Background/Aims  Musculoskeletal ultrasound (MSUS) has utility in optimising the use of conventional synthetic disease-modifying antirheumatic drugs (csDMARDs) and biologic DMARDs (bDMARDs) in patients with inflammatory arthritis. However, it is unclear whether this is useful among patients with concomitant chronic pain and/or fibromyalgia, who often have elevated disease activity scores. We aimed to evaluate the impact of MSUS on inflammatory arthritis patients with concomitant chronic pain and/or fibromyalgia who met criteria for treatment escalation in a district general rheumatology service. Methods  We conducted a retrospective audit of inflammatory arthritis patients with concomitant chronic pain and/or fibromyalgia who were eligible for DMARD escalation and underwent a MSUS since 2017. Scanning was performed by either a trained rheumatologist or musculoskeletal ultrasonographer. Synovitis was assessed following OMERACT guidelines. Results  43 patients with inflammatory arthritis and concomitant chronic pain and/or fibromyalgia who underwent MSUS were identified. The mean age was 57.0 years (SD 15.6), and 34 patients (79%) were female. Rheumatoid arthritis was the most frequent diagnosis with 32 patients (74%), with psoriatic arthritis in 5 (12%), undifferentiated inflammatory arthritis in 4 (9%) and axial spondylarthritis (axSpA) in 2 (5%). 20 patients (47%) had a concurrent diagnosis of fibromyalgia. The median tender joint count among non-axSpA patients was 10 (IQR 4-15) and 2 (IQR 0-4) for swollen joints. MSUS was requested for consideration of a bDMARD switch in 21 patients (49%), a new bDMARD in 15 (35%), and starting an adjunctive csDMARD among 7 (16%). 34 patients (79%) were already established on csDMARDs, with 15 patients (35%) being on one or more, and methotrexate being the most prescribed csDMARD in 26 (76%). 14 patients (33%) were already established on bDMARDs, 9 (21%) had been on them previously and 20 (47%) were bDMARD naïve. Among those on bDMARDs, anti-TNFs agents were the most prescribed (71%). Active synovitis was identified in 17 patients (40%). Greyscale synovitis, tenosynovitis and enthesitis were seen in 30 (70%), 10 (23%) and 2 patients (5%), respectively. Erosions were identified in 12 patients (28%), with 2 (17%) having new erosions. 27 patients (63%) had either a csDMARD started (n = 7, 33%), or a bDMARD started (n = 11, 31%) or switched (n = 9, 25%) after MSUS. Those with fibromyalgia were less likely to start or switch DMARDs (8/20 patients, 40%) than those without (19/23 patients, 82.6%), Pchi-squared = 0.004. Furthermore, active synovitis on MSUS was associated with DMARD escalation (14/17 patients [82.4%] with synovitis versus 13/26 patients [50%] without; Pchi-squared = 0.03). Conclusion  MSUS avoided unnecessary DMARD escalation in a significant proportion of patients with inflammatory arthritis and features of concomitant chronic pain and/or fibromyalgia (n = 26, 37%), potentially resulting in reduced patient exposure to harmful DMARD side effects, and cost savings for the service. Disclosure  M. Chen-Xu: None. D. Hyseni: None. K. Achilleos: None.

Case report - Introduction We present the case of a 70-year-old female with a background of anti-Ro positive Sjögren’s syndrome with difficult sicca symptoms who presented with worsening Raynaud’s, bilateral lower limb petechiae/purpura, fatigue, and progressive peripheral neuropathy. Blood tests revealed positive cryoglobulins with hypocomplementemia, and nerve conduction studies objectively confirmed the peripheral neuropathy. The patient was diagnosed with cryoglobulinaemic vasculitis and treated with pulsed intravenous cyclophosphamide and oral prednisolone, which resolved her rash and halted the progression of her symptoms. Cryoglobulinaemic vasculitis is a rare complication of Sjögren’s syndrome occurring in only 3–4% of patients with the disease. Case report - Case description A 70-year-old female with known Sjögren’s syndrome presented with a two-month history of an intermittent red, pin-prick rash affecting her lower legs, worsening fatigue and Raynaud’s, and a progression of longstanding symptoms of pins and needles from her ankles up to her knees, shortly after an acute Epstein-Barr virus infection. Her Sjögren’s syndrome was diagnosed after she presented with difficult sicca symptoms, fatigue and Raynaud’s phenomenon, and strongly positive ANA and anti-Ro antibodies (>240 u/mL). Past medical history was notable for microscopic colitis. On examination, she had bilateral pitting oedema with a purpuric rash affecting both legs. She had reduced sensation to both knees, but with normal power and downgoing plantars. Her joints examined normally. There were no ischaemic changes in her peripheries. Blood tests showed a positive cryoglobulin consisting of a monoclonal IgM paraprotein with polyclonal lambda light chains. She had a normal kappa: lambda ratio and Bence-Jones proteins. Inflammatory markers were raised (CRP 34mg/L, ESR 93mm/hour), with hypocomplementemia (C4 0.05g/L, normal C3). Otherwise, her full blood count, electrolytes, renal and liver function tests, chest X-ray, urine dipstick, hepatitis serology, ANCA profile, B12, folate and ferritin were unremarkable. Nerve conduction studies showed a length-dependent, moderately severe sensory motor axonal peripheral neuropathy, which Neurology agreed was due to a vasculitic process. The patient was diagnosed with a cryoglobulinaemic vasculitis with peripheral nerve involvement secondary to her Sjögren’s syndrome. This was initially treated with prednisolone 40mg daily, intravenous pulsed cyclophosphamide, which resolved her rash and halted the progression of her peripheral neuropathy. Pregabalin was prescribed for pain relief. After completing six cycles of cyclophosphamide, the patient was commenced on azathioprine. This was then replaced with mycophenolate due to leukopenia. She was gradually weaned off steroids, and her vasculitis to date remains biochemically and clinically stable. Case report - Discussion The presence of the combination of a petechial/purpuric rash on her lower limbs, worsening fatigue and Raynaud’s, and symptoms consistent with a progressive peripheral neuropathy raised the suspicion of a vasculitic process in this patient, which warranted urgent investigation. She had a type II mixed cryoglobulinemia which is the most common type of cryoglobulinemia found in Sjögren’s syndrome, evidenced by the presence of a monoclonal IgM paraprotein with polyclonal lambda light chains. Cryoglobulinaemic vasculitis is a systemic vasculitis characterised by the deposition of immune complexes into small vessels, commonly affecting the peripheral nerves, skin, and joints. Clinically, this can manifest with arthralgias/arthritis; constitutional symptoms, such as fatigue and fever; neurologically, with peripheral neuropathies, cranial nerve and central nervous system involvement; and with vascular symptoms, such as petechiae/purpura, skin ulcers, hyperviscosity syndrome, and Raynaud’s. Laboratory features consistent with a diagnosis of cryoglobulinaemic vasculitis aside from the sine qua non of positive cryoglobulins include hypocomplementemia (especially complement C4), positive rheumatoid factor, and a positive serum monoclonal component. We suspect that her cryoglobulinaemic vasculitis was most likely due to Sjögren’s syndrome, although it could have been triggered by the preceding Epstein-Barr virus infection, as this can be associated with cryoglobulinemia also. The decision to treat aggressively with pulsed intravenous cyclophosphamide and prednisolone was made given the severity of the patient’s symptoms, especially her progressive peripheral neuropathy. Given the paucity of data in the literature on the management of cryoglobulinaemic vasculitis secondary to rheumatological conditions, cyclophosphamide and prednisolone were chosen as these are proven in the other small vessel vasculitides, such as ANCA-associated vasculitis. This case is of interest as cryoglobulins are found in approximately 7–16% of patients with Sjögren’s syndrome, with cryoglobulinaemic vasculitis seen in only 3–4% of patients with the disease. Case report - Key learning points Cryoglobulins are uncommon in Sjögren’s syndrome, occurring in 7–16% of those with the disease. Symptomatic cryoglobulinaemic vasculitis among those with Sjögren’s syndrome is rare, seen in only 3–4% of cases. The presence of cryoglobulins in Sjögren’s syndrome is of clinical significance, as it is associated with higher global systemic disease activity and extra glandular involvement. Compared to non-cryoglobulinaemic patients with Sjögren’s syndrome, those with cryoglobulinemia are more likely to have lymphadenopathy, constitutional symptoms, peripheral nervous system and pulmonary involvement, and glandular, articular, and cutaneous features of the disease. The type of cryoglobulinemia found in Sjögren’s syndrome is the mixed type, which are either formed from a monoclonal immunoglobulin (usually IgM) and a polyclonal immunoglobulin (type II), or two polyclonal immunoglobulins (type III). Other conditions associated with mixed cryoglobulinemia include rheumatoid arthritis and systemic lupus erythematosus (SLE), although the most common cause of these is chronic hepatitis C (80–90% of cases). Other causes of mixed cryoglobulinemia include other viral infections, including Epstein-Barr virus and hepatitis B, and certain bacterial and parasitic infections. Most of the literature on the management of cryoglobulinaemic vasculitis is in the context of patients with this due to chronic hepatitis C and revolves around treating this with the appropriate antiviral therapy. Consequently, the current treatment options for moderate-to-severe cryoglobulinaemic vasculitis secondary to rheumatological conditions are the same as those for the other small vessel vasculitides, using a combination of cyclophosphamide and glucocorticoids to induce remission, and azathioprine as maintenance therapy. In severe cases, plasma exchange and rituximab can also be considered as agents to induce remission in cryoglobulinaemic vasculitis.

We report the case of a woman in her 30s who was referred to the ear, nose and throat department with sudden onset left-sided sensorineural hearing loss (SNHL), left anterior uveitis and erythematous lower limb lesions with bilateral pitting oedema. Based on her symptoms, an underlying inflammatory systemic disease was suspected. Autoantibodies were negative but an X-ray and high-resolution CT scan of the chest were suggestive of sarcoidosis, which was confirmed on endoscopic bronchial biopsy. Following treatment with a course of oral steroids, the patient’s hearing has improved but she still suffers from episodes of uveitis. While immune-mediated inner ear disorders are a recognised cause of SNHL, sarcoidosis is a very rare cause. This case demonstrates the importance of screening for systemic autoimmune aetiology in SNHL and highlights the importance of an effective multidisciplinary team in the diagnosis and management of these patients.

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We describe two cases of giant cell arteritis where involvement of the superficial temporal artery and maxillary artery were demonstrated using colour doppler ultrasonography. Maxillary artery involvement is responsible for the symptoms of jaw claudication and toothache, and even headaches might be due to the involvement of the middle meningeal artery which is a branch of the maxillary artery. The maxillary artery has been difficult to visualise until now. There are international consensus definitions of ultrasonographic abnormalities seen in the superficial temporal artery affected by giant cell arteritis. We have used those definitions to demonstrate hypoechoic changes in the maxillary artery affected by giant cell arteritis. The maxillary artery can be visualised in the infratemporal fossa from an echo window between the condylar and coronoid processes of the mandible. This is the first proof of concept evidence that maxillary arteries can be visualised using bedside ultrasonography in giant cell arteritis.