis, however there is a localised version that only affects the skin. Scleroderma translates directly to hardening of the skin
What is systemic sclerosis ?
An autoimmune inflammatory and fibrotic connective tissue disease.
Note: The cause of the condition is unclear. It most notably affects the skin in all areas but it also affects the internal organs
Two main patterns of disease in systemic sclerosis ?
-Limited cutaneous systemic sclerosis
-Diffuse cutaneous systemic sclerosis
What is limited cutaneous systemic sclerosis ?
The more limited version of systemic sclerosis. It used to be called CREST sydnrome. this forms a helpful mnemonic for remembering the features of limited cutaneous systemic sclerosis:
- C - Calcinosis
- R - Raynaud’s phenomenon
- E - oEsophageal dysmotility
- S - Sclerodactyly
- T - Telangiectasia
Diffuse cutaneous systemic sclerosis ?
Includes the features of CREST syndrome plus is affects internal organs causing:
-Cardiovascular problems, particularly HTN and coronary artery disease
-Lung problems, particularly pulmonary HTN and pulmonary fibrosis
-Kidney problems particularly glomerulonephritis and scleroderma renal crisis.
What is scleroderma, what appearance does it give and where are these changes most noticeable ?
Hardening of the skin. This gives the appearance of shiny, tight skin without the normal folds in the skin. These changes are most notable on the hands and the face.
What is sclerodactyly ?
Describes the skin changes that occur in the hands as a result of systemic sclerosis. As the skin tightens around the joints it restricts the range of motion in the joint and reduces the function. As the skin hardens and tightens further the fat pads on the fingers are lost. The skin can break and ulcerate
What are telangiectasia ?
Dilated small blood vessels in the skin. They are tiny veins that have dilated. They have a fine, thready appearance.
What is calcinosis ?
Where calcium deposits build up under the skin. This is most commonly found on the fingertips
