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Anti-melanoma differentiation-associated protein 5 (anti-MDA5) is a subset of dermatomyositis associated with respiratory complications, in which rapidly progressive interstitial lung disease (RPILD) is commonly cited, and spontaneous pneumomediastinum (SPM) is a rare complication. In medical literature, aggressive immunosuppressive therapy has been the mainstay of anti-MDA5-associated SPM management. Here, we report the first MDA5 case with SPM which was successfully treated with a double-lung transplant. We present a 48-year-old male who presented with multiple constitutional symptoms such as fevers, weight loss, malaise, and arthralgias, in association with erythroderma over the ears and fingers. Imaging of the chest demonstrated peripheral airspace disease, and myositis-specific serology returned positive for anti-Jo1 (medium-positive), anti-Ro52 (high-positive), and anti-MDA5 (weak-positive) autoantibodies. Therefore, the patient was begun on immunosuppressive therapy as the leading diagnosis included autoimmune myositis, possibly antisynthetase syndrome with interstitial lung disease (ILD). A year later, the patient presented with progressive shortness of breath, widespread macular erythematous facial rash, and new erythematous ulcerations over the fingertips. Imaging demonstrated a new SPM at this juncture. As the patient’s respiratory status continued to decline despite the use of immunosuppressive agents, a double-lung transplant was performed. Therefore, we propose that lung transplantation should be considered early in MDA5-SPM.

Background:Although MRI of the sacroiliac joints (SIJ) is the most sensitive imaging modality for early diagnosis of axial spondyloarthritis (axSpA) it is costly and not readily available. Therefore, clinicians still rely primarily on radiography. The relative degree to which radiography and MRI changes diagnostic ascertainment of axSpA in patients presenting with undiagnosed back pain has not been formally studied.Objectives:We aimed to assess the relative impact of radiography and MRI evaluation on diagnostic ascertainment of axial SpA in patients presenting with undiagnosed back pain to rheumatologists, and the impact of central evaluation.Methods:The multicenter Screening for Axial Spondyloarthritis in Psoriasis, Iritis, and Colitis (SASPIC) Study is aimed at early detection of axial SpA. Consecutive patients ≤45 years of age with ≥3 months undiagnosed back pain with any one of psoriasis, acute anterior uveitis (AAU), or colitis undergo routine clinical evaluation by a rheumatologist for axial SpA and MRI evaluation is ordered per rheumatologist decision. The rheumatologist determines the presence or absence of axial SpA at 3 consecutive stages: 1. After the clinical evaluation; 2. After the results of labs (B27, CRP) and radiography; 3. After the results of MRI evaluation. The eCRF, radiographs, and MRI scans were also assessed centrally.Results:The SASPIC cohort comprises 246 patients (52.4% male, mean age 34.5 years, mean symptom duration 7.3 years, mean back pain duration 7.1 years, B27+ 36.2%) referred with AAU (29.7%), psoriasis (18.7%), Crohn’s colitis (31.3%), ulcerative colitis (20.3%). MRI was conducted in 149 patients. The number of patients diagnosed with axSpA by the local rheumatologist decreased after radiography and then decreased further after MRI while confidence in the diagnosis progressively increased (Table 1). After central evaluation of all patient data, the number of patients diagnosed with axSpA decreased substantially compared to assessment by local readers (Table 2).Table 1Stage of global assessmentData sourceaxSpA YESaxSpA YES with confidence > 7NOT axSpAaxSpA NO with confidence <-41. N = 246Clinical169 (68.7%)50 (20.3%)77 (31.3%)40 (16.3%)2. N = 246Clinical plus radiography141 (57.3%)66 (26.8%)105 (42.7%)78 (31.7%)Patients who had MRI1. N = 149Clinical107 (71.8%)23 (15.4%)42 (28.2%)19 (12.8%)2. N = 149Clinical plus radiography94 (63.1%)30 (20.1%)55 (36.9%)38 (25.5%)3. N = 149Clinical plus radiography plus MRI70 (47.0%)44 (29.5%)79 (53.0%)70 (47.0%)Table 2Stage of global assessmentData sourceaxSpA YES, number (%)axSpA NO, number (%)2. N = 246Clinical plus radiography141 (57.3%)105 (42.7%)2. N = 246Clinical plus radiography after central reader assessment79 (32.1%)167 (67.9%)3. N = 149Clinical plus radiography plus MRI70 (47.0%)79 (53.0%)3. N = 149Clinical plus radiography plus MRI after central reader assessment45 (30.2%)104 (69.8%)Conclusion:In a setting of undiagnosed back pain and higher risk for axial SpA, imaging is primarily helpful in ruling out SpA and reduc...

Background:Classification criteria for axial spondyloarthritis (axSpA) that capture the spectrum of disease present challenges due to the frequency of back pain, the relative infrequency of axSpA, and limited physical and laboratory findings in early disease. Several cohorts have reported the performance of the ASAS classification criteria in settings where patients have been selected for certain features such as the presence of inflammatory back pain and/or short symptom duration.Objectives:We aimed to test the performance of the ASAS classification criteria in unselected patients referred with undiagnosed back pain who have presented with acute anterior uveitis (AAU), psoriasis, or colitis to their respective specialists and whether performance varied according to demographic factors and symptom severity.Methods: he multicenter Screening for Axial Spondyloarthritis in Psoriasis, Iritis, and Colitis (SASPIC) Study is aimed at early detection of axial SpA. Consecutive patients ≤45 years of age with ≥3 months undiagnosed back pain with any one of psoriasis, acute anterior uveitis (AAU), or colitis undergo routine clinical evaluation by a rheumatologist for axial SpA and MRI evaluation is ordered per rheumatologist decision. The rheumatologist determines the presence or absence of axial SpA and the degree of confidence in the diagnosis (-10 (definitely not SpA) to +10 (definite SpA)) at 3 consecutive stages: 1. After the clinical evaluation; 2. After the results of labs (B27, CRP) and radiography; 3. After the results of MRI evaluation. Assessment of imaging was conducted by local and central readers. We calculated the sensitivity and specificity of the ASAS criteria and the component imaging and clinical arms using the stage 3 diagnostic assessment by the local rheumatologist as gold standard.Results:A total of 246 patients were recruited, 47.6% being diagnosed with axSpA (61.5% male, age 33.7 years, symptom duration 7.6 years, B27 positive 52.1%), after stage 3 evaluation. Sensitivity/specificity of the ASAS criteria, imaging arm, clinical arm were 74.4/79.8%, 55.6/93.8%, 50.4/82.2%, respectively (Table). For patients diagnosed with a high degree of confidence sensitivity/specificity was 87.5/82.7%, 68.8/94.5%, 56.3/84.5%, respectively. Specificity, especially for the clinical arm, was notably higher in patients with a higher degree of back pain (≥5/10), and in those with longer symptom duration (≥5 years).Abstract THU0393 –Table 1 Patient Category Number ASAS criteria Imaging arm Clinical arm Sen Spec Sen Spec Sens Spec All patients 24674.479.855.693.850.482.2 Patients diagnosed with confidence >7 for axSpA yes and <-4 for not axSpA (-10 to +10 scale) 19087.582.768.894.556.384.5 Patients with back pain ≥5 (0-10 scale) 16574.783.754.494.251.984.9 Patients with back pain <5 (0-10 scale) 8173.772.157.993.047.476.7 Patients with symptom duration ≥5 years 14378.185.753.495.756.285.7 Patients with symptom duration <5 years 10368.272.959.191.540.978.0 Males 12973.678.961.189.548.684.2 Females 11775.680.646.79...

Background:Patients presenting with back pain and psoriasis, iritis, or colitis, represent a high-risk population for the presence of axial spondyloarthritis (axSpA). The Dublin Evaluation Tool (DUET)1, the Berlin algorithm2, and the ASAS modification of this algorithm3are recommended referral strategies aimed at early diagnosis of axSpA. DUET was developed for patients presenting with AAU. Validation of these algorithms in inception cohorts is limited.Objectives:1. To assess the performance of referral algorithms for diagnosis of axSpA when tested against the final local rheumatologist diagnosis in an inception cohort of patients presenting with undiagnosed back pain and extra-articular manifestations. 2. To determine whether different criteria for inflammatory back pain (IBP) impact the performance of the algorithms.Methods:The multicenter Screening for Axial Spondyloarthritis in Psoriasis, Iritis, and Colitis (SASPIC) Study at 11 sites is aimed at early detection of axial SpA in patients presenting with undiagnosed back pain to the rheumatologist. Consecutive patients ≤45 years of age with ≥3 months undiagnosed back pain with any one of psoriasis, acute anterior uveitis (AAU), or colitis diagnosed by the relevant specialist undergo routine clinical evaluation by a rheumatologist for axial SpA. The rheumatologist determines the presence or absence of axial SpA at 3 consecutive stages: 1. After the clinical evaluation; 2. After the results of labs (B27, CRP) and radiography; 3. After the results of MRI evaluation. Final diagnosis by the rheumatologist was used as external standard to test the performance of the algorithms. We tested the following criteria for IBP in the algorithm: ASAS, Berlin, rheumatologist global for likelihood of IBP >5 (0-10 scale), and DUET algorithm in AAU patients.Results:A total of 246 patients were recruited, 73 presented with iritis, 46 with psoriasis, and 127 with colitis, 47.6% were diagnosed with axSpA. The diagnosis of axSpA was established in 45.7%, 61.6%, and 40.2% of patients with psoriasis, AAU, and IBD, respectively. The performance of the ASAS-modification of the Berlin algorithm was superior to the original algorithm as reported previously3, primarily for enhanced sensitivity, and this was observed irrespective of the criteria used to define IBP (Table 1). Conversely, the performance of the Duet algorithm in the subset of patients with AAU was substantially worse than previously reported1.Conclusion:The ASAS modification of the Berlin algorithm is the preferred referral strategy for patients presenting with undiagnosed back pain to the rheumatologist.References:[1]Haroon M, et al. Ann Rheum Dis 2015; 74: 1990-5[2]Poddubnyy D, et al. J Rheumatol 2011; 38: 2452–60[3]Van den Berg R, et al. Ann Rheum Dis 2013;72:1646–53AlgorithmSensitivity (%)Specificity (%)Correct diagnosis (%)False negative (%)False positive (%)Original Berlin(ASAS criteria for IBP)65.376.671.116.712.2Original Berlin(Berlin criteria for IBP)64.476.670.717.112.2Original Berlin(IBP global >5)67.878.173.215.411.4ASAS Modification of Berlin algorithm (ASAS criteria for IBP)73.775.874.812.612.6ASAS Modification of Berlin algorithm (Berlin criteria for IBP)73.775.074.412.613.0ASAS Modification of Berlin algorithm(IBP global >5)76.377.376.811.411.8DUET84.450.071.29.619.2Disclosure of Interests:Ulrich Weber: None declared, Georg Kröber: None declared, Raj Carmona: None declared, James Yeung: None declared, Jon Chan: None declared, Sibel Aydin: None declared, Liam Martin: None declared, Ariel Masetto: None declared, Stephanie Keeling: None declared, Olga Ziouzina: None declared, Sherry Rohekar: None declared, Rana Dadashova: None declared, Amanda Carapellucci: None declared, Joel Paschke: None declared, Robert G Lambert: None declared, Walter P. Maksymowych Grant/research support from: AbbVie, Novartis, Pfizer, and UCB, Consultant of: AbbVie, Boehringer Ingelheim, Celgene, Eli Lilly, Galapagos, Janssen, Novartis, Pfizer, and UCB, Employee of: Chief Medical Officer of CARE Arthritis Limited, Speakers bureau: AbbVie, Janssen, Novartis, Pfizer, and UCB