MCQs in Rheumatology: Vasculitis Contributors: These MCQs were written by Dr Kar-Ping Kuet; and were reviewed by Dr Marian Regan. The MCQs were edited by Dr A Abhishek who also facilitated the review process. www.rheumatology.org.uk/education Question 1 A 43 year old lady is referred urgently to the rheumatology outpatient clinic. She is an ex-intravenous drug user. She has recently been diagnosed with hepatitis C and is under the infectious disease team but is yet to receive treatment. Recently, she developed pain of ankles and hands. She also has a palpable purpuric rash on her shins. There is no objective synovitis. Urine dipstick is 1+ protein, 1+ blood. BP is 120/80 mmHg. FBC, U&E and LFTs are normal. CRP and ESR are elevated at 60 mg/l, and 45 mm/hr respectively. ANCA screen is negative (PR3 and MPO awaited), ANA negative, and rheumatoid factor positive 11280. Blood culture – sterile at 48 hours. Which of the following tests is most likely to help establish a diagnosis? 1. 2. 3. 4. 5. Anti-CCP antibody Cryoglobulins Urine for M C & S HIV test Urine protein creatinine ratio Question 2 A 64 year old lady presents with fever and painful lesions on her scalp. She also complains of shortness of breath and chest x-ray shows patchy opacification throughout. She has a history of asthma of over 20 years and previous surgery for polyps. She recalls a similar presentation 3 years ago which was a presumed pneumonia and responded promptly to prednisolone, and antibiotics. She has been well in between otherwise. She also felt ‘viral’ prior to onset of symptoms. Investigations show the following Hb 10.9 g/dl WCC 16.5 x103/ml Plt 455 x105/ml Neut 10.2 x103/ml Eos 4.6 x103/ml CRP 124 mg/l UE and LFT are normal. ANCAs are negative. What is the most likely diagnosis? 1. 2. 3. 4. 5. Atypical pneumonia Granulomatous polyangitis with eosinophilia Polymyalgia rheumatic with underlying pneumonia Sarcoidosis Granulomatous polyangitis www.rheumatology.org.uk/education Question 3 A 52 year old previously healthy non-smoker Caucasian man presents to accident and emergency with shortness of breath, cough and haemoptysis of several weeks. He complains of intermittent fatigue, and pains in his right ankle and knee. On examination, his temperature is 36.80C, BP120/60 mm/Hg, pulse100 beats/min, saturations 92% on room air and respiratory rate is 20/minute. He has synovitis of his ankles, and left knee. There is a palpable purpuric rash on his lower limbs and elbows, his left eye is blood shot, and he has one small oral ulcer. Investigations are as follows Hb 10.1 g/l (13.1-16.6), WCC 10.4 x10 (normal differential count), Plts 600, U&E normal. CRP is 110 mg/l. Urine dipstick is normal. Chest X-ray shows a cavitating mass in the right upper zone. Immunology is pending. Which of the following is the most likely diagnosis? 1. 2. 3. 4. 5. Granulomatous polyangitis Granulomatous polyangitis with eosinophilia Lung malignancy with paraneoplastic vasculitis Right upper lobe pneumonia due to S Aureus Tuberculosis Question 4 A 28 year old lady was diagnosed with Takayasu’s arteritis when she presented 3 years ago with a myocardial infarction and stroke. Her diagnosis was confirmed on MR angiogram. Her inflammatory markers were normal on presentation. She is known to have stenosis of both internal carotid arteries. Her disease is well controlled on mycophenolate 1g BD and 5mg of prednisolone and she is asymptomatic. Most recently her CRP was <6 mg/l and ESR was 8 mg/l. You wish to reassess for disease activity. Which of the following is the most appropriate investigation? 1. 2. 3. 4. 5. CT angiogram Conventional angiography High resolution ultrasonography MR angiogram PET-CT www.rheumatology.org.uk/education Question 5 A 73 year old lady has presented to the medical assessment unit with a 3 month history of fever, weight loss and feeling unwell. She denies any other symptoms. These symptoms started after returning from a holiday in Greece, and she has no other medical history. On examination she looks unwell. Temperature is 37.6°C, BP 120/79, HR 100 beats/min, RR 18/min. There is no rash, or synovitis of the joints. Auscultation of chest is normal and heart sounds reveal no added sounds. Abdominal examination is unremarkable. Hb 8.9 WCC 9.8 Plt 677 MCV 79.9 Alb 28 LFTs normal Ur 15.6 mmol/l Ct 256 mmol/l K 4.5 mmol/l Na 135 mmol/l Urine dipstick protein3+ blood 3+ pANCA is positive with elevated MPO 186 Chest x-ray is normal. Blood cultures x3 no growth after prolonged cultures Transthoracic echocardiogram no vegetation CT of thorax abdomen and pelvis is normal What is the most likely diagnosis 1. Granulomatous polyangitis 2. Granulomatous polyangitis with eosinophilia 3. Goodpasture syndrome 4. Microscopic polyangiitis 5. Polyarteritis nodosa www.rheumatology.org.uk/education Question 6 A 56 year old lady presents with fever, night sweats, and weight loss for 12months. She has raised inflammatory markers. PET CT scan has shown increased uptake in the ascending, arch and descending aorta. She is diagnosed with a large vessel vasculitis. She has been commenced on oral prednisolone and has responded well. You now wish to initiate a steroid sparing agent as adjunctive therapy. Which of the following agents do you consider to be the most appropriate? 1. 2. 3. 4. 5. Azathioprine Cyclophosphamide Leflunomide Methotrexate Mycophenolate mofetil Question 7 An 87 year old female with type 2 diabetes and hypertension presents to the ophthalmology department with sudden loss of vision in her left eye. She also reports jaw pain and malaise. Fundoscopy reveals pale oedematous disc. ESR is 66 mg/l and CRP is 129 mg/l. You are concerned she has giant cell arteritis and arrange a temporal artery biopsy for her. She is concerned about commencing prednisolone because she is diabetic. Which of the following is the most appropriate initial treatment for her? 1. Methyl prednisolone 0.5 gm i.v. O.D. for 3 days 2. Prednisolone 40 mg/day tapered slowly 3. Prednisolone 60mg/day tapered slowly 4. Prednisolone 60mg/day tapered slowly, and methotrexate 7.5/week increased slowly 5. Prednisolone 40mg/day tapered slowly, and methotrexate 7.5/week increased slowly www.rheumatology.org.uk/education Question 8 A 61 year old man with long standing RA is admitted to hospital with anaemia, weight loss, weakness and rash. He is unable to give a clear history about the duration of his symptoms. He suffers with hypertension, and his medications include leflunomide, amlodipine, and tramadol. On examination he has synovitis of the MCP and PIP joints with swan neck deformity of the right index finger. The left small toe is ischaemic with palpable pedal pulses. He also has a left foot drop and a punched out ulcer on the medial malleolus of this right leg. Systemic examination is otherwise normal. Hb 9.9 gm/dl (normocytic, normochromic) WCC 10.3 x 103/ml Platelets 543 x 103/ml CRP 156 mg/l ESR 100 mg/l UE normal LFTs normal Rheumatoid factor 256 Anti-CCP>600 IU/ml ANCA screen negative Urine protein creatinine ratio 4.5mmol/gm What is the most likely diagnosis? 1. 2. 3. 4. 5. Granulomatous polyangitis Granulomatous polyangitis with eosinophilia Malignancy Peripheral vascular disease Rheumatoid vasculitis www.rheumatology.org.uk/education Answers: Q1. 2. Cryoglobulins This lady has hepatitis C which is not currently being treated. The most likely diagnosis is cryoglobulinemia secondary to her underlying hepatitis. The treatment for this would be to treat the underlying cause. Q2. 2. Granulomatous polyangitis with eosinophilia This lady has Granulomatous polyangitis with eosinophilia. She has a history of asthma, nasal polyps, complains of painful nodular scalp lesions and has lung infiltration on x-ray. ANCAs are not always positive, but if positive hepatitis B status should be checked. Q3. 1. Granulomatous polyangitis Granulomatous polyangitis is a multi-system necrotizing granulomatous disease. Clinical manifestations of WG can include ENT, lung, joints, skin and scleritis and can affect any organ. It is always important to consider mimics of vasculitis including malignancy and infection as these can present in similar ways. Q4. 5. PET-CT PET-CT is useful in demonstrating disease activity especially in those individuals with normal inflammatory markers where it is difficult to ascertain whether symptoms are due to disease activity or damage. Q5. 4. Microscopic polyangiitis The most likely diagnosis is microscopic polyangiitis since she has renal involvement with positive pANCA and elevated MPO titres. The other diagnoses would not really fit in with this presentation. Q6. 4. Methotrexate This lady has a large vessel vasculitis. This condition usually requires long term glucocorticoid use. There is a modest role for methotrexate as an adjunctive therapy to steroids. See Eular recommendations for the management of large vessel vasculitis. Ann Rheum Dis 2009; 68 (3) 310-23. Q7. 1. Methyl prednisolone 0.5 gm i.v. O.D. for 3 days This history is very suggestive of giant cell arteritis (GCA). Even if the temporal artery biopsy is negative this may well be due to skip lesions and the patient should still be treated for GCA. Since this lady has already developed visual symptoms you would give i.v methylprednisolone. There is a role for starting methotrexate with corticosteroids in patients with relapsing –remitting GCA, but not at clinical presentation. See BSR guidelines http://www.rheumatology.org.uk/includes/documents/cm_docs/2010/m/2_manageme nt_of_giant_cell_arteritis.pdf Q8. 5. Rheumatoid vasculitis This gentleman has rheumatoid vasculitis. He presents with multi-organ - joints, skin, renal and neurological - involvement. Management would consist of pulsed www.rheumatology.org.uk/education methylprednisolone and i.v. cyclophosphamide. Rheumatoid vasculitis remains a rare but potentially life threatening condition. www.rheumatology.org.uk/education