Course Of Oral Prednisolone Research Articles

Surgical management of unilateral restrictive strabismus in an 8-month-old dog

ABSTRACT Case history An 8-month-old male, entire, mixed-breed dog was presented with a 1-month history of left exophthalmos and green mucopurulent ocular discharge. Subsequently, exophthalmos resolved but esotropia (medial strabismus) developed in the left eye, prompting referral to an ophthalmologist. Clinical findings At the initial referral consultation, enophthalmos and esotropia of the left eye were identified. The patient showed mild improvement after a 3-week tapering course of oral prednisolone and doxycycline. MRI was performed and showed left medial rectus muscle atrophy with increased contrast enhancement which was consistent with chronic extraocular muscle myositis (EOM). A forced duction test was performed to confirm the diagnosis of fibrosing esotropia, which is likely a sequela of chronic EOM. Diagnosis Fibrosing esotropia presumably caused by untreated EOM. Treatment and outcome One month later, esotropia progressed to a marked ventro-medial strabismus resulting in visual deprivation. Surgical release of the ventral oblique, medial and ventral recti muscles was performed, resulting in immediate resolution of the enophthalmos. Despite a tapering post-operative course of oral prednisolone, mild esotropia was present 4 weeks later. In an effort to stabilise the globe position, the low dose of prednisolone was increased to a higher anti-inflammatory dose before slowly tapering over 2 months. The vision in the left eye was improved after surgery and has been maintained since without further treatment. Clinical relevance This is the first documented case of fibrosing esotropia in a young dog with prior signs of acute exophthalmos. Fibrosing esotropia has been documented in certain breeds or as a sequela to chronic EOM. In this patient, it was presumably caused by EOM, which was strongly supported by the case history, progression and MRI findings. Most historical reports of EOM described it as a bilateral condition that resolves with systemic corticosteroids at an anti-inflammatory dose. EOM has been shown to also present unilaterally and it can progress to strabismus if not promptly recognised and treated with systemic steroids. Surgical management can restore vision when severe strabismus results in visual deprivation.

Checkpoint inhibitor immunotherapy induced inflammatory arthritis secondary to Nivolumab and Ipilimumab: a pediatric first

BackgroundImmune checkpoint inhibitors (ICIs) have expanded the arsenal of cancer therapeutics over the last decade but are associated with a spectrum of immune-related adverse events (irAEs), including inflammatory arthritis. While these complications are increasingly recognized in the adult population, no cases of inflammatory arthritis irAEs have been reported in the pediatric literature.Case PresentationA 14-year-old female with metastatic epithelioid mesothelioma was referred to the pediatric rheumatology clinic after developing progressive inflammatory joint pain in her bilateral shoulders, hips, and small joints of hands following the second cycle of Nivolumab and Ipilimumab. Initial examinations showed bilateral shoulder joint line tenderness, positive FABERs test bilaterally, tenderness over bilateral greater trochanters, and bilateral second PIP effusions. Her serological profile was notable for positive HLA-B27, positive anti-CCP, negative Rheumatoid Factor, and negative ANA. PET-CT scan performed for disease response following immunotherapy showed symmetric increased metabolic activity primarily involving the supraspinatus, gluteus medius and minimus, and semimembranosus tendon insertions. Her presentation was consistent with a grade 1 irAE that worsened to a grade 2 irAE despite NSAID therapy, prompting a short course of oral prednisolone. She achieved clinical remission of her mesothelioma following six cycles of Nivolumab and Ipilimumab and her inflammatory arthritis was controlled on Celebrex monotherapy.ConclusionsTo our knowledge, this is the first pediatric case of ICI-induced inflammatory arthritis and enthesitis. This case highlights the importance of increasing awareness of diagnosis and management of irAEs in children.

Acute posterior multifocal placoid pigment epitheliopathy following inactivated SARS-CoV-2 vaccination: A case report

A 25-year-old woman presented with redness and pain in both eyes after the first dose of the inactivated vaccine Corona Vac (Sinovac Biotech Ltd., Beijing, China). She had non granulomatous anterior segment inflammation and multiple creamy placoid lesions within the posterior pole and midperipheral retina bilaterally. Fluorescein angiography (FA) revealed early hypofluorescent and late hyperfluorescent lesions, while indocyanine green angiography (ICGA) demonstrated hypofluorescent lesions throughout all phases. A diagnosis of acute posterior multifocal placoid pigment epitheliopathy (APMPPE) was made, and the patient was started on a course of oral prednisolone that was tapered and the inflammation was completely resolved.

P10 Collapse, confusion and acute kidney injury presenting in the context of bullous skin disease in a young male patient

P10 Collapse, confusion and acute kidney injury presenting in the context of bullous skin disease in a young male patient

P04 A case of zoledronate-induced ANCA associated vasculitis

Abstract Introduction Bisphosphonates are a well-established treatment for osteoporosis and other metabolic bone disorders. In up to 40% of recipients, infusion is associated with a transient cytokine-mediated acute-phase response. It has been hypothesised that this can rarely cause autoimmune reactions, with case reports linking bisphosphonate infusion to the development of giant cell arteritis (GCA), urticarial vasculitis and dermatomyositis. In this report, we describe a case of ANCA associated vasculitis (AAV) with 3rd nerve palsy, finger joint synovitis, fevers and cough, preceded by zoledronate infusion. To our knowledge, this is the first description of a zoledronate-induced AAV. Case description A 75-year-old lady with a background of ulcerative colitis and gout was referred to rheumatology for the management of osteoporosis after sustaining a left neck of femur fracture. A decision was made to commence zoledronate treatment. Within a few hours of receiving zoledronate, the patient began to feel unwell with fevers, vomiting, headache, and general malaise. Within 24 hours of the infusion she developed pain, stiffness and swelling to the small joints of her hands with fatigue, mild intermittent cough without breathlessness, and ongoing headaches. The initial impression was that she had developed a side effect to zoledronate which would be self limiting and she was treated with over the counter analgesics. Two weeks later she remained symptomatic and bloods showed a newly raised CRP of 29 and ESR of 18. Rheumatoid factor, ANA and CCP were negative. Her joint symptoms settled with a short course of oral prednisolone, which was prescribed for a possible diagnosis of zoledronate-induced inflammatory osteoarthritis. By this point, her chest symptoms had completely resolved. Four weeks after the zoledronate infusion, the patient developed a sudden onset 3rd nerve palsy, headache, eye pain and tingling to her face whilst in Prague. CRP was 1 and PR3 was strongly positive (131.5). MRI brain did not show any inflammation. She was treated with intravenous methylprednisolone for 6 days and her symptoms improved. On return to the UK, she was seen by rheumatology, ophthalmology, and neurology teams. Examination showed a resolving 3rd nerve palsy, without features of retinal vasculitis or any systemic symptoms, likely due to an AAV. She has continued on oral prednisolone and her symptoms have resolved. Her bloods show a down trending PR3 (131.5 to 23.7). Discussion Although zoledronate is generally well-tolerated, up to 40% of patients develop transient flu-like symptoms after infusion. Commonly reported symptoms include fever, chills, muscle and joint pains and headache. These symptoms are thought to be due to a transient acute-phase response mediated by pro-inflammatory cytokines. Symptoms generally reach their peak during the first two days post-infusion, with a rapid decrease in frequency after 3 days. In our case, the patient described many features in keeping with the well-described acute-phase response. Atypically, her symptoms were more persistent than usual with joint pain and swelling lasting for 2 weeks and an associated rise in inflammatory markers. Following an initial improvement in symptoms with oral prednisolone, our patient developed sudden-onset 3rd nerve palsy. Investigations were in keeping with AAV and the patient responded well to corticosteroid therapy. Although the literature is sparse, case reports and series have suggested a rare link between zoledronate and the development of autoimmune disease in patients with pre-existing risk factors . In these patients, it has been hypothesised that the acute phase response can trigger autoimmunity, however further work is needed to understand the underlying pathophysiology. Existing literature suggests that zoledronate can trigger a range of autoimmune pathology. Narkovits et al described six patients who developed a new-onset or an exacerbation of known autoimmune disease within 3 months of zoledronate infusion. These included new-onset rheumatoid arthritis, polymyalgia rheumatica and autoimmune haemophilia with other patients reporting flares of pre-existing Crohn’s disease. Some case reports have described the development of vasculitis secondary to zoledronate. Swarnkar et al, 2021, reported a case of zoledronate induced biopsy-confirmed urticarial vasculitis and there have been two case reports of GCA possibly triggered by zoledronate (Naderi, 2019 and Metyas et at, 2014). To our knowledge, there have been no other reports of zoledronate induced AAV. Key learning points Zoledronate infusion is associated with a proinflammatory acute phase response in up to 40% of recipients. The most common symptoms include fevers, joint pain and swelling, gastrointestinal symptoms including abdominal pain, diarrhoea, nausea and vomiting and general features including fatigue, dizziness, headache and malaise. Very rarely, zoledronate is associated with eye symptoms including pain and scleritis. There have been case reports linking zoledronate infusion to the development of autoimmune and autoinflammatory disease, including vasculitis. Although further work is needed to fully understand this association, clinicians should be aware of these rare reactions in order for rapid diagnosis and prompt management. In this case, zoledronate-associated AAV responded effectively to conventional therapies with corticosteroids.

BI11 Cutaneous presentation of Kaposi sarcoma following allogeneic haematopoietic stem cell transplantation for myeloma

Abstract Iatrogenic Kaposi sarcoma (KS) is a relatively frequent occurrence following solid-organ transplants (0–5%), in part due to prolonged immunosuppression. However, its occurrence after haematopoietic stem cell transplantation (HSCT) is rare, with only 15 cases described in the literature. We report a case of KS presenting in the skin of a patient who had received a matched unrelated donor allogeneic HSCT for IgG kappa myeloma. A 55-year-old HIV-negative white man with myeloma underwent an allogeneic HSCT from a matched, unrelated donor following conditioning chemotherapy with fludarabine–melphalan and antithymocyte globulin. Post-transplant, he was maintained on ciclosporin as graft-versus-host disease (GVHD) prophylaxis. On day 14 post-transplant, he developed a widespread eczematous eruption that rapidly evolved into an erythroderma; a skin biopsy was consistent with acute GVHD. He was managed with superpotent topical steroids and a 5-day course of oral prednisolone. He began extracorporeal photopheresis (ECP) on day 33 post-transplant, having failed to respond adequately to systemic steroids. He also completed four cycles of rituximab. Ciclosporin was stopped by day 130 post-transplant. He had completed 14 cycles of ECP by day 139 post-transplant, with resolution of cutaneous GVHD; however, he had developed a rapidly growing solitary dusky-red nodule on his right neck that was excised due to diagnostic uncertainty. Histology revealed a dermal haemorrhagic exophytic vascular tumour composed of lobules and sheets of epithelioid cells with an eosinophilic cytoplasm and pleomorphic nuclei. Immunoperoxidase staining was negative for S100, MNF-116 and AE1/AE3. The same cells were strongly and diffusely positive for the vascular markers CD31, CD34 and ERG. The lesional cells were positive for human herpesvirus (HHV)-8 confirming a diagnosis of Kaposi sarcoma. He subsequently developed a second lesion on his left forearm, despite being off all immunosuppression. A repeat bone biopsy confirmed that he remains in morphological remission from his myeloma. Positron emission tomography–computed tomography has not identified extracutaneous disease. KS following HSCT is rare, but it can be fatal. Skin lesions are the dominant clinical presentation. HHV-8 has an important role in the pathogenesis, and transplant-related KS may occur secondary to reactivation of the virus as a result of significant immunosuppression following HSCT. However, it could also occur as primary infection or be transmitted with donor cells. The outcome of KS after HSCT is variable. A watch and wait strategy can be applied if immune recovery is expected. In our patient, it developed 5 months after HSCT; shortly after immunosuppression had been tapered, he remains well and will be closely monitored for further suspicious lesions.

Investigation of the Relationship between Aspirin-Sensitivity and Poor Response to Medical Management in NSAIDs-exacerbated Respiratory Disease Patients with Sinonasal Polyposis.

NSAID-exacerbated respiratory disease (N-ERD) is a highly heterogeneous disorder with various clinical symptoms. The aspirin challenge test is a gold standard method for its diagnosis, and there are still no reliable in vitro diagnostic biomarkers yet. Oral challenge tests are time-consuming and may be associated with a risk of severe systemic reactions. This study aimed to evaluate whether patients with poor responses to medical management are more susceptible to being aspirin-sensitive. In this cohort study, after CT scanning of all patients and subject selection, conventional medical treatment was started as follows and continued for three consecutive months: at first, saline nose wash twice per day, intranasal beclomethasone spray one puff in each nostril twice per day, montelukast 10 mg tablet once daily, a ten-day course of oral prednisolone starting with the dose of 25 mg per day and taper and discontinued thereafter. Sinonasal outcome test 22 (SNOT22) was used for the evaluation of symptom severity. Statistical analyses were performed with SPSS version 23, and data were analyzed using an independent samples T-test, paired T-test, and Receiver operating curve analysis. 25 males and 53 females were enrolled in this study, with an average age of 41.56 ± 11.74 years old (18-36). Aspirin challenge test results were positive in 29 (37.2%) patients. The average SNOT22 scores before the treatment were 52.97 ± 17.73 and 47.04 ± 18.30 in aspirin-sensitive and aspirin-tolerant patients, respectively, and decreased to 27.41 ± 16.61 and 24.88 ± 16.72 in aspirin-sensitive and aspirin-tolerant patients after the treatment, respectively. There was no significant difference in SNOT22 scores between the groups. The severity of symptoms before treatment and clinical improvement after treatment are not good predictors of N-ERD.

MO257: Glucocorticoid Dosing in Induction Remission Treatments for ANCA-Associated Vasculitis

Abstract BACKGROUND Anti-neutrophil cytoplasm antibodies (ANCA) associated vasculitis (AAV) is a rare and complex autoimmune disease. Induction remission and maintenance treatment typically includes glucocorticoids (GC) in addition to other immunosuppressive medications. Recent trials such as PEXIVAS (1) and ADVOCATE (2) have suggested lower dose GC as induction treatment with no significant detrimental effect on outcomes such as end-stage renal disease (ESRD) or mortality. METHOD A cohort study of biopsy-proven pauci-immune glomerulonephritis was constructed from a single centre between 2007 and 2021. Retrospective analysis of patients with AAV from a single centre in the North West, UK, compared ESRD and mortality between groups that received high dose versus low dose GC treatment at induction. High dose GC treatment was defined as 1.5 g intravenous (IV) methylprednisolone over 3 days followed by a tapering course of oral prednisolone starting with 60 mg per day. Low dose GC treatment was defined as a single dose of 250 mg pulsed IV methylprednisolone followed by a tapering course of oral prednisolone starting with 30 mg per day. Regression analysis was applied to outcomes including relapse and remission rates, glucocorticoid toxicity index (GTI) and significant adverse events. RESULTS Fifty-three patients with biopsy-proven ANCA vasculitis were identified. A total of 28 patients received high dose GC induction treatment and 25 patients received a lower dose GC induction regime. The median age for the high dose patients was 68 years (IQR 65–74), which was lower than the low dose patients; 70 years (IQR 58–78). There was a slight (60%) female preponderance in the low dose group compared with a slight male predominance in the high dose group (67%). The Charlson Comorbidity Index (CCI) was completed for all patients. The higher dose cohort had a mean CCI score of 4.8 whilst the lower dose cohort had a lower CCI of 3.8. The number of patients requiring acute dialysis was similar across both the high dose and low dose groups (29% and 32%, respectively). ESRD was also similar between the two groups at 6 months (P = .39). Time to remission was shorter in the lower dose GC cohort (P = .01) and risk of relapse was also reduced (P = .04), as shown in Fig. 1. Those that received the lower dose GC regime were on less prednisolone at 6 months than those starting with a higher induction dose and this was statistically significant when adjusting for confounders including age, gender and co-morbidities (P = .01). The risk of death was higher in the high dose GC group (21.4% versus 4%, respectively), although this did not reach statistical significance (P = .09). In addition, the risk of adverse events and GC toxicity was higher in the high dose cohort; P = .55 and P = .12, respectively. CONCLUSION Treatment of ANCA vasculitis remains complex and glucocorticoids remain the cornerstone of induction remission treatment regimes. Whilst our initial data did not show a significant difference in mortality and ESRD outcomes between those that received high versus low dose GC treatment, it demonstrated that low dose GC may be sufficient in the management of AAV. Our small size study suggests a lower dose GC induction remission treatment regime to be safe, effective and associated with fewer adverse effects relating to GC toxicity. Further large studies are needed to look at the narrow therapeutic window of GC and the role of GC-sparing treatments so as to improve outcomes in these patients.

MO202: Pulmonary-Renal Syndrome in Patient with Chronic Hepatitis C And HIV Disease

Abstract BACKGROUND AND AIMS Pulmonary-renal syndrome (PRS) is characterized by rapidly progressive glomerulonephritis and lung vasculitis. Most common causes of PRS are Goodpasture's disease, antineutrophil cytoplasm antibody (ANCA) associated vasculitis and systemic lupus erythematosus; rarely it could be caused by cryoglobulinemic vasculitis. ANCA and cryoglobulins are present in a wide range of infectious, inflammatory and malignant conditions with or without evidence of clinical vasculitis. ANCA positivity has been described in patients with human immunodeficiency virus (HIV) or chronic hepatitis C (HCV), but only a negligible proportion of these patients developed true ANCA vasculitis. Cryoglobulinemia is seen in ∼50% of patients with HCV and ∼5% of these patients have symptomatic disease. According to previous studies, rapidly progressive glomerulonephritis and pulmonary involvement are rare manifestations of cryoglobulinemia. We describe a patient with HIV and HCV infection who presented with rapidly progressive glomerulonephritis and lung vasculitis. METHOD Case report presentation. RESULTS A 43-year-old male presented with fatigue, nausea and decreased urinary output of 3–4 days. Vital signs were stable and physical examination showed abdominal distention and peripheral oedema. His past medical history included heroin abuse. However, he denied any illicit drug use, and he had been on methadone maintenance treatment for 8 years. The patient was diagnosed with HIV 11 years previously. His antiretroviral therapy included abacavir, dolutegravir and lamivudine. CD4 count before admission was 180–200 cells/mm3. In addition to HIV infection, the patient had HCV diagnosed 20 years ago, but he has not received any treatment. The laboratory tests revealed pancytopenia (leukocytes 1.3 × 109/L, haemoglobin 66 g/L, platelets 66 × 109/L), kidney injury (proteinuria 0.8 g/24 h and haematuria) with renal insufficiency (creatinine 727 mcmol/L, urea 36.6 mmol/L). Ultrasound workup demonstrated diffuse changes in liver parenchyma with signs of portal hypertension, and normal-sized, non-obstructed kidneys with Grade 0 parenchymal echogenicity. Chest CT revealed wide areas of ground-glass opacity with patchy airspace consolidations, more severe in the left upper lobe. A serologic workup was positive for cryoglobulins, ANA, c-ANCA (high titre of anti-PR3 143 IU/mL), with decreased levels of C3 and C4. CRP level was slightly elevated (40 mg/L). Moreover patient had positive treponemal and non-treponemal syphilis serological tests. An ultrasound-guided renal biopsy showed crescentic glomerulonephritis with predominant IgM and focal C3, C1q deposition in glomerular capillary walls without any significant glomerular proliferative features. Changes could be interpreted as an immune complex and/or cryoglobulin-mediated glomerulonephritis, but overlapping pauci-immune glomerulonephritis could not be ruled out. Haemodialysis was initiated due to kidney failure and hyperhydration. The patient was treated with pulse methylprednisolone followed by a tapering course of oral prednisolone. Rituximab 1000 mg was prescribed after excluding HIV opportunistic infections, and treatment of syphilis with three doses of benzathine penicillin was used. Due to low GFR, HIV treatment was switched to Dolutegravir/Rilpivirine. The patient refused to initiate HCV therapy. The patient responded well to treatment and lung vasculitis and kidney function improved, therefore haemodialysis was discontinued. Serum creatinine level decreased to 130–120 mcmol/L and remained stable after 18 weeks since the treatment. CONCLUSION PRS in a patient with chronic infections, positive cryoglobulins and positive c-ANCA clinically seems more applicable to c-ANCA vasculitis than to cryoglobulinemic vasculitis. Still overlapping of both diseases could not be excluded.

P158 A case of refractory paediatric Cogan’s syndrome treated with tocilizumab

Background/AimsCogan’s syndrome is a rare vasculitis, characterised by progressive sensorineural bilateral hearing loss, vestibular symptoms and non-syphilitic interstitial keratitis. We present a paediatric case which was refractory to initial treatment and subsequently successfully treated with Tocilizumab.MethodsCase report.ResultsA previously well 14-year-old boy presented with sudden onset hearing loss, tinnitus and vertigo. There was no history of new medication, trauma or tick bite. There was no family history of autoimmune disease or genetic hearing losses. On examination his cranial nerves except for vestibulocochlear were grossly intact. He had a steady gait with no cerebellar signs. Otoscopy was unremarkable. Audiology showed a moderate to severe sensorineural hearing loss bilaterally, worse on the right. He had normal type A tympanograms. An autoimmune screen was carried out which showed normal FBC, inflammatory markers, ACE and Chitotriosidase. ANCA, Rheumatoid factor and lyme serology were negative. MRI of the internal auditory meati was normal. He subsequently developed visual disturbance and was diagnosed with bilateral interstitial keratitis. A unifying diagnosis of Cogan’s syndrome was made. An MRI scan of his head, neck and upper thorax looking for evidence of large vessel vasculitis was normal. Genetics looking for evidence of a monogenic autoinflammatory disorder and primary immunodeficiency were negative. He was initially treated with a weaning course of oral prednisolone with improvement in symptoms. Unfortunately, 4 months later had clinical and audiological deterioration in hearing. He therefore received pulsed IV methylprednisolone and commenced subcutaneous methotrexate and adalimumab. 3 months later, there was both clinical and audiological improvement and he started to wean prednisolone. 7 months later, he presented with a 24-hour history of reduced hearing on the right, confirmed on audiogram. He received a further pulse of IV methylprednisolone and a short course of high-dose oral prednisolone, followed by a slowly weaning course. 3 months later, again he felt his hearing had deteriorated and this was confirmed on audiogram, with a 40-decibel loss in his previously good ear. He received two doses of IV methylprednisolone and background steroids were increased to 20mg daily. Due to frequent relapses, adalimumab was changed to IV tocilizumab at 10mg/kg 2-weekly, alongside methotrexate. IV tocilizumab was changed to the subcutaneous route during the COVID-19 pandemic and was tolerated well. His hearing subsequently improved and tocilizumab interval was extended to 3-weekly in Feb 2021. At last review he was stable and successfully transitioned to adult services.ConclusionEvidence regarding treatment options in paediatric patients is lacking due to the rarity of the condition and consequent difficulty in arranging high-quality trials. This is the first case report of use of tocilizumab for Cogan’s syndrome in children, highlighting it as a well-tolerated and successful treatment modality.Disclosure C. Anderson: None. K. McAllister: None. J. Walsh: None.

A localised vasculitic-like skin rash following the second dose of COVID-19 vaccine

Since December 2020, a number of different types of COVID-19 vaccines have been developed to limit the spread of the pandemic [Jedlowski and Jedlowski, 2021Jedlowski P. Jedlowski M. Morbilliform rash after administration of Pfizer-BioNTech COVID-19 mRNA vaccine.Dermatol Online J. 2021; 27 (https://doi.org/)https://doi.org/10.5070/D3271052044Crossref PubMed Google Scholar]. Although there have been reported side effects and adverse reactions to COVID-19 vaccines, they are still considered safe as the benefits of halting the spread of the virus and reducing the severity of COVID-19 infection outweigh the risks. Skin rash and reactions have been reported as possible side effects associated with different types of COVID-19 vaccines. Almost 20% of vaccinated participants in Saudi Arabia developed a skin rash after receiving the AstraZeneca vaccine [Al Bahrani et al., 2021Al Bahrani S. Albarrak A. Alghamdi O. Alghamdi M. Hakami F. Al Abadi A. et al.Safety and Reactogenicity of the ChAdOx1 (AZD1222) COVID-19 Vaccine in Saudi Arabia.Int J Infect Dis. 2021; 110: 359-362Abstract Full Text Full Text PDF PubMed Scopus (21) Google Scholar]. Herein we present a 45-year-old female patient who is usually fit and healthy with no previous history of drug or vaccine allergy and who presented with a skin rash that developed three days following the second dose of AstraZeneca vaccine. It was described by the patient as non-itchy red spots which involve both legs. The patient did not report any other symptoms, such as fever, sore throat, cough, flu-like symptoms, redness at the injection site, pruritis, joint pain, abdominal pain, or change in the colour of the urine. The patient had the first dose of the vaccine two months ago, and apart from temporary shivering, she felt fine afterwards and did not develop any skin reactions. Vital signs on admission (respiratory rate 15, oxygen saturation 98% on room air, temperature 36.2, blood pressure 118/59 and pulse 87). Physical exam revealed a red, raised, palpable and non-blanchable skin rash (most likely purpuric rash) localised mainly on the extensor surface of both legs (figure 1). The rash does not extend above the knee level, and no rash was seen elsewhere on the body. The rest of the physical exam was normal. A urine dipstick did not show any evidence of proteinuria or haematuria. There were no signs of lung cavitation or haemorrhage on chest X-ray. Blood workup was unremarkable, including full blood count, urea and electrolytes, liver function test, C-reactive protein, coagulation profile, and vasculitis screen. The patient was discharged home 24 hours after the hospital admission on a short course of oral prednisolone. Unlike our case who developed a purpuric rash, a petechial rash has been reported one day following the first dose of Sinovac COVID vaccine [Cebeci and Kartal, 2021Cebeci F. Kartal İ. Petechial skin rash associated with CoronaVac vaccination: first cutaneous side effect report before phase 3 results.Eur J Hosp Pharm. 2021; (ejhpharm-2021-002794)Crossref PubMed Scopus (14) Google Scholar]. A generalised purpuric skin rash in association with thrombocytopenia has been reported in a 60-year-old male patient a few days after receiving the first dose of the Moderna vaccine [Malayala et al., 2021Malayala S. Mohan G. Vasireddy D. Atluri P. Purpuric Rash and Thrombocytopenia After the mRNA-1273 (Moderna) COVID-19 Vaccine.Cureus. 2021; https://doi.org/10.7759/cureus.14099Crossref Google Scholar]. In our case the purpuric skin rash was localised to the lower limbs, and the platelet count was normal. Interestingly, two patients developed systemic vasculitis affecting the kidneys after the first dose of the Moderna vaccine [Anderegg et al., 2021Anderegg M. Liu M. Saganas C. Montani M. Vogt B. Huynh-Do U. et al.De novo vasculitis after mRNA-1273 (Moderna) vaccination.Kidney Int. 2021; (https://doi.org/)https://doi.org/10.1016/j.kint.2021.05.016Abstract Full Text Full Text PDF Scopus (40) Google Scholar]. In our case, we believe that the patient developed a post-vaccination localised cutaneous vasculitic-like reaction. In conclusion, the main learning points from our case are, firstly, a localised cutaneous vasculitic-like reaction on the non-injected limbs without associated systemic manifestations might be considered a possible side effect of the AstraZeneca vaccine. Secondly, this skin reaction can develop after the second dose even with no history of previously reported dermatological reactions following the administration of the first dose. The authors declare that they have no conflict of interest

Macrophage activation syndrome triggered by systemic lupus erythematosus flare: successful treatment with a combination of dexamethasone sodium phosphate, intravenous immunoglobulin, and cyclosporine: a case report

BackgroundMacrophage activation syndrome is classified as a secondary form of hemophagocytic lymphohistiocytosis. It is a hyperinflammatory complication observed to be comorbid with a variety of autoimmune diseases, including adult-onset Still’s disease and systemic juvenile idiopathic arthritis. Macrophage activation syndrome is less commonly detected in adult patients with systemic lupus erythematosus, which, if untreated, can be fatal, though determining the optimum treatment strategy is still a challenge.Case presentationHerein, we report a case of macrophage activation syndrome in a 33-year-old Egyptian female as an unusual complication of a systemic lupus erythematosus flare in adult patients. Our patient was initially treated with a combination of intravenous methylprednisolone pulse therapy and intravenous immunoglobulin therapy, which was followed by a course of oral prednisolone and oral cyclosporine with little response. Switching from oral prednisone to intravenous dexamethasone sodium phosphate showed a more favorable clinical and biochemical response.ConclusionMacrophage activation syndrome is less commonly detected in adult patients with systemic lupus erythematosus. Our case demonstrates that dexamethasone sodium phosphate can be a successful alternative treatment for patients with systemic lupus erythematosus complicated by macrophage activation syndrome in whom the response to pulse methylprednisolone was inadequate to manage their illness, proving to be remarkably effective in a relatively short time frame.

O22 An inflammatory journey from brain to skin: a case of En Coupe De Sabre scleroderma presenting with raised intracranial pressure, seizures, and uveitis

O22 An inflammatory journey from brain to skin: a case of En Coupe De Sabre scleroderma presenting with raised intracranial pressure, seizures, and uveitis

SAT-490 A Case of Unilateral Exophthalmos Due to Thyroid Orbitopathy and Its Association with Chronic Kidney Disease

Graves ophthalmopathy(GO)is the most common extra-thyroidal manifestation of Graves’ disease (GD). Most cases of GO are bilateral which may be asymmetric, whereas unilateral ophthalmopathy is less common and has been observed in 9-15% cases. Association between chronic kidney disease and unilateral Grave’s ophthalmopathy in a clinically euthyroid patient is rare. We report a case of a 24-year-old male with no previous history of any chronic medical illnesses who presented with protruded right eye for the past 6 months. He did not have any other visual symptoms or symptoms related to thyroid disease. Laboratory results revealed low TSH, normal free T3 and free T4. TSH receptor antibodies were positive. He also had elevated serum creatinine at 418 umol/L (normal levels 64 - 110 umol/L). US KUB showed bilateral small sized kidneys and increased parenchymal echogenicity suggestive of chronic kidney disease. MRI Head showed features suggestive of unilateral thyroid associated orbitopathy. Patient received 1-week course of oral prednisolone 10 mg per day after which his exophthalmos improved.Case report: A 24 year old male with no previous history of any chronic medical illnesses, presented to the clinic with protruded right eye for the past 6 months that was progressively getting worse. There was no eye pain, visual changes, ophthalmoplegia, dryness or discharge from eye. Patient did not report any other symptoms, Physical examination revealed a comfortable man with protruded right eye, lid retraction, normal eye movements and no signs of orbital cellulitis. Neck examination was significant for a mild diffuse goitre. Laboratory studies were significant for haemoglobin of 12.1 g/dl (normal 13-17 g/dl). He also had elevated serum creatinine at 418 umol/L (normal 64 - 110 umol/L). Serum electrolytes, liver function tests and lipid profile were within normal range. 24 hr urine collection showed 3.08 gm/24 hr proteinuria. Serum TSH was 0.04 mIU/L (normal 0.45 - 4.5 mIU/L), free T4 was 13.8 pmol/L (normal 9 - 20 pmol/L) and free T3 was 4.56 pmol/L (normal 2.89 - 4.88 pmol/L). Thyrotropin Receptor Ab titre was 4.69 IU/L (normal 0.00 - 1.75 IU/L). ANA, ANCA, C3, C4, Anti thyroid peroxidase and Anti GBM antibodies were negative. Screening for hepatitis B, C and HIV was negative US KUB showed bilateral small sized kidneys and increased parenchymal echogenicity suggestive of CKD. MRI Head was remarkable for proptosis of the right eye with increased retro-orbital fat, thickening and T2 hyper-intensity with sparing of the tendinous insertion involving the right inferior, medial, superior and lateral rectus muscles with crowding at the orbital apex. Features were suggestive of unilateral thyroid associated orbitopathy. Patient received 1-week course of oral prednisolone 10 mg per day after which his exophthalmos improved. An association between CKD and GO in a clinically euthyroid patient is rare.

A Case Report: Recurrence of Vogt-Koyanagi-Harada Disease after Rapid Tapering of Corticosteroids

Background: Vogt-Koyanagi-Harada's disease (VKHD) is an autoimmune disorder that targets melanocyte-rich tissues in genetically susceptible individuals. It can affect the eyes, inner ears, skin, hair, and meninges. When VKHD affects the eye, it can cause bilateral granulomatous panuveitis with exudative bullous retinal detachment and is usually associated with meningismus. It may lead to permanent loss of visual acuity. While the pathogenesis remains unknown, viral infection has been implicated. The most accepted theory remains a T-cell mediated autoimmune reaction against one or more antigens associated with melanocytes, melanin, and retinal pigment epithelium (RPE). Case presentation: We report the case of a 17-year-old male patient presenting to our outpatient department for vision loss. The patient reported a 2-week history of hearing loss, tinnitus, and episodic headaches associated with vomiting. Physical examination showed white lashes (poliosis) and a bilateral decrease in visual acuity. Fluorescein angiography showed bullous exudative retinal detachments. The patient was diagnosed with VKHD. His symptoms resolved following a course of oral prednisolone that was initiated at 60 mg per day (1mg/Kg/day), then tapered down over 6 weeks. Six months later, the patient presented again with more pronounced symptoms. His Best Corrected Visual Acuity (BCVA) was lower than it was on his first presentation, and the retinal exam showed a sunset glow fundus and degenerative changes in the RPE. Combined therapy with prednisolone and methotrexate resulted in a complete resolution of symptoms, and his BCVA returned to more than 20/40. Conclusion: This case emphasizes the importance of avoiding the rapid tapering of corticosteroids in VKHD due to the high risk of disease recurrence.

4. A paraneoplastic case of dermatomyositis

4. A paraneoplastic case of dermatomyositis

3. Dermatomyositis and underlying bilateral benign endometrioma

IntroductionThe link between ovarian malignancy and paraneoplastic dermatomyositis is well established. Association with underlying benign ovarian pathology is much less certain, with only scarce case report evidence available. This represents the first case of dermatomyositis with associated bilateral ovarian chocolate cysts.Case descriptionA 45-year-old female presented with classical features of dermatomyositis, with a background of hypertension and non-alcoholic fatty liver disease. She had proximal limb weakness, neck flexor weakness, dysphagia and dysphonia, accompanied by a scaly, erythematous rash over her face, chest, abdomen and lateral thighs. Serum creatine kinase (CK) at presentation was 4923 IU/L. An MRI scan of her thighs showed widespread muscle oedema affecting both legs and the pelvis, including the quadriceps, iliacus and psoas muscles. A punch biopsy of the skin on her thigh showed only post inflammatory pigmentation. Lung volumes were reduced (FEV1 69% predicted). Video fluoroscopy confirmed moderate-severe pharyngeal stage dysphagia. CT scan of the chest, abdomen and pelvis revealed enlarged, unilocular cysts in each ovary, the right cyst measuring 7 cm and the left, 5 cm in maximal diameter. Subsequent transvaginal ultrasound confirmed bilateral unilocular, thick walled cysts with a ground-glass appearance, which would be consistent with benign endometriomas. A further interval ultrasound after 1 month showed no change in their appearance or size. Serum CA125 was 104 kU/L.The patient was treated with 3 pulses of 1g intravenous (IV) methylprednisolone, followed by 2 courses of IV immunoglobulin 2g/kg over 5 days. This was followed by a gradually reducing course of oral prednisolone, starting at 50mg OD PO alongside 15mg weekly of oral methotrexate. She has responded well to treatment, with near normalisation of her objective manual muscle testing scores and normalisation of the serum CK value. Surgery is now planned for a total abdominal hysterectomy and bilateral oophorectomy.DiscussionExtensive investigation has not demonstrated any other underlying malignancy to account for the development of dermatomyositis. Initial limited and unsustained clinical response to IV methylprednisolone prompted further treatment with IV immunoglobulin. Surgical histology results are now awaited to confirm the suspected diagnosis. It will be very interesting to see if surgical removal of the cysts has any impact on the clinical manifestation of disease.Key learning pointsBenign ovarian pathology should not be dismissed as a potential underlying mechanism for development of dermatomyositis.Conflicts of interestThe authors have declared no conflicts of interest.

5. Isolated head drop: first symptom of anti-synthetase syndrome

IntroductionHead drop is an unusual clinical manifestation of myositis or neuromuscular weakness, and typically requires extensive investigation to identify the underlying pathology. Case report data are limited on this topic.Case descriptionA 72-year-old man with a background of extractable nuclear antigen (ENA) negative mixed connective tissue disease, trigeminal neuralgia, interstitial nephritis and ischaemic heart disease presented with an isolated head drop. On further prompting, he confirmed mild shortness of breath on exertion. He had sclerodactyly but no features of mechanic’s hands. Serum creatinine kinase was 1398 IU/L. Electromyography was myelopathic. Muscle biopsy taken from the neck was inconclusive. He had a strongly positive anti-nuclear antibody (ANA) test, and borderline myositis ENA panel result with borderline positive anti-EJ antibody.The myositis panel result prompted further investigation for underlying interstitial lung disease. Pulmonary function testing revealed a restrictive picture, with KCO of 82%. High resolution computed tomography showed areas of fine interstitial fibrotic change in all lobes.He was treated with a reducing course of oral prednisolone and 15mg weekly oral methotrexate with an excellent clinical response, with resolution of the head drop and normalisation of serum CK value. Repeat pulmonary function testing remains stable after 5 months.DiscussionThis is an unusual presentation of presumed anti-synthetase syndrome. As methotrexate had already been prescribed before the underlying lung pathology was identified with resulting excellent clinical response and stable respiratory symptoms, a multi-disciplinary decision was made to continue with this choice of disease modifying agent. The decision to use methotrexate in the presence of interstitial fibrosis is always difficult, and requires close liaison between rheumatology and respiratory colleagues.Key learning pointsPositive anti-synthetase antibodies should prompt further investigation for underlying interstitial lung disease. Head drop can be a first symptom of autoimmune myositis and warrants further investigation.Conflicts of interestThe authors have declared no conflicts of interest.

18. A case of atypical adult-onset Still’s disease

IntroductionAdult-onset Still’s disease, an auto-inflammatory disease, is well described in the literature and tends to respond well to IL-1 blockade. A sub-group of adult patients may present with some of the typical symptoms and findings which are commonly associated with autoinflammatory conditions but may not fulfil the criteria to obtain a specific diagnosis. It has been reported in the literature that these patients may represent an ’undifferentiated systemic autoinflammatory disorder’ and case reports support the use of anakinra as a viable treatment option in this sub-group.Case descriptionA 54-year-old female presented to rheumatology in 2014 with a two year history of recurrent episodes of sore throat, pyrexia, night sweats, polyarthralgia, headaches and bilateral episcleritis. Each episode recurred monthly and lasted three to five days before resolving. This patient’s condition did not fulfil Yamaguchi’s criteria for adult-onset Still’s disease. Investigations revealed an elevated ferritin which ranged between 500-2700, CRP greater than 300, ESR greater than 120. Extensive investigations excluded an infective or malignant cause and included virology, autoimmune and vasculitis screen, IGRA, blood cultures, transthoracic echo, ultrasound abdomen, CT chest/abdomen/pelvis and CT-PET scan. Genetic screening for hereditary autoinflammatory disorders was negative. Serum immunoglobulins (including IgD) and serum protein electrophoresis was unremarkable. The patient’s symptoms and inflammatory markers were responsive to high dose oral prednisolone, however, her symptoms recurred with steroid tapering. The patient was commenced on Methotrexate 25mg once weekly by subcutaneous injection to facilitate steroid weaning in 2015. The patient initially responded well to Methotrexate and was successfully weaned off steroids completely, she remained asymptomatic for two years. In 2017, she experienced frequent flares of her condition and continued to have active episcleritis on ophthalmological assessment. The patient required multiple courses of oral prednisolone to control her regular flares. The decision was taken to commence the patient on anakinra 100mg once daily subcutaneous injections one year ago in combination with methotrexate. The patient’s condition has remained in remission since commencing anakinra in 2018 with resolution of her symptoms, episcleritis and serum inflammatory markers and is no longer requiring oral prednisolone.DiscussionThis patient exhibited signs and symptoms which are commonly associated with auto-inflammatory conditions. Due to the clinical phenotype and negative genetic testing, this patient did not fulfil any criteria to be diagnosed with a specific auto-inflammatory disease. She did not fulfil Yamaguchi’s criteria for adult-onset Still’s disease. It is likely that this patient represents a case of ’atypical adult-onset Still’s disease’ or an ’undifferentiated systemic autoinflammatory disorder’ which is supported by her excellent response to IL-1 blockade in the form of anakinra.Key learning pointsThis case highlights the importance of considering ’undifferentiated systemic autoinflammatory disorder’ or ’atypical adult-onset Still’s disease’ as a potential diagnosis for the subgroup of patients presenting atypically who do not fulfil any current classification or diagnostic criteria for a specific autoinflammatory disease. This case also illustrates that IL-1 blockade is potentially a viable treatment option in these patients.Conflicts of interestThe authors have declared no conflicts of interest.

31-Year-Old Woman With Jaundice and Abdominal Pain

31-Year-Old Woman With Jaundice and Abdominal Pain