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R he u m a to o g y finalsinfocus@gmail.comrd @finalsinfocus @finalsinfocusntod uton:Question 1: A 45-year-old woman presents to her GP with joint pain. Her pain initially started in her right knee, but she has now developed pain and stiffness in her 1st and 3rd distal What is the most likely diagnosis? interphalangeal joints of her right hand. Her symptoms are worse in the morning and improve throughout the day. Her A. Rheumatoid arthritis PMH includes Achilles tendonitis, which she is undergoing physiotherapy for. B. SLE On examination, her right 1st and 3rd digits are swollen with maximal tenderness over the distal interphalangeal joints.You also notice that her nails have an abnormal C. Osteoarthritis appearance. D. Psoriatic arthritis Her GP completes a basic rheumatological screen which shows a raised CRP but normal rheumatoid factor and anti- CCP . E. Polymyositis Diagnosis of psoriatic arthritis Joint pain • Symptoms of inflammatory arthritis - joint pain, which is worse after long periods of rest, features prolonged morning stiffness of >30 minutes, and improves with activity. • Mostly affects the DIP joints, RA typically affects the MCPJs • Typically in an oligoarticular pattern but other patterns can exist Skin changes • Psoriasis rash • Nail changes can take several forms • Pictured is onycholysis - the nail plate has separated from the nailbed typically resulting in a well-defined area of white opaque nail • Skin/nail changes are sometimes absent or unreported and psoriatic rash or nail changes are not essential to diagnosis. Features unique to psoriatic arthritis • Dactylitis – sausage fingers • Enthesitis – inflammation of a tendon Investigations • Mostly based on clinical assessment in secondary care by a specialist • Seronegative – think PEAR (psoriatic, enteropathic, ank spond, reactive) • CRP raised in active disease • X-ray of the hands = pencil in cup deformityQuestion 2: A 56-year-old man presents to the emergency department with an acutely painful right knee. On further questioning he tells you the pain started What is the most appropriate treatment? yesterday morning, reaching maximum severity at night, and meant he struggled to sleep at all. He describes the A. Allopurinol pain as stabbing and has also noticed that his knee is red and hot to touch. His PMH includes non-Hodgkin’s lymphoma, for which he is on a chemotherapy regime of B. Naproxen ICE (ifosfamide, carboplatin, and etoposide), and a duodenal ulcer. C. Colchicine On examination, his right knee is grossly swollen and D. Prednisolone erythematous. Observations: RR = 14 breaths per minute, sats 100% on air, BP = 130/94mmHg, HR = 72 beats per minute, and a temperature of 36.9ºC E. Febuxostat Joint aspiration is performed which shows negatively birefringent, needle shaped crystals.P art 1:Diagnosis of gout A 56-year-old man presents to the emergency department with an acutely painful right knee. On ‘Acutely painful right knee’ further questioning he tells you the pain started • Septic arthritis – patient afebrile yesterday morning, reaching maximum severity at night, • Gout and meant he struggled to sleep at all. He describes the • Pseudogout - stems normally mention risk factor pain as stabbing and has also noticed that his knee is red and hot to touch. His PMH includes non-Hodgkin’s haemochromatosis, hyperparathyroidism, lymphoma, for which he is on a chemotherapy regime of acromegaly ICE (ifosfamide, carboplatin, and etoposide), and a • Haemarthrosis – no precipitating trauma duodenal ulcer. mentioned On examination, his right knee is grossly swollen and ‘Non-Hodgkin’s lymphoma, for which he is on a erythematous. Observations: RR = 14 breaths per chemotherapy regime of ICE’ minute, sats 100% on air, BP = 130/94mmHg, HR = 72 • Chemotherapy is a risk factor for gout as it causes beats per minute, and a temperature of 36.9ºC hyperuricaemia, due to increased production of uric acid, released after tumour cell death Joint aspiration is performed which shows negatively (purines converted to urate) birefringent, needle shaped crystals. ‘Negatively birefringent, needle shaped crystals’ • Examination of the fluid aspirate under polarised red light • Pseudo «» Positive (2 P) • Negative «» Needles (2 N’s = gout) • No crystals in septic arthritisP art 2: Management of gout A bit more about gout: • The most common inflammatory arthritis • Triggered following prolonged hyperuricaemia due to purine breakdown which results in the accumulation of monosodium urate (MSU) crystals in the joint • Typically affects 50–60-year-old males • Acute attacks typically affect the first metatarsal-phalangeal joint (MTP of the great toe) (70%) • Pain reaches maximum severity within 24 hours and resolves in 5-15 days • In chronic gout, urate crystals deposit to form tophi surrounding the joint, cartilage and in the joints themselves. Management of gout NSAIDs or colchicine are first line in an acute attack of gout NSAIDs are contraindicated in this patient as they have a duodenal ulcer. Prednisolone is second line (NSAIDs / colchicine ineffective or contraindicated) Allopurinol (1st line) and febuxostat (2nd line) are both used in urate lowering therapy – xanthine oxidase inhibitors.Traditionally thought this makes acute flares of gout worse and delaying urate lowering therapy until inflammation has settled is still recommended in guidelinesQuestion 3: A 37-year-old female attends rheumatology outpatients Given the most likely diagnosis, which of the following a referral from her GP after she presented with a following investigations is most sensitive? number of different symptoms.These included a pruritic facial rash that spared the nasolabial folds, bilateral A. Anti-dsDNA polyarthralgia of the hands and feet and generally feeling B. Rheumatoid factor much more fatigued than normal. She has no relevant PMH C. Anti-histone and is not on any medication. D. Antinuclear antibody E. Anti-SmithQuestion 3: A 37-year-old female attends rheumatology outpatients Most likely diagnosis is systemic lupus erythematous following a referral from her GP after she presented with a • Malar rash – pictured below, key feature sometimes mentioned in exam questions is that it’s nasolabial fold number of different symptoms.These included a pruritic sparing facial rash that spared the nasolabial folds, bilateral • Polyarthralgia – inflammatory pattern, normally polyarthralgia of the hands and feet and generally feeling affecting lots of joints • Constitutional symptoms – fatigue, some patients have much more fatigued than normal. She has no relevant PMH weight loss and malaise also and is not on any medication. • Age demographics – 10:1 female: male ratio, peak incidence 30-40Sensitivity and specificity of SLE blood tests Sensitivity Thinking more about lupus and the question: Sensitivity is like the ability of a test to correctly detect people who really have the In lupus, 99% are ANA positive - this high sensitivity makes it a condition. So, if a test has high sensitivity, it means it's really good at picking up the useful rule out test, but it has low specificity. People with RA, condition in people who truly have it. If a test has low sensitivity, it might miss a lot of systemic sclerosis, polymyositis but even positive in a number people who actually have the condition, giving false-negative results. of different viruses EBV, parvovirus etc If you test 100 people with a disease, a test that is 90% sensitive will pick up 90 of 20% are rheumatoid factor positive those 100 anti-dsDNA: highly specific (> 99%), but less sensitive (70%) A sensitive test helps rule out a disease when the test is negative (e.g. negative amylase in pancreatitis). Highly SeNsitive = SNOUT = rule out. anti-Smith: highly specific (> 99%), sensitivity (30%) Specificity Specificity is about the ability of a test to correctly identify people who don't have the Anti-histone is elevated in drug induced lupus condition. So, if a test has high specificity, it means it's really good at saying someone doesn't have the condition when they really don't. If a test has low specificity, it might wrongly identify people who don't have the condition as having it, giving false- Monitoring = CRP and complement (complement is low as C3 positive results. / 4 form complexes with autoantibodies meaning that free complement is used up) If you test 100 people without the disease, a test that is 90% specific will say that 10% have the disease when they don’t A specific test helps rule a disease in when positive (e.g. urine dipstick for nitrites in UTI). Highly SPecific = SPIN = rule in. In simpler terms, sensitivity is about not missing sick people (true positives), and specificity is about not mislabelling healthy people (true negatives). Both are important for understanding how reliable a test is.Question 4: A 56-year-old woman presents to her GP with . What is the most appropriate management option increasing pain and stiffness in her hands. She is at this stage? currently under secondary care for rheumatoid arthritis, for which she takes methotrexate. She A – IM methylprednisolone admits she has been under more stress recently. B – Oral prednisolone On examination, there is symmetrical swelling, C – Intra articular methylprednisolone stiffness and tenderness of the 2nd, 3rd, and 4th MCPJs and PIPs bilaterally. D – Oral Naproxen E – Topical ibuprofenManaging a flare of RA in GP .NICE guidelines • 1 line = Intra-articular steroid or an intramuscular steroid • Given that this patient has inflammation of multiple small joints in the hand, IA steroids would be inappropriate • 2 ndline = Oral steroids • NSAIDs can be added in alongside steroids but shouldn’t be given as monotherapy • All patients should be referred to rheumatology / advice and guidance in GP .Question 5: A 44-year-old male presents to his GP with a persistent dry cough which started 12 weeks ago. He denies any haemoptysis but reports feeling more out of breath than What is the most likely diagnosis? normal. He also reports losing a significant amount of weight and has noticed a new rash appear on his legs, A – Systemic sclerosis which he would like looked at as well. He has no history of foreign travel and no relevant PMH. B – Myeloma Observations: C – Lung Malignancy RR: 18 BP: 130/76 D – TB HR: 78 Temp: 38.0 E – Sarcoidosis Sats: 99 Cardiovascular and respiratory examinations are normal. His calves are SNT, but he has several, tender, raised erythematous nodules on his shins bilaterally. The GP takes some bloods and orders an urgent chest x-ray. The results are as follows: Hb 140 (135-180) Plts 210 (150-400) WBC 7 (4-11) Calcium 2.9 (2.1-2.6) Serum ACE 50 (20-70) .Sarcoidosis: History A 44-year-old male presents to his GP with a persistent dry What is sarcoid? cough which started 12 weeks ago. He denies any • Non-caseating granulomatous inflammatory disease of haemoptysis but reports feeling more out of breath than unknown aetiology normal. He also reports losing a significant amount of weight and has noticed a new rash appear on his legs, • Granulomas are nodules made of macrophages which he would like looked at as well. He has no history of • Granulomas can deposit anywhere in the body causing a foreign travel and no relevant PMH. variety of different symptoms How does it present? - ABUGSLIFE Observations: RR: 18 A – Arthropathy, arrythmias BP: 130/76 HR: 78 B - Bell's Palsy, bilateral hilar lymphadenopathy Temp: 38.0 Sats: 99 U - Uveitis Cardiovascular and respiratory examinations are normal. His calves are SNT, but he has several, tender, raised G - Granulomas, non-caseating erythematous nodules on his shins bilaterally. S - Serum calcium/ ACE elevated The GP takes some bloods and orders an urgent chest x-ray. The results are as follows: L - Lupus Pernio, liver affected Hb 140 (135-180) I - Interstitial fibrosis (upper lobes) Plts 210 (150-400) WBC 7 (4-11) F - Fever, swinging Calcium 2.9 (2.1-2.6) Serum ACE 50 (20-70) . E - Erythema NodosumSarcoidosis: Investigations A 44-year-old male presents to his GP with a persistent dry cough which started 12 weeks ago. He denies any haemoptysis but reports feeling more out of breath than Bloods normal. He also reports losing a significant amount of • FBC may show anaemia, leucopoenia, weight and has noticed a new rash appear on his legs, which he would like looked at as well. He has no history of thrombocytopaenia foreign travel and no relevant PMH. • Hypercalcaemia and hypercalciuria can occur due to increased vitamin D activation Observations: RR: 18 by macrophages in sarcoidosis BP: 130/76 • Raised ALP if liver involved HR: 78 • Serum ACE raised - sensitivity of 60% and Temp: 38.0 Sats: 99 specificity of 70% and are therefore not reliable in the diagnosis of sarcoidosis Cardiovascular and respiratory examinations are normal. His calves are SNT, but he has several, tender, raised Other erythematous nodules on his shins bilaterally. • Chest x-ray - bilateral hilar lymphadenopathy The GP takes some bloods and orders an urgent chest x-ray. • CT – upper zone fibrosis The results are as follows: • Tissue biopsy of granulomas is gold standard Hb 140 (135-180) Plts 210 (150-400) Management WBC 7 (4-11) • Steroids but only used in disease severe Calcium 2.9 (2.1-2.6) Serum ACE 50 (20-70) (organ threatening) .Question 6 A 59-year-old woman is brought to the emergency department by her husband with symptoms of fever, Which drug has caused her presentation? shortness of breath, fatigue, and a sore mouth. She also notices a number of bruises have appeared on her arms and legs in the last few days, without any obvious cause. Her A – Flucloxacillin PMH includes RA, for which she takes methotrexate, and she B – Co-trimoxazole was recently treated for a diabetic foot infection although she can’t remember the name of the drug, she was C – Clarithromycin prescribed to treat it. She has bloods taken which show the following results: D – Doxycycline Hb 86 (115-160) E – Gentamicin Plts 39 (150-400) WBC 2.7 (4-11) Neutrophils 1.9 (2-7) Lymphocytes 0.3 (1-3.5) Monocytes 0.1 (0.2 – 0.8)Methotrexate side effects This patient has bone marrow aplasia as characterised by: . 59-year-old woman is brought to the emergency department by her husband with symptoms of fever, • Pancytopaenia on bloods shortness of breath, fatigue, and a sore mouth. She has also • Fever – increased susceptibility to infections due to low WBC noticed a number of bruises have appeared on her arms • SOB + fatigue – anaemia and legs in the last few days, without any obvious cause. Her • Bruising – low platelets PMH includes RA, for which she takes methotrexate, and she • Sore mouth – characteristic of aplastic anaemia (might suggest was recently treated for a diabetic foot infection although she can’t remember the name of the drug she was thrush, blood blisters or mouth ulcers) prescribed to treat it. She has bloods taken which show the Methotrexate and the trimethoprim component of co-trimox both following results: inhibit folate synthesis through inhibition of dihydrofolate reductase.This disrupts folate metabolism in the bone marrow which Hb 86 (115-160) leads to decreased production of blood cells. Plts 39 (150-400) WBC 2.7 (4-11) Other adverse effects of methotrexate: Neutrophils 1.9 (2-7) Mucositis Lymphocytes 0.3 (1-3.5) Monocytes 0.1 (0.2 – 0.8) Myelosuppression Pneumonitis - most common pulmonary manifestation, similar to hypersensitivity.Typically develops within a year of starting treatment either acutely or subacutely and presents with non-productive cough, dyspnoea, malaise, fever Pulmonary fibrosis Liver fibrosisQuestion 7 A 25-year-old male presents to general practice with a Given the most likely diagnosis, what would you 2-week history of lower limb joint pain.The specific expect to find if you aspirated the right knee joint? joints that are painful are his right knee, left ankle and numerous MTPJs. He also reveals that he is going to A – Staph aureus the toilet much more and that it hurts when he passes urine. He has also been using over the counter eye B – Chlamydia trachomantis drops over the past few days, as his eyes have been red and irritated, and has a new rash on his feet. His C – Neisseria gonorrhoea only PMH is that he was treated for an STI 4 weeks ago. D - Salmonella enteritidis E – No organism growthQuestion 7 A 25-year-old male presents to general practice with a This patient has reactive arthritis: 2-week history of lower limb joint pain.The specific • Asymmetrical polyarthralgia of the lower limbs • Urethritis – dysuria and increased urinary frequency joints that are painful are his right knee, left ankle and • Conjunctivitis – seen in 10-30% numerous MTPJs. He also reveals that he is going to • Can’t see pee or climb a tree the toilet much more and that it hurts when he passes • Keratoderma blennorrhagicum, presenting as hyperkeratotic urine. He has also been using over the counter eye lesions predominantly on the soles and, less commonly, on the drops over the past few days, as his eyes have been palms. red and irritated, and has a new rash on his feet. His • Common trigger of chlamydia infection 4 weeks prior only PMH is that he was treated for an STI 4 weeks ago. Seronegative spondyloarthropathy triggered by either a diarrhoeal illness or chlamydia infection. Not directly caused by the organism – it’s thought that the immune response against the precursor infection causes an inflammatory process in the synovium of the joint pain. Lasts 4-6 months Managed with NSAIDs in the acute phase of illness HLA-B27 associatedQuestion 8 You are an F2 working in rheumatology outpatients. A 35-year-old man attends clinic for a review of his ankylosing spondylitis. His back pain well controlled on oral NSAIDs, but he has read online that Which of the following is not associated with ankylosing spondylitis can affect other parts on his ankylosing spondylitis? body, and he would like to know more. A – Achilles tendonitis B – Heart block C – Anterior uveitis D – Aortic regurgitation E – Bibasal fibrosisAnkylosing spondylitis: 7A ’s Classic presentation is a young man presenting with morning back pain and stiffness that improves throughout the day However, like most rheum conditions, ank spond is a multi-system disease… • A – Anterior uveitis • A – Aortic regurgitation • A – Atrioventricular block (heart block) • A – Apical lung fibrosis (fibrosis of the upper lobes of the lungs) • A – Anaemia of chronic disease • A – Achilles tendonitis • A - Amyloidosis (CKD)