009 RARE AUTO-ANTIBODY NEGATIVE SYSTEMIC LUPUS ERYTHEMATOSUS: A CASE REPORT Alison Kinder 1 and Sarah Parker 2 1 Rheumatology, Leicester Royal Infirmary, Leicester, UNITED KINGDOM, and 2 Psychiatry, Leicester Royal Infirmary, Leicester, UNITED KINGDOM Background: Systemic lupus erythematosus (SLE) is an autoimmune disease usually characterised by the presence of autoantibodies and ds-DNA on serological testing. ANA, ENA and ds-DNA negative SLE is rare. A literature search (1970 to present) highlighted just 11 cases. We report a 39 year old Caucasian female with no past medical history who presented in February 2013 with night sweats, 13kg weight loss, fatigue, mouth and nose ulcers, swollen painful fingers, headaches and Raynaud’s phenomenon. She had haematuria and proteinuria on urine dip, was hypertensive and routine bloods showed lymphopenia. Serological tests were normal. She later developed malar and discoid rashes, scleritis, pericarditis, pleurisy, peritonitis, photosensitivity, left knee synovitis and alopecia. She met 8 out of 11 ACR criteria for SLE. A diagnosis of SLE was subsequently made on the basis of clinical presentation and a positive skin biopsy. Methods: Treatment of this patient has been difficult. It commenced with weekly pulses of methylprednisolone and methotrexate. Oral methotrexate was not tolerated and when given subcutaneously, it failed to bring her symptoms under control. Hydroxychloroquine was subsequently commenced but did not control her symptoms. Rituximab and MMF initially put the lupus into remission; however upon restarting exercise and work she became symptomatic. Fortnightly IV cyclophosphamide combined with four monthly infu- sions of rituximab was also unsuccessful in controlling the disease. Although she improved significantly on four-weekly infusions of tocilizumab and 40mg oral prednisolone, she experienced further flares consisting of pericarditis with effusion, peritonitis, pleurisy, synovitis of proximal interphalangeal joints (PIPs) right hand and left knee, malar rash and significant alopecia. A decision was made to increase the frequency of tocilizumab to three-weekly infusions and to reintroduce MMF. Despite a reduction in her joint effusions and feeling better within herself, clinically, she still had pericarditis, synovitis left knee & right PIPs and malar rash. To date, she remains lymphopenic (which lowers further during an acute flare) and since 2017, serological tests show low c3 and c4. Results: Please see the conclusion section. Conclusion: Given most antibody negative SLE cases presented in the literature developed a positive response to subsequent serologic testing within five years, it is too early to state whether our patient will follow suit. However, this case is important as it raises awareness that SLE can initially present as ANA and ENA negative; and in some cases, remain this way. Negative immunology should not prevent or delay the diagnosis and subsequent treatment of SLE when the presentation fits the classical clinical picture. This case also illustrates the therapeutic challenges of treating aggressive cases of SLE. Disclosures: The authors have declared no conflicts of interest. 010 A RARE CASE OF PALMAR FASCIITIS WITH POLYARTHRITIS OF OVARIAN MALIGNANCY Mithun Chakravorty 1 and Shaza Obaid 1 1 Rheumatology, East Midlands Hospital, Lincoln, UNITED KINGDOM Background: A 57 year old Caucasian female presented with rapidly progressing swelling and stiffness in both hands five weeks after injuring her left palm with a thorn during gardening. This was promptly removed but an erythematous patch developed over the same area two weeks later. This progressed to affect multiple small joints of her hands with associated swelling and tightness of the skin. Later, both knees became painful and swollen, and she developed flexion deformity in hands and knees. She was systemically well with no symptoms to suggest underlying connective tissue disease. There were no recent infections, vaccinations or travel abroad. Past medical history included hypothyroidism and epistaxis. There were no new medications or known allergies. She was a non-smoker and teetotal. Her mother had breast cancer aged 48 and her brother was treated for leukaemia. Methods: On examination there was patchy erythema, skin thicken- ing, tightening and tethering of the palms and fingers with some flexion contracture of the fingers. Bilateral hand grips were compromised. Bilateral knee effusions were evident but no other joints were affected. Skin was normal in the forearm and upper arms and she had no telangiectasia. Cardiovascular, respiratory and gastrointestinal exam- inations were unremarkable. Breast examinations did not reveal any masses or lymphadenopathy. Results: Blood tests were unremarkable and she had negative immunology and vasculitic screen. MRI of both hands showed soft tissue oedema but no synovitis. Knee aspirate was negative for microscopy, culture and cytology. Skin biopsy of her left palm showed non-specific neutrophil infiltration and was negative for IgG4. Nail fold capillaroscopy was normal. A CT of her chest, abdomen and pelvis revealed omental thickening and caking, of uncertain significance. Her CA 125 was mildly raised but other tumour markers were normal. Omental biopsy demonstrated high grade serous adenocarcinoma. Conclusion: This was a challenging case with wide differential diagnoses at presentation, requiring multiple speciality input. An erysipeloid reaction was suspected on initial review by dermatology but no improvement was seen with a week of IV tazocin. Eosinophilic fasciitis was considered but skin biopsy and the lack of peripheral blood eosinophilia were not supportive. Whilst awaiting the omental histology, a rapidly progressing scleroderma was considered in view of subpleural fibrosis on HRCT and small pericardial effusion on echocardiogram. However, the absence of Raynaud’s, normal nail fold capillaroscopy and negative immunology were not supportive. Mild improvement was achieved with low dose prednisolone. Her malignancy is suspected to be of peritoneum or fallopian tube in origin. Therefore, her symptoms are likely to be consistent with palmar fasciitis and polyarthritis of ovarian malignancy; a paraneoplastic syndrome. She has recently undergone neoadjuvant chemotherapy prior to surgery and we hope that some of her symptoms will improve after treatment. Disclosures: The authors have declared no conflicts of interest. 011 TAKAYASU ARTERITIS: A CASE SERIES OF UNUSUAL COMPLICATIONS AND COMORBIDITIES Christopher D. Box 1 , Veena Dhillon 1 , Neil McKay 1 , Nicole Amft 1 and Barbara Hauser 1,2 1 Rheumatology Unit, Western General Hospital, Edinburgh, UNITED KINGDOM, and 2 Centre for Genomics and Experimental Medicine, Institute of Genetics and Molecular Medicine, University of Edinburgh, Edinburgh, UNITED KINGDOM Background: Takayasu arteritis (TA) is a rare vasculitis typically presenting in young women. Localised symptoms result from impaired vascular supply, whilst systemic symptoms are also typical. It mainly affects the thoracic aorta and its branches. Co-existing rheumatolo- gical conditions are rarely reported and may lead to diagnostic delay and potentially devastating complications. Methods: We reviewed four cases of patients with Takayasu Arteritis who were seen by the Rheumatology Department in 2017. Results: The first patient presented with fever, back pain, stiff hips and lower limb claudication. MRI of the spine and pelvis showed hip synovitis, enthesitis and inflammation suggestive of axial spondyloar- thropathy (axSpA). CT-angiography showed abnormal appearance bilaterally of the common femoral, superficial femoral, popliteal and crural arteries. Rapid onset and symptom severity required potent immunosuppression with prednisolone (1 mg/kg/day), methotrexate (15 mg orally/week) and tocilizumab (162 mg subcutaneously/week). The second patient was diagnosed with TA in 2006 following left frontoparietal stroke due to right internal carotid artery stenosis. Further investigations revealed additional left subclavian artery stenosis. She received high-dose prednisolone and methotrexate (15 mg/week), later reduced to 7.5 mg methotrexate weekly. Nine years after the initial event she was admitted to ICU due to subarachnoid haemorrhage secondary to a dissecting right vertebral artery aneurysm. She subsequently developed bowel infarction secondary to superior mesenteric artery stenosis. CT-angiogram showed stenosis of the superior and inferior mesenteric arteries. In ITU treatment was escalated to pulsed iv methylprednisolone and monthly iv tocilizumab (8mg/kg). The third patient was diagnosed in 2000 with left subclavian artery stenosis despite radiological evidence since 1993. Additionally she developed thrombosis in the left subclavian artery and was diagnosed with primary antiphospholipid syndrome. Treatment with iv weekly tocilizumab (8 mg/kg/month) and methotrexate had to be stopped due to liver impairment. After 16 months off treatment she presented with fatigue, raised inflammatory markers and repeat CT-angiogram showed new vertebral artery stenosis. Treatment with iv tocilizumab was restarted at a reduced dose (4mg/kg/bimonthly). The fourth patient had been referred to the vascular surgeons with aches in her upper limbs and intermittent hand discolouration. She had been diagnosed with hypertension aged 25. Duplex angiography demonstrated left subclavian and axillary ste- noses and right brachial stenosis. CT-angiography showed wide- spread aortic and arterial wall thickening and stenoses, particularly subclavian and axillary arteries, mild thickening of proximal common iii46 Tuesday 1 May 2018 POSTER VIEWING I Downloaded from https://academic.oup.com/rheumatology/article/57/suppl_3/key075.235/4971135 by guest on 25 April 2022