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Joint Pain/ Rash/ Brittle AsthmaJoint Pain and RashJoint Pain and Rash History • Joint Pain • Site: which joints involved • Symptom onset: acute vs chronic (6 wks) • Inflammatory symptoms: early morning stiffness >30 minutes, improves with activity worsens with rest, swelling/reddness of joint • Rash • What does it look like? Where is it? Any photos? • Exacerbating/ relieving factors? • Any new medications or new chemicals? • Sunlight exposure?Systemic Erythematous Lupus What is it • SLE is a long-term autoimmune condition • Immune system which normally protects us against illness and infection, starts to attack the body's own tissues instead • In lupus, the autoimmune system targets multiple different organs (hence systemic) such as kidneys, lungs, skin and joints Who is more at risk? • Female • >30v • African descentPresentation • Symptoms have a relapsing remitting course • Main symptoms • Joint pains (early morning stiffness); myalgia • Photosensitive malar rash • Fatigue • Other symptoms • Constitutional: weight loss, fatigue • Cutaneous: oral ulcers, Raynauds, hair loss • Psychiatry: anxiety, depression • Renal: peripheral oedema (nephrotic syndrome)Differential diagnosis • Rheumatoid arthritis • Both present with inflammatory arthritis • Systemic sclerosis • Both have Raynaud's • Differences: SLE (rash); systemic sclerosis (sclerodactyly and calcinosis) • Antiphospholipid syndrome • Both have recurrent VTE • Antiphospholipid syndrome recurrent miscarriagesInvestigations General bloods • FBC: anemia, leukopenia, thrombocytopenia • U+Es: identify renal involvement --> elevated urea and creatinine • ESR and CRP: ESR usually elevated Specific bloods • Antinuclear antibody (ANA) • positive in virtually all patients with SLE • Not diagnostic in itself as positive in other connective tissue disease – used for screening • Anti-dsDNA • highly specific and confirmatory of SLEScreen for other organ involvement • All patients should have urinalysis and BP monitoring to screen for renal disease • All patients with lupus nephritis have proteinuria • Also check for haematuria and casts • CXR and ECG • If patients have signs of cardio/respiratory disease Treatment not curative Patient education • Encourage high factor suncream, exercise, stop smoking, psychological intervention • Learn to recognise triggers • If they want to become pregnant --> make sure it is T reatment planned and check for Ro, La and antiphospholipid antibodies Pharmacological treatments • Hydroxychloroquine: long term medication to reduce inflammation. Patients need regular eye tests • NSAID + PPI: treat inflammation and pain in joints • Advanced disease: DMARDS e.g. methotrexateComplications • Infection • due to disease and immunosuppressant use • Anaemia of chronic disease • SLE affects bone marrow causing chronic normocytic anaemia • Also causes leukopenia (low WCC), neutropenia (low neutrophils) and thrombocytopenia (low platelets) • Pericarditis • Look for pleuritic chest pain, relieved by leaning forward, worse lying down • Pleuritis • Pleuritic chest pain, relieved by taking shallow breathes, worse when coughing • Lupus nephritis • Recurrent miscarriage • Complication of development of antiphospholipid syndrome • Blood clotsLupus Nephritis What is it • Occurs when lupus autoantibodies affect structures in your kidneys that filter out waste Symptoms (nephrotic syndrome) • Haematuria • Proteinuria: ask about foamy urine • Oedema: Having extra fluid that your kidneys cannot remove that causes swelling in body parts like your legs, ankles, or around your eyes. • Weight gain: due to the fluid your body is not able to get rid of. • High blood pressureAntiphospholipid syndrome • Predisposition to both venous and arterial thromboses • Presentation • History of recurrent thrombosis • Miscarriages • History SLE • Arthralgia/arthritisInvestigations • 1st line • ANA/anti-dsDNA (underlying lupus) • Anticardiolipin antibodies • FBC: thrombocytopenia • Coagulation studies: paradoxical rise in APTT • Other • Venous doppler USS – DVT • CTPA – PE • Management: LWMHPractice questionPractice questionJoint Pain and Brittle Asthma • The name for inflammation of the blood vessels. • There are many different types of vasculitis that affect different sizes of blood vessel. • They are categorised based on whether they affect small vessels, medium sized Vasculitis vessels or large vessels. • They each have some unique features that will help you spot them in exams. • Focus on Granulomatosis with polyangiitis (Wegener’s granulomatosis) and Eosinophilic Granulomatosis with Polyangiitis (Churg- Strauss syndrome)Types of vasculitis Types of Vasculitis Affecting The Small Vessels • Henoch-Schonlein purpura • Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome) • Microscopic polyangiitis • Granulomatosis with polyangiitis (Wegener’s granulomatosis) Types of Vasculitis Affecting The Medium Sized Vessels • Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome) • Kawasaki Disease Types of Vasculitis Affecting The Large Vessels • Giant cell arteritis- should know from last year go over came up in our OSCE • Takayasu’s arteritis Presentation of vasculitis There are some generic Purpura-> These are purple- features that apply to most coloured non-blanching types of vasculitis. These are spots caused by blood Joint and muscle pain Peripheral neuropathy Renal impairment things that should make you leaking from the vessels think about a possible under the skin. vasculitis: Gastrointestinal disturbance They are also associated (diarrhoea, abdominal pain Anterior uveitis and scleritis Hypertension with systemic Fatigue and bleeding) manifestations of: There are some features Fever Weight loss Anorexia (loss of appetite) Anaemia that are more specific to individual types and these are discussed laterKey Investigations • Inflammatory markers (CRP and ESR) are usually raised in vasculitis. test to remember for vasculitis.tibodies (ANCA) is the blood • There are two type of ANCA blood tests: p-ANCA and c- ANCA. • P-ANCA are also called anti-MPO antibodies. ANCA tests are associated with different types of vasculitis: • p-ANCA (MPO antibodies): Microscopic polyangiitis and Churg-Strauss syndrome • c-ANCA (PR3 antibodies): Wegener’s granulomatosis • Discuss more detail later This Photo by Unknown Author is licensed under CC BY-NC-NDOverall management • The management of vasculitis depends on the type. Suspected cases should be referred to a specialist, usually a rheumatologist, to guide diagnosis and management. • Treatment usually involves a combination of steroids and immunosuppressants. • Steroids can be administered to target the affected area: • Oral (i.e. prednisolone) • Intravenous (i.e. hydrocortisone) • Nasal sprays for nasal symptoms • Inhaled for lung involves (e.g. Churg-Strauss syndrome) • Immunosuppressants that are used include: • Cyclophosphamide • Methotrexate • Azathioprine • Rituximab and other monoclonal antibodiesCommon vasculitis conditions Know these in a little more detail for exams with specific signs An IgA vasculitis that commonly presents with a purpuric rash affecting the lower limbs or buttocks in children. Inflammation occurs due to immunoglobulin A deposits in the blood vessels of affected organs such as the skin, kidneys and gastro- tonsillitis) or a gastroenteritis. It is most common in children under the age of 10 years. The rash is caused by inflammation and leaking of blood from small blood vessels under the skin, forming purpura. Henoch- schonlein • The four classic features are purpura (100%), joint pain (75%), abdominal pain (50%) and renal involvement (50%). HSP affects the kidneys in about 50% of patients, causing an IgA nephritis. purpura • Management is typically supportive, such as simple analgesia, rest and proper hydration. The benefits of steroids are unclear. (HSP) • The abdominal pain usually settles within a few days. Patients without kidney involvement can expect to fully recover within 4-6 weeks. A third of patients have a recurrence of the disease within 6 months. 1% of patients will go on to develop end stage renal failure. • Palpable rash • Eosinophilic granulomatosis with polyangiitis used to be called Churg-Strauss syndrome and is still often referred to by this name. It is a small and medium vessel Eosinophilic vasculitis. Granulomatosis • It is most associated with Polyangiitis with lung and skin problems but can affect (Churg-Strauss other organs such as kidneys. syndrome) • It often presents with severe asthma in late teenage years or adulthood. A characteristic finding is elevated eosinophil levels on the full blood count.Granulomatosis with polyangiitis (Wegener’s granulomatosis • A small vessel vasculitis. It affects the respiratory tract and kidneys. • In the upper respiratory tract it commonly affects the nose causing nose bleeds (epistaxis) and crusty nasal secretions, ears causing hearing loss and sinuses causing sinusitis. A classic sign in exams is the saddle shaped nose due to a perforated nasal septum. This causes a dip halfway down the nose. • In the lungs it causes a cough, wheeze and haemoptysis. A chest x-ray may show consolidation and it may be misdiagnosed as pneumonia. • In the kidneys it can cause a rapidly progressing glomerulonephritis. • Crescentic glomerulonephritis (often called rapidly progressive glomerulonephritis) is a serious complication of ANCA-associated vasculitides. This can present with both nephrotic and nephritic features clinically. Light microscopy shows crescent formation in the glomerulus. High-dose immunosuppression is required for this. • Kawasaki disease is a medium vessel vasculitis. It affects young children, typically under 5 years of age. There is no clear cause. This is the most common form of vasculitis in children a • Clinical features are: • Persistent high fever > 5 days • Erythematous rash • Bilateral conjunctivitis • “Strawberry tongue” (red tongue with prominent papillae)les Kawasaki • Oral mucous membrane changes (e.g. strawberry tongue) • Peripheral extremity changes (erythema, oedema and/or disease periungual desquamation of the hands/feet) • Polymorphous rash • Cervical lymphadenopathy • A key complication is coronary artery aneurysms. Treatment is with aspirin and IV immunoglobulins. • Takayasu’s arteritis is a form of large vessel vasculitis. It mainly affects the aorta and its branches affects woman (>80%) and is mainly seen in patients of Asian ethnicity. • These large vessels and their branches can swell and form aneurysms or become narrowed and blocked. T akayasu's This leads to it’s other name of “pulseless disease”. • It usually presents before the age of 40 years with arteritis non-specific systemic symptoms, such as fever, malaise and muscle aches, or with more specific symptoms of arm claudication or syncope. It is diagnosed using CT or MRI angiography. Doppler ultrasound of the carotids can be useful in detecting carotid disease.