Hyperglycemia Associated With Raynaud Phenomenon and Liver Dysfunction After COVID-19 Vaccination in Type 1 Diabetes Mellitus.

Background/Objective: The association of COVID-19 vaccinations and the changes in glycemic control remains debatable. We report a case of a patient with type 1 diabetes mellitus (DM) with previously well-controlled glucose on a hybrid closed-loop insulin pump who developed significant glucose variation, new onset Raynaud phenomenon, and liver dysfunction after the vaccination. Case Report: A 33-year-old man with type 1 DM since the age of 5 years was on an insulin pump for 17 years. He had a reasonable controlled glucose level with a hemoglobin A1c level of 6.8% (51 mmol/mol). Three days after he received the COVID-19 vaccination, his glucose level started to fluctuate in the range of 46 to 378 mg/dL with 3.5 times higher total daily insulin requirement. The patient developed white-pale cold hands, weight gain, fatigue, and liver dysfunction. Computed tomography of the abdomen revealed mild hepatomegaly, and laboratory workup was negative for hepatitis. One month later, his glucose level became better controlled, and his liver function improved. Continuous glucose monitoring revealed that his glucose profile returned to baseline after 6 weeks. Discussion: COVID-19 vaccination resulted in significant glucose variation and fluctuations in this patient. It could be explained by the vaccine-induced immune response causing an increase in insulin resistance, such as in adipose tissue and muscle cells. Immune stimulation could have also caused the abnormal liver function and explain his new onset Raynaud phenomenon. Conclusion: We described, for the first time, the long-term continuous glucose monitoring glucose profile with a hybrid closed-loop system in type 1 DM after COVID-19 vaccination. Clinicians need to keep alert to glycemic excursion and side effects after immunization in type 1 DM.

Covid-19 Vaccine-Induced Myositis

Background/Aims Through the COVID pandemic there have emerged reports of autoimmunity or new rheumatic diseases presenting in patients after they had COVID-19. This is thought to be caused by cross-reactivity of the COVID-19 spike protein to human antigens. Given the use of mRNA COVID-19 vaccinations which express the spike protein we might expect to see presentation of new rheumatic diseases following their use. We discuss a case where this appears to have occurred. Methods Our patient is a 24-year-old male with mixed phenotype acute leukaemia who had been treated with allogenic stem cell transplant and was currently in remission. He presented with fevers, palpitations, myalgia and bilateral arm and leg swelling. Symptoms began the day after receiving the first dose of an mRNA COVID-19 vaccination (Pfizer/BioNTech.) There were no other symptoms or recent change in medications. Physical examination revealed tender oedema in his forearms, biceps and thighs bilaterally with sparring of the hands. He had reduced power with shoulder (MRC 3/5), elbow (4), wrist (4+) and hip (4) movements. Observations revealed tachycardia and fevers up to 40C. Results Laboratory studies showed markedly elevated C-reactive protein (202), creatinine kinase (6697) and troponin (593) whilst investigations for infection were negative. An autoimmune panel was positive for anti- PM-SCL-75-Ab. An electrocardiogram showed sinus tachycardia. Echocardiogram was normal. Bilateral upper limb dopplers revealed no deep vein thrombus. An MRI of his thighs showed diffuse symmetrical oedema within the muscles, in keeping with an inflammatory myositis. A quadricep muscle biopsy showed evidence of MHC class 1 up-regulation, suggesting an inflammatory process. In addition, there were numerous macrophages evident in the endomysium. While this can be seen in graft-versus-host disease (GVHD), they would usually be found in the perimysium. After discussion between haematology, rheumatology and neurology, this was felt to be a case of vaccine induced myositis and myocarditis. Autoimmune myositis was thought to be less likely due to the relative sparing of the hands and the absence of Raynaud's phenomenon. 1 gram of intravenous methylprednisolone was then given for 3 days. The patient had a marked response with defervescence, improving laboratory markers, improved myalgia and decreased limb swelling. The patient was stepped down to a reducing regime of prednisolone and discharged. Due to relapse whilst weaning he has started on mycophenalate mofetil and rituximab and now continues to improve. Conclusion There are case reports of myositis following COVID-19 vaccination but our patient's case is complicated by the differential diagnosis of GVHD and concurrent myocarditis. Ongoing work is needed to clarify the exact link between vaccination and the presentation of a new inflammatory myositis, but it is important to recognise and start treatment early in order to preserve muscle bulk and ensure recovery.

Chronic Chilblain Like Lesions Associated with the Covid-19 Pandemic

Background/Aims We report the features of chronic chilblain-like digital lesions newly presenting since the start of the covid-19 pandemic. Comparison with primary perniosis and acrocyanosis, reveals a unique phenotype which appears to be a long-covid phenomenon. Methods The case records of 26 patients with new onset persistent chilblain-like lesions presenting to the Rheumatology service of St George's University Hospital, London between Autumn 2020 and Spring 2022 were reviewed. Demographic and clinical features, serology, imaging, treatment response and outcome up to Summer 2022 were collated retrospectively. Results Chilblain-like lesions first occurred between September and March;2019/ 2020 6 cases, 2020/2021 18 cases and 2021/2022 2 cases. Mean age 35.4 (17-60) years, 88% female, 85% white, all non-smokers. Median body mass index (BMI) 20.2, range 17.0 - 33.2. BMI underweight (<18.5) in 27%. All cases reported new red-purple-blue colour changes of the fingers, some with pain, swelling and pruritis, affecting both hands in 12, one hand in 6, and both hands and feet in 8 cases. There was a past history of cold sensitivity or primary Raynaud's in 54%. Covid was confirmed in 3 cases, 2 - 8 months prior to onset of chilblain-like symptoms. Possible covid, unconfirmed, was suspected in 5 cases, 1 - 11 months earlier. Affected digits appeared diffusely erythro-cyanotic in 81%, with blotchy discrete maculo-papular erythematous lesions in 42%, some with both features. Involvement was asymmetric in 54%, thumbs spared in 69%. Complement was low in 50% (8/16), ANA positive in 26% (6/23). MRI of hands showed phalangeal bone marrow oedema in keeping with osteitis in 4 of 7 cases. More severe signs and symptoms were associated with low BMI, low C3/4 and a past history of cold sensitivity or Raynauds. Cold avoidance strategies were sufficient for 58%. Pain prompted a trial of NSAIDs, aspirin, nitrates, calcium channel blockers, hydroxychloroquine, oral or topical corticosteroid or topical tacrolimus in 42%. In general, these were minimally effective or not tolerated. 4 severe cases received sildenafil or tadalafil, effective in 2. In 27% complete remission occurred during the first summer season after symptoms commenced, median duration 6 (range 2 - 10) months. In the remaining 19 cases, chilblain-like symptoms returned or worsened in the subsequent second winter period, with 6 of 19 entering remission the following summer. For the remaining 13 persistent cases the total duration of symptoms spans more than a year, and in four cases more than 2 years. Conclusion This series illustrates a distinct chronic chilblain-like condition. Features similar to primary perniosis include female predominance, middle age, pruritic painful blotchy lesions, asymmetry and low BMI. Features in keeping with acrocyanosis include chronicity, extensive diffuse erythro-cyanotic discoloration, relative improvement in warm weather and lack of association with smoking.

Covid-19 Booster Vaccine Related Dermatitis: A Case Study

Case Report: A 28 year old male with a past medical history of hypothyroidism and positive ANA presented to an outpatient dermatology clinic with a diffuse pruritic rash two weeks after the administration of his first Moderna COVID booster vaccine. He denied any other accompanying symptoms such as fever or chills as well as any similar rashes to prior doses of the Moderna COVID vaccine. The rash consisted of pink erythematous minimally scaly papules, thin plaques and patches involving the left and right dorsal hands, forearms, wrists, face, neck and left shoulder. The remainder of the patient's skin including the bilateral lower extremities, the eyelids, conjunctiva and oral mucosa was clear. The patient denied any similar rashes in the past. The patient denied any allergies to medications, or food or environmental allergies. He denied any notable contact allergen exposures, including to soaps, lotions, and cosmetic products. The patient also denied any significant family history or past surgical history. The patient was on Armour Thyroid for hypothyroidism and testosterone for low levels since age eighteen. The patient was started on cetirizine 10 mg once daily for the rash with minimal improvement. Autoimmune workup for the rash was notable for an elevated anti-RNP and as the patient's past medical history included Raynaud's phenomenon and ANA positivity for ten years, the patient was diagnosed with mixed connective tissue disease (MCTD). Autoimmune conditions can often have an indolent course, where symptoms progressively develop and worsen. MCTD is an autoimmune overlap syndrome that can consist of the following three connective tissue diseases: systemic lupus erythematosus, scleroderma, and polymyositis. Millions of individuals across the world are receiving COVID vaccines to protect themselves and members of their community, and it is of utmost importance that we continue to investigate adverse events. Although of low incidence, these rare effects have the ability to impact large numbers of people within both healthy and immunocompromised populations. It is critical that we examine and document them in a rigorous manner, to ensure safe vaccine delivery and reassure the public about vaccine safety overall.

Temporal association between COVID-19 vaccination and Raynaud's phenomenon: A case series.

COVID-19 vaccine-related adverse events are mostly minor to moderate, and serious events are rare. Single cases of Raynaud's phenomenon (RP) in temporal proximity to COVID-19 vaccination have been reported. Demographic data, medical history, and detailed information regarding vaccination status and RP characteristics were obtained from patients with confirmed RP after COVID-19 vaccination. Fifteen participants reported the initial manifestation of RP, which occurred in 40% after the first, in 33% after the second, and in 27% after the third vaccination. RP development and occurrence of episodes were not linked to any specific vaccine type. New onset of disease was observed in 40% of the vaccinees after BNT162b2, in 33% after mRNA-1273, and in 27% after ChAdOx1 vaccination. Three out of four participants with preexisting RP prior to COVID-19 vaccination reported aggravation in frequency and intensity after immunization. Although COVID-19 vaccination is pivotal in controlling the pandemic, the observed temporal association between vaccine administration and RP occurrence warrants global activities to support pharmacovigilance for the detection of adverse reactions, one of which may include RP.

Possible immune reaction to COVID-19 vaccination - a case report

Introduction Side effects may occur after vaccination against COVID-19. Temporary reactions such as redness, swelling and pain at the injection site, high temperature, fever, tiredness, etc. may be signs of the body's response to the vaccine. Such reactions usually develop within two days after vaccination and last for a few days. With the growing number of vaccinations against SARSCoV- 2 a rising number of reports also showed serious side effects. In some of the most severe cases, life-threatening thrombotic events may develop. We present a case that shows further symptoms that may be due to an immune reaction to the vaccine. Method In this case report a 67 male smoker presented to our outpatient clinic in April 2022. A few days after vaccination against SARS-CoV-2 with an mRNA vaccine the patient developed pain at all finger tips. The clinical examination showed cool and livid discoloration of all fingers to different degrees;toes were not involved. The symptoms developed progressively over the following weeks into a severe form with progressive fingertip skin necrosis. Results The blood test showed a CRP of 9.18 mg/l (reference range: 0-3 mg/l) as well as an increased fibrinogen and factor VIII activity. D-dimers were only slightly increased to 290 ng/ml (reference range: < 230 ng/ml) during initial examination. Cold agglutinins, cryoglobulin and cryofibrinogen were tested negative. Angiologic examination revealed small multiple thrombi in the ulnar and digital arteries. Furthermore, the resting ECG showed no dilated ventricles and no indication of a hemodynamically relevant defect. The assessment revealed a good cardiac function overall with no evidence of embolism. Therapy was started with Nifidipine (gold standard in Raynaud's disease), Eliquis 5 mg 1-0-1, and diclofenac following hospital admission. In the further course, the therapy regimen was changed to Ilomedin IV for 4 days once a month. After two weeks, symptoms significantly improved and the signs of necrosis at the fingers disappeared. Conclusion In summary, a circulatory perfusion disorder associated with microthrombotic events may be a possible side effect of SARS CoV-2 vaccination. A combination of Nifidipine, DOAC and pain therapy has been shown to be an effective treatment of "COVID-fingers" in this case report.

Eosinophilic fasciitis triggered by intensive exercise

A 59-year-old white female who was previously fit and well, developed gradual tightening and thickening of the skin on her forearms progressing to the abdomen, chest and lower legs associated with restricted movement. She also noticed bruise-like patches on her trunk. There were no systemic symptoms and no history of Raynaud syndrome. Since the beginning of the COVID-19 lockdown, the patient had engaged in increasing amounts of exercise compared with normal;this included yoga once weekly for 75 min, high-intensity interval training for 20 min on alternate days, running three times weekly for 45 min, lifting 2.5 kg weights for the arms every day and regular long walks. Examination showed a 'groove' sign on her forearms and a peau d'orange appearance of the skin with a woody induration and hardness on palpation. Symmetrical and circumferential involvement on the forearms and lower legs and bruise-like indurated patches on the abdomen were noted. Differential diagnoses included eosinophilic fasciitis (EF), morphoea, EF/morphoea overlap, scleroderma, scleromyxoedema and nephrogenic systemic fibrosis. Blood investigations showed an eosinophilia of 1.2 x 109 cells L-1, erythrocyte sedimentation rate of 31 mm h-1, a C-reactive protein of 20 mg L-1 and negative autoimmune and viral serology. She underwent two incisional biopsies down to fascia. The first was taken from the back, which showed an interstitial inflammatory cell infiltrate composed of lymphocytes, plasma cells and very occasional eosinophils. The subcutaneous septa were minimally thickened. The second biopsy taken from the left forearm showed striking thickening of the subcutaneous septa, with an associated inflammatory cell infiltrate, composed predominantly of lymphocytes and plasma cells. This process was deeper and more established than that seen in the biopsy from the trunk. The appearances were clearly those of a sclerosing process of the dermis and subcutis and consistent with eosinophilic fasciitis. Our diagnosis was EF with morphoea overlap and she was treated with oral methotrexate 15 mg weekly and oral prednisolone 50 mg once daily (weight 60 kg), reducing the dose by 5 mg every 2 weeks. An 80% improvement was seen in functionality within 3 months, but the skin remained tight and thickened and therefore the patient was referred for phototherapy [ultraviolet A 1 (UVA1)] as combination therapy. We present a rare case of EF, which appears to have been triggered by intensive exercise. Other causes include insect bites, radiation, infections (Mycoplasma and Borrelia) and paraneoplastic. Haematological associations have been seen, including aplastic anaemia and lymphoma. Treatment options for EF include prednisolone, UVA1/psoralen + UVA, immunosuppressive systemic agents (including ciclosporin and methotrexate), biological agents (including infliximab and rituximab) and physiotherapy.

Worsening of Primary Raynaud's Phenomenon During Episodes of Pyrexia and Rigors in SARS-CoV-2 Infection.

Acute SARS-CoV-2 infection is associated with several cutaneous manifestations, including vasculitic digital ischemia. The reversal of digital ischemia in the primary Raynaud phenomenon (RP) arises from endothelial hypersensitivity to circulating adrenaline and noradrenaline and diminished vasodilatory innervation. SARS-CoV-2 can infect endothelial cells and be associated with raised adrenaline levels, reduced microvascular dilatory responses, and exaggerated clotting mechanisms. We report worsened RP in a patient with previously mild winter-time primary RP during the periods of fever and rigors from an acute SARS-CoV-2 infection contracted in a warm summer period. This reverted to the mild phenotype after recovery, and we discussed possible mechanisms for the brief exacerbation.

Multiple opportunistic infection in a male with systemic lupus erythematosus and concomitant human immunodeficiency virus infection

Background/Purpose: Concomitant systemic lupus erythematosus (SLE) and human immunodeficiency virus (HIV) infection cases are rare worldwide. It is established that SLE patients have an increased risk of opportunistic infection due to immune dysregulation, as well as in HIV. Method(s): A case of a 25-year- old Filipino man with systemic lupus erythematosus admitted due to a 1-week intermittent fever associated with headache, loss of appetite, and generalized body weakness was reviewed in a tertiary hospital in the Philippines. Result(s): An initial diagnosis was made from the clinical presentation of Raynaud's phenomenon, an elevated antinuclear antibody (1:320;nuclear, speckled), 2+ proteinuria, thrombocytopenia, and nail fold capillaroscopy findings consistent with mixed connective tissue disease. Patient was started on hydroxychloroquine and prednisone. He was admitted as a case of Streptococcus bacteremia with COVID-19 pneumonia after initial diagnosis, presenting as fever, and thrombocytopenia as low as 23.000/mul. Patient presented with a scaly erythematous annular lesion at his left wrist since December 2021 where a skin punch biopsy showed findings consistent with dermatophytosis. Direct immunofluorescence staining showed deposition of granular IgM (+3), C3 (+1), Fibrinogen (+3), and C1q (+1) in the basement membrane zone consistent with Lupus Erythematosus. Additional findings were oral thrush, dermatophytosis, and Pneumocystis pneumonia. Patient was started on antibiotics, remdesivir, and antifungal medications. Being severely immunocompromised, work up for HIV was initiated. Rapid HIV screening was positive, CD4 count revealed 7 (3.14%), and subsequent confirmatory western blot was positive. Additional treatment included hydroxychloroquine, methylprednisolone pulse therapy, and platelet concentrate transfusion. He was referred for CD4 monitoring, and ARV treatment enrollment, however, the patient expired a month after his discharge. Conclusion(s): This case is thereby reported to document a rare case of systemic lupus erythematosus (SLE) male patient with concomitant HIV, SARS-CoV- 2, and opportunistic infections secondary to AIDS. Diagnosis becomes challenging in patients with autoimmune diseases and multiple infectious diseases as clinical presentations tend to overlap and may show similar manifestations. In this setting, skin biopsy utilizing direct immunofluorescence can help establish an accurate diagnosis especially when clinical features and histopathology are overlapping.

A CASE OF ANTISYNTHETASE SYNDROME: THE IMPORTANCE OF HISTORY IN DISTINGUISHING ETIOLOGY OF INTERSTITIAL LUNG DISEASE IN A COVID-19 WORLD

SESSION TITLE: Systemic Diseases Causing Pulmonary Havoc SESSION TYPE: Rapid Fire Case Reports PRESENTED ON: 10/18/2022 10:15 am - 11:10 am INTRODUCTION: In the coronavirus disease 2019 (COVID-19) era, the etiology of interstitial lung disease (ILD) should remain broad to ensure accurate diagnosis and the proper treatment of patients. Vital to the art of medicine is taking a comprehensive history, and anchoring on a common diagnosis such as COVID-19 can result in early dismissal of alternate etiologies that physicians have an obligation to explore. CASE PRESENTATION: A 58-year-old male with a history of diabetes, hypothyroidism, and hypertension presented to the emergency department (ED) with dyspnea and fever. Initial CT chest imaging was significant for reticular and fibrotic changes with peripheral ground-glass and solid nodular opacities, some with areas of central clearing. Despite negative PCR testing, he was diagnosed with COVID-19 and discharged on oxygen with pulmonary follow-up. He continued to have arthralgias, proximal muscle weakness, low-grade fevers, and weight loss. He re-presented to the ED and was admitted for hypovolemia and further exploration into a potential autoimmune etiology of his symptoms. Labs were significant for a creatine kinase of 3,381 U/L, positive autoimmune antibodies [ANA (1:320), Jo-1 (>8.0 U), and SS-A/Ro (1.4 U)], and elevated ESR and CRP (30 mm/hr and 81 mg/L). Repeat CT revealed persistent parenchymal changes. Bronchoscopy was performed without anatomical abnormalities, and bronchoalveolar lavage (BAL) fluid was normal in appearance and negative for infectious etiologies. Though the patient was a farmer and possessed risk factors for hypersensitivity pneumonitis, lack of lymphocytic predominance on BAL, negative hypersensitivity panel, and uncharacteristic CT findings helped exclude this diagnosis. The patient was diagnosed with antisynthetase syndrome and treated with pulse dose intravenous solumedrol before transitioning to prednisone with resolution of muscle weakness and radiographic improvement in lung infiltrates. Muscle biopsy was deferred given the rapid clinical response and serum markers consistent with the diagnosis. DISCUSSION: Antisynthetase syndrome is a rare cause of ILD and often presents with myositis, arthritis, skin changes, Raynaud's phenomenon, and fever [1]. These symptoms, combined with the aminoacyl-tRNA synthetase antibody—most commonly the Jo-1 antibody—help confirm the diagnosis [2]. Due to a lack of established diagnostic criteria, muscle biopsy is often used to exclude other causes of myositis [3]. The ILD associated with antisynthetase syndrome is a significant cause of morbidity and mortality, and delay in diagnosis can lead to progression of lung injury. CONCLUSIONS: Chest imaging findings in COVID-19 are nonspecific, and post-COVID lung disease often presents similarly to other ILDs [1]. Because of this, history and physical exam remain crucial tools to reflect on alternate diagnoses for ILD and will continue to be necessary as we evolve through this COVID-19 era. Reference #1: Devi HG, Pasha MM, Padmaja MS, Halappa S. Antisynthetase Syndrome: A Rare Cause for ILD. Journal of Clinical and Diagnostic Research : JCDR. 2016;10(3):OD08. doi:10.7860/JCDR/2016/16872.7361 Reference #2: Cavagna L, Trallero-Araguás E, Meloni F, et al. Influence of Antisynthetase Antibodies Specificities on Antisynthetase Syndrome Clinical Spectrum Time Course. Journal of Clinical Medicine. 2019;8(11). doi:10.3390/jcm8112013 Reference #3: Schmidt J. Current Classification and Management of Inflammatory Myopathies. J Neuromuscul Dis. 2018;5(2):109-129. doi: 10.3233/JND-180308. PMID: 29865091;PMCID: PMC6004913. DISCLOSURES: No relevant relationships by Dustin Norton No relevant relationships by Alyssa Simon No relevant relationships by Kang Rui Xiang

ATYPICAL VARIANT OF GRANULOMATOSIS WITH POLYANGIITIS WITH EOSINOPHILIA

SESSION TITLE: Critical Systemic Disease Case Report Posters SESSION TYPE: Case Report Posters PRESENTED ON: 10/19/2022 12:45 pm - 01:45 pm INTRODUCTION: Granulomatosis with polyangiitis (GPA) is a necrotizing granulomatous vasculitis affecting small-to-medium sized blood vessels. GPA is highly associated with antineutrophil cytoplasmic antibodies (ANCAs) and often triggered by environmental factors such as medications and infectious agents. Tracheobronchial stenosis and diffuse alveolar hemorrhage are serious complications of GPA. CASE PRESENTATION: A 35-year-old Caucasian male with a history of chronic sinusitis requiring balloon sinuplasty and recent tympanostomy had presented multiple times to the emergency room due to dyspnea and cough with pinkish sputum production. This was associated with sore throat and fever, which were attributed to his COVID-19 infection and treated with supportive care. Due to persistent drainage through his tympanostomy he was prescribed levofloxacin by his ENT specialist. After the second dose of levofloxacin, he developed Raynaud's phenomenon, diffuse purpuric lesions and swelling over his lower extremity, eyelids, and elbows. Four days later he developed worsening hemoptysis and dyspnea for which he was admitted for further evaluation. Laboratory findings were remarkable for peripheral eosinophilia, elevated ESR 19mm/hr, CRP 9.2mg/dl, c-ANCA 1:320 titer, positive proteinase-3 antibodies and normal p-ANCA titers. Urinalysis with microscopic hematuria. Chest CT scan showed ground glass opacity, consolidative infiltrate with subpleural sparing and minimal left bronchial stenosis. Bronchoscopy suggestive of diffuse alveolar hemorrhage. Limited lung biopsy showed ulcer and granulation tissue with abundant eosinophils, but no granulomatous inflammation noted. Pulse dose steroids and Rituximab were initiated, and rapid clinical improvement was noted. Patient was discharged on prednisone taper and Pneumocystis jiroveci prophylaxis. DISCUSSION: We believe that GPA may have been triggered by recent COVID-19 infection and levofloxacin use. Mild peripheral blood and tissue eosinophilia (<12%) has been described in GPA, however it is a rare finding. GPA and eosinophilic granulomatosis with polyangiitis (EGPA) are both ANCA vasculitis that involve lungs and kidneys. GPA presents with sinusitis, alveolar hemorrhage and high titers of PR-3 antibodies. EGPA presents with a history of atopic, asthma and high titers of myeloperoxidase-ANCA along with abundant peripheral eosinophils. Our patient best fits the diagnostic criteria for GPA with eosinophilia variant rather than EGPA. Our patient had no history of asthma or atopic disease and p-ANCA was normal, which also points away from EGPA. CONCLUSIONS: Clinicians should recognize the differential diagnosis for eosinophils in ANCA vasculitis. Early diagnosis of ANCA vasculitis and initiation of appropriate treatment is important to decrease morbidity and mortality. Reference #1: Potter MB, Fincher RK, Finger DR. Eosinophilia in Wegener's Granulomatosis. Chest 116: 1480-1483, 1999 Reference #2: Krupsky, Meir et al. Wegener's Granulomatosis With Peripheral Eosinophilia. CHEST, Volume 104, Issue 4, 1290 - 1292 Reference #3: Kitching AR, Anders HJ, et al. ANCA-associated vasculitis. Nat Rev Dis Primers. 2020 Aug 27;6(1):71. doi: 10.1038/s41572-020-0204-y. PMID: 32855422. DISCLOSURES: No relevant relationships by Afoma King No relevant relationships by Joshuam Ruiz Vega No relevant relationships by Krishna Shah no disclosure on file for Milos Tucakovic;

UNUSUAL CASE OF ANTI-SYNTHETASE SYNDROME CORRELATED WITH EXPOSURE TO COVID-19 INFECTION OR VACCINE

SESSION TITLE: Systemic Disease with Diffuse Lung Symptoms Case Posters SESSION TYPE: Case Report Posters PRESENTED ON: 10/17/2022 12:15 pm - 01:15 pm INTRODUCTION: Since its first detection at Wuhan, China, SARS-CoV-2 (novel coronavirus 2019) has engulfed the world with more than 100 million cases and manifestations of COVID19 have been evolving over time. Various post COVID19 syndromes are being recognized. Reactive arthritis, connective tissue disorders such as myositis and pulmonary complications have been correlated with exposure to COVID infection. We describe the case of antisynthetase syndrome in a patient correlated with exposure to COVID infection or vaccine. CASE PRESENTATION: A 68 year old female with history of hypertension and exposure to COVID infection in the family member, presented with 2-3 months worsening generalized body ache/pain started 2 weeks after receiving second dose of mRNA vaccine. Patient also reported dyspnea and leg swelling for 1 month. Upon presentation, she was placed on 4 liter oxygen via nasal cannula. Chest x-ray concerning for infiltrates, possibly COVID. CT chest no pulmonary embolism but evidence of pneumonia superimposed on chronic appearing bronchiectasis. Flu and Covid testing were negative. Patient was started on IV antibiotics for community acquired pneumonia. Labs showed elevated ESR, CRP and CK level. No fever, weakness, mechanics hands, rash or Raynaud's phenomenon. Infectious work up remained negative. No lymphadenopathy on CT chest to suggest sarcoid. ACE level normal. ANA and anti aminoacyl-tRNA synthetase antibody positive but other ENA were negative. HMG-COA ab negative. MPO/PR3 neg. Echocardiogram was unremarkable. Work up was suggestive of Anti synthetase syndrome with interstitial lung disease(ILD), a form of dermatomyositis. Patient was started on intravenous steroid with good improvement in symptoms and later transitioned to oral prednisone. Patient was discharged on minimal home oxygen with plan to start immunosuppressive medications. DISCUSSION: We are unsure if our patient had COVID19 infection since COVID testings were negative (antigen, antibody and nucleic acid detection ). The likelihood of autoimmune and rheumatic diseases in COVID19 survivors is a big issue. COVID19 infection may unmask previously undiagnosed rheumatic conditions and precipitate de novo disease, both of which may persist after resolution of the initial infection. Corticosteroids remain the cornerstone of early treatment with initial doses at 1mg/kg of the ideal body weight. In an effort to reduce steroid related side effects, other immunosuppressive agents should be considered at the outset of therapy, particularly when treating anti-synthetase syndrome with manifestations of ILD. CONCLUSIONS: Patients with anti-synthetase syndrome with ILD could have correlation with exposure to COVID infection or vaccination, and are steroid responsive. It is likely that clinical improvement may result from prompt suppression of inflammatory systemic response by corticosteroid. Reference #1: 1. Ahmed S, Zimba O, Gasparyan AY. COVID-19 and the clinical course of rheumatic manifestations. Clin Rheumatol. 2021;40(7):2611-2619. doi:10.1007/s10067-021-05691-x Reference #2: 2. Witt LJ, Curran JJ, Strek ME. The Diagnosis and Treatment of Antisynthetase Syndrome. Clin Pulm Med. 2016;23(5):218-226. doi:10.1097/CPM.0000000000000171 DISCLOSURES: No relevant relationships by ELINA MOMIN No relevant relationships by Mohammedumer Nagori

Myositis and Myocarditis With Anti- Jo-1 Antibodies Following SARS-CoV-2 mRNA Vaccination Or COVID-19 Infection

Background: Myocarditis without myositis has been described following mRNA SARS-CoV-2 vaccination. The literature on post-vaccine antibody mediated myositis is limited and to date no case series have been reported with a distinct clinical syndrome and a single myositis specific antibody, related to SARS-CoV-2 mRNA vaccination or COVID-19. Over a 6-month period in 2021, 54 patients were referred to our tertiary referral centre for suspected myositis. Out of 25 patients with a diagnosis of myositis, we identified three patients with a distinct clinical syndrome with myositis and myocarditis with anti-Jo-1 antibodies, following SARS-CoV-2 mRNA vaccination (BNT162-Pfizer-BioNtech;n=2) or following a mild COVID-19 infection (n=1). Results: Three patients (one woman, two men;49, 50 and 58 years old) developed progressive muscle weakness and muscle pain following either vaccination (patient 1 and 2) or mild COVID-19 infection (patient 3). Patients 2 and 3 had a history of anti- CCP positive rheumatoid arthritis (RA), which had been untreated for three years in patient 2. Both post-vaccine cases had severe pitting edema of the legs, patient 2 also had arthritis. None of the patients had mechanic's hands, Raynaud's phenomenon, or interstitial lung disease (ILD). The time interval between the SARS-CoV-2 trigger and the onset of progressive muscle weakness was between 10 and 14 days (patient 1 and 3) and was estimated cybetween three and seven days in patient 2. Laboratory tests showed highly elevated CK levels (17-32 times upper limit of normal (ULN)) and troponine T levels (14-34 times ULN). In patient 2, in addition, troponin I was tested (42 times ULN), which is more specific for myocardial involvement. In patient 1 supraventricular tachycardia, unspecific ST- and Twave abnormalities and elevated NTproBNP were found. In all patients, testing for myositis specific antibodies (MSAs;EUROline myositis 16 Ag. lineblot assay) showed anti-Jo-1 antibodies (semi-quantitatively in the highest possible range). Muscle MRI showed widespread muscle edema in all patients and extensive fascial and subcutaneous edema in the legs in the post-vaccine cases (figure 1). Muscle biopsies showed inflammatory myopathy. Cardiac MRI showed abnormalities in all patients: Pericardial effusion and/or late contrast enhancement of the epicardial myocardium (figure 1). All patients showed major improvement in response to immunosuppressive therapy and could discontinue highdosed steroids after three and six months. Discussion: In conclusion, we report three patients with a distinct clinical picture of anti-Jo-1 myositis and myocarditis without ILD, following SARS-CoV-2 mRNA vaccination or COVID-19. Although it is difficult to determine a causal relationship between SARS-CoV-2 and anti-Jo-1 myositis based on these small numbers, we suspect a SARSCoV- 2 trigger of anti-synthetase syndromes given the typical combination of symptoms and previously demonstrated association with antecedent viral infections. In addition, we have collected nationwide data on myositis specific antibodies (MSAs) in 2019 (pre- COVID-19) and 2021 (during COVID-19) from six medical centers in the Netherlands. We are currently analysing these data to examine whether the proportion of positive MSAs in 2021 is higher as compared to 2019. The results will be presented at the ICNMD.

PERFORMANCE of AN EARLY TRIAGE SYSTEM for IDENTIFICATION of PATIENTS with INFLAMMATORY RHEUMATIC DISEASES

Background: The preselection of patients with suspicion of an infammatory rheumatic disease is not easy for general practitioners and orthopedists. In countries with a limited number of practicing rheumatologists waiting lists are often long, since a full rheumatologic examination often needs a long consultation time. Objectives: To test the performance of an early triage strategy for early identif-cation of patients with infammatory rheumatic diseases. Methods: Prior to the SARS-CoV 2 pandemic, physicians caring for patients contacting a tertiary rheumatologic cente were frst contacted by a health-care professional (HPR) who offered an appointment the timing of which was based on the symptoms reported (Step 1). Patients were then seen by a rheumatolo-gist who, within a 10-minute consultation (Step 2), shortly examined the patient to determine the urgency of a planned full work up. The main outcome of the study was the comparison between the initial assessment and the fnal expert diagnosis (Step 3). Results: Within 9 months, physicians caring for 1.180 patients contacted the hospital, 972 of whom kept their appointment (82.4%). Most patients were transferred by GPs (73.1%) and orthopedists (22.1%). The mean time between Step 1 and Step 2 was 10.4 days, while 6.2% of patients were seen within 4 days, 24.4% within 7 days and 69.3% within 12 weeks. Only 36 patients (3.7%) of patients had an already established rheumatic disease. Complaints lasting between 0-4 weeks were reported by 69 (7.1%), of > 4-12 weeks by 100 (10.3%), and of > 12 weeks by 973 (82.6%) patients. Almost 90% of patients reported a pain intensity >4/10 (NRS) for < 2 weeks. An elevated CRP was found in 207 patients (24.5%). Prior treatment with glucocorticoids was reported in 163 (16.8%) and with NSAIDs in 730 (75.1% of) patients. The confirmed diagnosis at Step 3 was rheumatoid arthritis in 127 (13.1%), spondyloarthritis including pso-riatic arthritis in 72 (7.4%), systemic diseases including connective tissue diseases in 112 (11.5%), vasculitides in 41 (4.2%), and crystal arthropathy in 38 (3.9%) patients, while 38 (3.9%) had an infection, a malignancy or a differential diagnosis such as Raynaud's phenomenon or sicca syndrome. Degenerative joint diseases (n=254;26.1%) and non-inflammatory soft tissue syndromes such as fibromyalgia (n=369;38%) accounted for more than half of the patients. Conclusion: This study describes the performance of a standardized triage system hereby confrming the need for an early identifcation and preselection of patients with rheumatic musculoskeletal symptoms, including involvement of HPRs in the initial phase of contact. Based on the results, three patients with musculoskeletal complaints had to be examined in order to identify one patient with an infammatory rheumatic disease.

IMPACT of ILOPROST WITHDRAWAL in SCLERODERMA PATIENTS DUE to COVID-19 PANDEMIC

Background: Intravenous iloprost is currently recommended in the treatment of Raynaud's phenomenon (RP) refractory to oral therapy and of digital ulcers (DUs) related to systemic sclerosis (SSc). In real-life practice there is a huge heterogeneity about the Iloprost regimens used, because of a lack of specifc recommendations. Coronavirus pandemic resulted in an unexpected emergency leading to sudden and synchronous withdrawal of programmed iloprost infusions for most SSc patients in March 2020, in order to limit virus dissemination. At the same time, lockdown forced people to stay at home, thus reducing the exposure to coldness. Both these unavoidable circumstances were close to an experimental condition, clearly non-replicable in routine conditions. Objectives: The aim of the survey was to evaluate the consequences related to a sudden and simultaneous iloprost discontinuation in a cohort of SSc patients. Methods: A telephone survey was carried out on SSc patients that interrupted Iloprost infusion at our centre. They were specifcally asked to compare acral vascular symptoms just before Iloprost withdrawal, in February and just after the missed infusion in March (0-10 scale). Demographic and disease characteristics, severity and frequency of R P, new DUs onset or aggravation of those pre-existing were reported for each patient. Last available capillaroscopic images were also evaluated to assess the pattern. Results: The analysis included 50 patients. After iloprost withdrawal, 11 patients reported a RP worsening because of enhanced intensity (4.6±2.1 vs 5.2±2.3, p=0.007). Only 8 patients of them also complained of an increased frequency (4.5±2.3 vs 5.0±2.4, p=0.07). None of the patients experienced digital ulcers for the frst time during quarantine. Among the 27 patients with a history of digital ulcers, 9 reported worsening and 7 recurrences of DUs. Overall, 17 patients (34.0%) complained of a worsening of SSc vascular acral manifestations, namely RP or DUs. Disease and general features did not statistically correlate with worsening of RP except for reduced capillary density. Of note, each unit increase of capillary density corresponds to an average 44% decrease in the odds of RP worsening (OR 0.56, CI 95% 0.36-0.97, p=0.037). History of DUs-either active or former at the time of ilo-prost discontinuation-was the only clinical predictor of worsening of DU severity. As for RP worsening, the worsening of DU was associated with a lower capillary density. Conclusion: Low capillary density can predict a worsening of both RP and DUs within a month after iloprost discontinuation in SSc patients. Further studies are needed to assess whether the capillaroscopy should be used to personalize ilo-prost regimen in SSc patients.

CORRELATES of CANCELLED HEALTHCARE APPOINTMENTS in PATIENTS with SYSTEMIC SCLEROSIS during the COVID-19 PANDEMIC

Background: Rheumatologists have noted challenges in treating patients with systemic sclerosis (SSc) during the COVID-19 pandemic, such as patients on immunosuppressants and those with preexisting pulmonary issues.1 Also of potential concern was patients delaying treatment due to fears of COVID. Objectives: This study examined to what extent patients with SSc cancelled health-care appointments due to fears of COVID, how this correlated with their mental and physical functioning, and their levels of anxiety and depression. Methods: Participants were 60 people with SSc who had not had COVID-19 or been vaccinated for COVID. Participants had a mean age of 58 years (SD=11.5) and were primarily female (92%) and white (87%). Participants completed an online survey after learning about it from a sclero-derma organization newsletter or at their visit with a rheumatologist specializing in scleroderma in New York State, USA. Data were collected August 2020 through March 2021. Participants completed measures assessing health-related appointments attendance, Patient Reported Outcome Measurement Information System (PROMIS) measures (anxiety, depression, fatigue, sleep disturbance, pain intensity, physical function), coping strategies to deal with the pandemic, and reported functioning relevant to SSc (hand, gastrointestinal, dyspnea, and Raynaud's disease). Results: Due to COVID-19 concerns, 28 respondents (47%) cancelled one or more appointments with their primary care physician, specialist, physical or occupational therapist, pulmonologist, or dentist. Compared to those who did not cancel appointments due to COVID concerns, participants who cancelled reported higher anxiety, t(58) = 2.46, p =.02, higher sleep disturbance, t(56) = 3.31, p =.002, worse physical functioning, t(56) = 2.00, p =.05, more constipation, t(55) = 2.05, p =.045, and lower positive reframing as a coping strategy, t(56) = 2.08, p =.04. Many participants reported moderate or severe anxiety (36%), depression (22%), fatigue (32%), sleep disturbance (28%), and physical dysfunction (40%). As shown in Table 1, anxiety scores were positively correlated with scores on measures of fatigue, sleep disturbance, physical dysfunction, diarrhea, and the coping strategies of substance use, self-distraction, emotional support, behavioral disengagement, and venting. Depression scores were positively correlated with scores on measures of fatigue, sleep disturbance, physical dysfunction, pain intensity, dyspnea, diarrhea, and behavioral disengagement as a coping strategy. Conclusion: Almost half of participants cancelled one or more health appointments due to COVID fears. This subgroup reported significantly higher anxiety, sleep disturbance, constipation, worse physical functioning, and less coping with the pandemic by looking for the positive. Anxiety and/or depression also played a role in 42% of SSc patients and were related to a variety of worse mental and physical health correlates. Future research should examine the extent to which changes in health-related care during the pandemic have implications for disease progression in patients with SSc.

PRIMARY SJÖGREN'S SYNDROME with JUVENILE ONSET: REPORT of 15 CASES

Background: Primary Sjögren's syndrome (pSS) is a chronic and progressive multisystem autoimmune disease which rarely onset in children and adolescents. Diagnostic delay in large part of patients are common due to the non-specifc and variable symptoms and the slow progression of disease. Objectives: To analyse demographic data, specifc extraglandular, salivary and ocular manifestations, laboratory parameters and therapy of pSS with juvenile onset. Methods: Retrospective study of all patients (pts) with pSS in single center. Results: pSS was verifed in 15 pts (6.7% were boys), which amounted to 23.8% of all pts with SS in our pediatric rheumatologic department. The median age of pSS onset was 8.0 y.o. [IQR 7.0;10.2]. The median of disease duration at the time of pSS verifcation was 2.75 years [2.2;5.6]. All patients had systemic manifestations at onset: constitutional abnormalities-33.3%, nonerosive polyarthritis-64.3%, polyarthralgias-26.7%, lymphadenopathy-73.3%, cutaneous involvement-53.3% (2-xerosis, 2-annular erythema, 1-erythema nodo-sum, 2-Raynaud phenomenon, 2-nonspecifc spotty rashes, 1-hemorrhagic rash). At the time of diagnosis 7 pts (46.7%) had isolated involvement of salivary glands, 8 pts (53.3%)-combined with involvement of lacrimal glands. The decrease in salivary gland function was recorded in 80% of cases, hypolacrimia-in 46.7%, 1 patient had isolated hypolacrimia. Recurrent parotitis was present in 6 pts (40.0%). At time of diagnosis pulmonary involvement had 20.0% of pts, 1 patient had renal tubular acidosis. 8 pts (53.3%) had various hematological disorders: anemia-in 3 pts (20.0%), leukopenia-in 6 (40.0%). ANA Hep-2 were detected in 100% pts (in titer 1/640-4, 1/1280-7, 1/2560-3, 1/20480-1, with mixed patterns in all pts: speckled + homogeneous-9 pts, speckled + homogeneous+cytoplasmic-6 pts), anti-Ro-in 12 pts (80.0%), anti-La-in 8 pts (53.3%), RF+-in 9 pts (60.0%). 6 pts (40.0%) had polyclonal hypergammaglob-ulinemia, max 42%. 2 pts (13.3%) had concomitant autoimmune non-rheumatic disease;1-cutaneous psoriasis, 1-autoimmune thyroiditis. The treatment of each patient was justifed by the main individual manifestations: 93.3% received glucocorticoids, 26.7%-methotrexate, 33.3%-hydroxychloroquine, 6.7%-mycophenolate mofetil. Treatment with biologics (B) was received by 13 (93.3%) pts (7-rituximab (RTM), 6-abatacept (ABA)) with a good response in 10 pts, including improvement in the function of the salivary and lacrimal glands in 7 pts. 1 patient received 2B-RTM and ABA sequentially due to the development of MAS 7 days after 1st RTM infusion. B was discontinued in 3 pts: 1 due to development of hemorrhagic vasculitis 2 days after the 1st RTM infusion, 1-COVID-19 with lung involvement (CT 3-4) 2 weeks after the 1st RTM infusion, 1-inefficiency of ABA during 15 months. Conclusion: In our pediatric rheumatologic department pts with pSS made up less than a quarter of all pts with SS. The diagnosis was verifed delayed in all pts, which can be explained by a wide range of nonspecifc manifestations at the onset. However, the manifestations of SS that were present at the time of diagnosis were brought under control on the background of complex therapy, including the prescription of B, with a good efficacy and safety profile of therapy.

USEFULNESS of AN ELECTRONIC CONSULTATION SYSTEM between PRIMARY CARE HEALTH CENTERS and RHEUMATOLOGY DEPARTMENT of A TERTIARY HOSPITAL

Background: The EPISER study is the frst Spanish epidemiological study that has confrmed the great burden of rheumatic diseases in the general population: they consume a large quantity of health resources (doctor visits, medical products) and imply a high social impact in terms of work absenteeism. Rheumatic diseases represent almost 30% of Primary Care medical consultations in Spain1,2. Electronic consultation could be an alternative response to the increase of this demand, both to make an early diagnosis and derivation and to improve communication with Primary Care physicians3,4. Objectives: To analyze the demand of Primary Care and its resolution through the electronic consultation system of the Rheumatology Department of a tertiary hospital. Methods: Retrospective descriptive study of the data collected in the request and information system (Sistema de Peticiones Electrónicas, SIPE) that supports electronic consultation between primary care physicians of the health area and the Rheumatology Department of a tertiary hospital, between July 2020 and May 2021.The following variables were collected: age, sex, reason for consultation, response time in days and destination (primary care/outpatient follow-up). Descriptive statistics were used to present the results. Results: The last 500 consecutive electronic consultations registered in the system, referring to 496 patients, were collected. Mean age was 59.5±17.7 years;74.2% women. Mean response time was 2 days, median response time 1 day and range 0-45. The reasons for consultation (see Graph 1) were: osteoporosis assessment 55 (11%), treatment adjustment 50 (10%), appointment request 49 (9.8%), loss to follow-up 43 (8.6%), local-regional pathology assessment 39 (7.8%), infltration request 28 (5, 6%), suspected rheumatoid arthritis 19 (3.8%), fare 18 (3.6%), suspected polymyalgia rheumatica or giant cell arteri-tis 16 (3.2%), COVID vaccine consultation 14 (2.8%), Raynaud's phenomenon 13 (2.6%), monoarthritis assessment 12 (2.4%), assessment of polyarthritis 11 (2.2%), adverse effects of treatment 11 (2.2%), suspected spondyloarthritis 11 (2.2%), suspected psoriatic arthritis 8 (1, 6%), generalized pain 7 (1.4%), suspected Sjögren's syndrome 5 (1%), suspected systemic lupus erythematosus 1 (0.2%), suspected other systemic autoimmune diseases 9 (1.8%), others 81 (16.2%). Fifty-seven and four % (287) of the patients required an appointment at the Rheumatology outpatient clinic and in 42.6% of the patients (213) the electronic consultation was successful, so it was not necessary to refer the patient to the hospital. Conclusion: Forty-two and six percent of the queries were resolved thanks to the electronic consultation system in an average of two days, otherwise that patients would have been referred to specialized care. The main reasons for consultation were osteoporosis assessment and clarifcation of doubts about the treatment of patients who were already being followed up by the Rheumatology Department.

NOT EVERYTHING IS COVID

CASE: 45-year-old African American female with history of hypertension, hypothyroidism and prior tobacco abuse was admitted to hospital with shortness of breath and hypoxia. She was diagnosed with COVID-19 pneumonia due to her respiratory symptoms, CT scan findings of bilateral pulmonary infiltrate and positive COVID IgG antibodies although a PCR test was negative. The patient was discharged and later seen in pulmonary clinic where on further questioning she complained of fatigue, bilateral wrist and knee pain, and exertional dyspnea. On auscultation, bilateral rales were noted in the lower lung fields. She was noted to desaturate upon ambulation. PFTs (pulmonary function tests) revealed severe restrictive spirometry and severe gas transfer defect. A HRCT revealed bilateral infiltrates suggestive of organizing pneumonia. CPK was elevated at 449. Serologies were positive for ANA and antijo1 and negative for other connective tissue diseases. The patient was diagnosed with anti-synthetase syndrome (ASS). She was treated with oxygen, steroids and tacrolimus with reported improvement in her symptoms. IMPACT/DISCUSSION: ASS is a rare chronic systemic autoimmune disorder that predominantly affects females with a median age of 50. It is characterized by autoantibodies against aminoacyl-tRNA synthetase enzyme. The role of these autoantibodies in the development of ASS is not fully understood. Several autoantibodies have been identified including anti-Jo1, anti-EJ, antiOJ, anti-PL7, anti-PL12, anti-SC, anti-KS, anti-JS, anti-HA, anti-YRS. Among them, anti-Jo1 is the most common. The ASS is characterized by myositis, interstitial lung disease( ILD), arthritis, fever, Raynaud's phenomenon, mechanic's hand plus positive serologic testing of the Anti- aminoacyl-tRNA synthetase enzyme. The majority of the patients with Anti- Jo-1 antibodies develop ILD. An organizing pneumonia pattern can be seen in the settings of connective tissue disease and is commonly found in those with the ASS. ILD may be the first manifestation of the disease. CONCLUSION: We present a case of a 45 year old female mistakenly diagnosed with COVID pneumonia who on further evaluation was found to have ILD secondary to Antisynthetase Syndrome, a form of inflammatory myositis. An organizing pneumonia pattern on HRCT can be found in many settings other than COVID pneumonia. Careful attention to the history, physical examination, lab findings and COVID test results remain important in identifying etiologies other than COVID 19 for a patient's respiratory symptoms during the pandemic. Delays in diagnosis can be quite harmful to patients.

British Society for Rheumatology Annual Conference 2022

The proceedings contain 341 papers. The topics discussed include: safety of vaccination against sars-cov-2 in people with rheumatic and musculoskeletal diseases: results from the eular coronavirus vaccine (COVAX) physician-reported registry;a cohort of 79 children with pediatric multisystem inflammatory syndrome associated with covid-19;impact of the covid-19 pandemic on rheumatology training: results of the regional survey from the north west of England;COVID-19 admissions and mortality in patients with early inflammatory arthritis: results from a national cohort;lower incidence of COVID-19 but higher mortality in patients with inflammatory arthritis compared to controls in Wales, united kingdom: a population epidemiological study;single-center experience of the use of rituximab for inflammatory arthritis during the COVID-19 pandemic;a phase 2 randomized placebo-controlled trial investigating the effect of the angiotensin ii type 2 receptor agonist C21 on cold-induced vasoconstriction in patients with Raynaud's phenomenon secondary to systemic sclerosis;and development of an automated deep learning-based system for distinguishing between 'systemic sclerosis' and 'normal' capillaries.