Steroid Therapy Research Articles

Objective: To investigate the clinicopathological characteristics of type 2 autoimmune pancreatitis (AIP) and to explore its relationship with inflammatory bowel disease (IBD). Methods: AIP cases confirmed by pathology in the First Affiliated Hospital of Zhejiang University School of Medicine from 2009 to 2024 were collected. According to the International Consensus Diagnostic Criteria (ICDC) for AIP, 11 patients were identified as histological level 1 (definite) or level 2 (probable) type 2 AIP. Their clinical manifestations, laboratory test results, imaging features, and histopathological characteristics were analyzed, and a follow-up was conducted. Meanwhile, 130 patients with type 1 AIP diagnosed in our hospital during the same period were selected as control group. Results: Among 141 AIP patients, 11 cases (7.8%, 11/141) were diagnosed with type 2 AIP, including 7 cases of histologically level 1 and 4 cases of level 2. There were 10 male patients and 1 female patient, with a median age of 37(31,46) years (range: 25-47 years). Three patients were complicated with ulcerative colitis (UC). Compared with type 1 AIP patients, type 2 AIP patients were younger, often presented with acute pancreatitis or abdominal pain as the initial symptom, and had a close association with IBD (P<0.05). Laboratory tests showed that only 1 patient had slightly elevated serum IgG4, while the other 10 patients had normal serum IgG4 levels. Serum CA19-9 was elevated in 8 patients, and the percentage of peripheral blood neutrophils was increased in 9 patients. Imaging findings revealed diffuse pancreatic enlargement in 8 patients and localized enlargement in 3 patients (2 cases in the pancreatic head and 1 case in the pancreatic body-tail). Magnetic resonance cholangiopancreatography (MRCP) showed main pancreatic duct stenosis in 5 cases (5/7). Histopathological features included 7 cases of level 1 type 2 AIP that showed neutrophilic infiltration in the pancreatic duct epithelium and massive neutrophilic infiltration between the acini. Immunohistochemistry showed that only 1 case had <5 IgG4-positive plasma cells per high-power field (HPF), while the other 10 cases were negative. All 11 patients with type 2 AIP received steroid therapy, and no recurrence was observed during the follow-up period of 5 to 174 months. Conclusions: Type 2 AIP has unique clinicopathological characteristics. It is more commonly found in young patients and often presents with manifestations similar to acute pancreatitis. Histologically, neutrophilic infiltration in the ductal epithelium is the common feature. Type 2 AIP is closely associated with IBD, especially UC.

Background: Tolosa-Hunt syndrome (THS) is a rare, inflammatory condition characterized by painful ophthalmoplegia due to inflammation of the cavernous sinus or superior orbital fissure, most commonly involving the oculomotor, trochlear, and abducens nerves. This case report describes an atypical presentation of Tolosa-Hunt syndrome in a 64-year-old male with sequential involvement of the right abducens (CN VI) and facial (CN VII) nerves, along with persistent right-sided headache unresponsive to anti-inflammatory drugs. Case presentation: The patient initially presented with diplopia due to right abducens nerve palsy and persistent drug-resistant headache lateralized to the right side of the head (without face involvement or accompanying neuro-vegetative autonomic symptoms), followed months later by facial weakness, involving upper and lower facial muscles. Contrast-enhanced brain MRI revealed inflammation of the right facial nerve, without any other acute lesions. CSF analysis and blood exams were unremarkable, excluding other possible infective or inflammatory causes. Based on the clinical course of the disease and exclusion of other neurological disorders, a diagnosis of Tolosa-Hunt syndrome was made. The patient was treated with high-dose oral corticosteroids, resulting in partial clinical improvement in the next few days and complete resolution of symptoms at 1-month follow-up. Conclusion: This case report highlights the variability of Tolosa-Hunt syndrome, including its atypical presentation with facial nerve involvement, and underscores the importance of early diagnosis and steroid therapy for favorable outcomes.

Aim Predicting treatment outcomes in periprosthetic joint infections (PJI) remains challenging as numerous surgical, patient-specific, and implant-related variables influence outcomes throughout the entire treatment course over several weeks. In Addition, therapy regimes may change based on pathogen identification or wound complications, impacting and altering infection eradication. This study aimed to evaluate a machine learning (ML) model for predicting infection eradication using a specific PJI database for this purpose, focusing on (a) predictive accuracy and (b) the influence of pre- and postoperative variables. Method A retrospective analysis of 649 PJI cases treated at a university hospital (2010–2023) included 218 clinical variables. After data cleaning, scaling, and preprocessing, 57 variables from 413 cases with ≥12-month follow-up were used to predict infection eradication according to the 2013 Delphi consensus criteria. Two XGBoost classifiers were trained: one using only 39 preoperative variables and one using the full 57-variable dataset. LASSO regression guided feature selection. Model performance was assessed via accuracy, area under the curve (AUC), sensitivity, and specificity. Univariate logistic regression identified significant clinical predictors for comparison with conventional statistics. Results The ML model using all variables achieved an AUC of 0.89, sensitivity of 0.87, and specificity of 0.84. Using only preoperative data, AUC remained high at 0.88 (sensitivity 0.85, specificity 0.81). Logistic regression identified key risk factors, including prior infection (OR 0.19), fistulas (OR 0.23), rifampicin use (OR 2.8), oral antibiotics (OR 0.68), surgery duration (OR 0.03), histology at reimplantation (OR 4.1), number of previous surgeries (OR 0.29), and steroid therapy (OR 0.02). All were confirmed by the ML model, which also revealed additional potential predictors. Conclusions Machine learning enabled accurate prediction of PJI resolution. Notably, preoperative data alone yielded robust predictive performance. The model validated known risk factors and uncovered further clinically plausible predictors, warranting future prospective validation.

Giant cell myocarditis is a rare, rapidly progressive, potentially lethal disease caused by a T-cell-mediated inflammation of the myocardium. Rapid, early diagnosis is complicated by the diverse clinical picture (acute coronary syndrome, heart failure, arrhythmia episodes). Our 52-year-old female patient has no significant medical history; she had an upper respiratory tract infection prior to admission. She presented to the ER with chest pain and dyspnea. ECG showed anterior ST-elevation. Urgent coronary angiography showed an intact coronary system. Initially, she was hemodynamically stable, and echocardiography showed a mildly reduced EF. During a short observation period, her condition rapidly progressed, she became hypotonic, and due to third-degree AV block and significant pausa, temporary PM implantation was performed. She was admitted to our clinic due to suspected progressive myocarditis. Her condition stabilized with dual circulatory support. Due to continuous PM requirement, performing cardiac MRI was not possible. Cardiac biopsy confirmed giant cell myocarditis. We started high-dose IV steroid therapy. As a result, left ventricular function improved and necroenzyme levels decreased. According to the latest literature recommendations, her steroid treatment was supplemented with mycophenolate mofetil and tacrolimus, which gradually improved the EF and AV conduction, eliminated the need for a pacemaker, we removed the iPM, and stopped the inotropes and vasopressors. Extensive bacteriological, viral serological and immunological tests did not confirm active infection or immunological abnormalities. Cardiac MRI showed good left and right ventricular ejection fractions and late non-ischemic contrast enhancement. She was discharged after 2 weeks of treatment in a stable clinical condition, with the use of complex immunosuppressive therapy. She receives regular follow-up in the heart transplantation department of our clinic. With immunosuppressive treatment, she is symptom-free, EF 60%, and has no arrhythmia or conduction disorders. Our patient's case draws attention to the fact that in case of suspected myocarditis, myocardial biopsy and rapid diagnosis can be life-saving. It is rare that with combined immunosuppressive therapy and regular follow-up over a period of 1 year, the patient remained symptom-free, had good cardiac function, and was able to avoid possible heart transplantation and implantation of a circulatory support device.

Background Adrenal crisis, characterized by acute cortisol deficiency, is a rare, life-threatening condition that can precipitate cardiovascular collapse and heart failure (HF). Its role in HF with preserved ejection fraction (HFpEF) is underrecognized, particularly in cancer patients receiving therapies that impair adrenal function. This case series examines the clinical features, management, and outcomes of HFpEF induced by adrenal crisis, emphasizing early diagnosis and treatment. Methods We retrospectively analyzed four patients diagnosed with HFpEF secondary to adrenal crisis between January 2022 and January 2025 at Qingdao Central Hospital and Qingdao Municipal Hospital. Inclusion criteria included clinical evidence of adrenal crisis (low cortisol, hypotension, steroid responsiveness) and echocardiographic confirmation of HFpEF (EF ≥50%). Data on demographics, clinical presentation, laboratory findings, echocardiography, and outcomes were analyzed descriptively. Results The cohort comprised three males and one female (aged 41–77 years), all with HFpEF (EF 50%–60%). Two presented with myocardial infarction (one NSTEMI, one STEMI), and two had malignancy with adrenal metastasis (renal, lung). Three exhibited hypotension. Initial BNP levels ranged from 518.93–619.13 pg/mL, decreasing to 108.06–287.63 pg/mL pre-discharge after hormone replacement therapy and HF management. Mean EF improved by 1.75% (range: 0%–3%) at one-month follow-up, with BNP further declining to 20.36–177.24 pg/mL. All patients achieved symptom resolution with no recurrence reported. Conclusion Adrenal crisis is a rare, reversible etiology of HFpEF in patients with diverse underlying conditions, potentially including those with cancer-related adrenal dysfunction or prior therapies. Prompt steroid therapy appears to improve cardiac function and outcomes, suggesting a need for heightened awareness and consideration of adrenal screening in at-risk populations, such as those with malignancy, tuberculosis, or other causes of adrenal insufficiency. Larger studies are needed to confirm these preliminary findings and establish the prevalence of this etiology across different subpopulations.

Isolated oculomotor nerve palsy (ONP) following mild traumatic brain injury (TBI) is rare and often presents diagnostic challenges. Typically associated with diffuse axonal injury and poor prognosis, ONP lacks comprehensive radiological documentation when no skull base fractures along the course of the 3rd Nerve or brainstem injuries are evident. This study explores the diagnostic utility of contrast-enhanced magnetic resonance imaging (CEMRI) in identifying ONP cases in mild TBI patients. A retrospective analysis was conducted on six patients diagnosed with isolated ONP after mild TBI, with no evident skull base fractures along the course of the 3rd Nerve or brainstem findings. All patients underwent CE-MRI to identify structural or vascular anomalies along the course of the third cranial nerve. Clinical presentations, imaging findings, and outcomes were meticulously documented and reviewed by a neuroradiologist. CE-MRI findings revealed consistent abnormalities in all cases. Thickening, blooming, and post-contrast enhancement of the cisternal portion of the third cranial nerve were observed, with two cases demonstrating extension into orbital segments. Despite the absence of fractures or direct injuries, partial recovery was noted in most cases, facilitated by targeted steroid therapy in some instances. These imaging patterns suggest indirect mechanisms such as traction, vascular compromise, or intraneural hemorrhage as potential causes of ONP. High-resolution CE-MRI proves instrumental in diagnosing isolated ONP in mild TBI patients, even without conventional radiological indicators. Early imaging and intervention may improve recovery outcomes. This study underscores the significance of including CE-MRI in evaluation protocols for ONP. It highlights the importance of further research to unravel the underlying pathophysiology and optimize therapeutic approaches for these patients.

The current phase of immune checkpoint inhibitors has improved the clinical outcomes of many cancer therapies; however, these agents may also lead to some high-consequence immune-related adverse events. We report two cases of cancer patients treated with tislelizumab, one with liver metastases after surgery for rectal cancer and one with non-small cell lung cancer. In both cases, elevated cardiac enzyme markers were observed, and one developed fatal myocarditis, while the other was treated with high-dose methylprednisolone steroid therapy and eventually improved. The probability of immune-associated myocarditis has been reported to be only 0.39%, but once myocarditis occurs, the mortality rate is more than 50%, making it a rare but serious side effect. Here, we discuss the antitumor treatment, the mechanism of immune-associated myocarditis induced by tislelizumab, the evaluation of the association, and the treatment after the development of immune-associated myocarditis in two patients, with the aim of improving clinical awareness of tislelizumab-induced immune-associated myocarditis and promoting timely judgment and appropriate therapeutic measures.

Introduction: Cholestasis is a pathological condition associated with immune-mediated processes that interfere with bile flow, thus requiring early diagnosis and prompt treatment to ensure optimal outcomes. The use of steroid therapy in the treatment of cholestasis has not been previously reported. Case: A 2-month and 9-day-old male infant presented with jaundice beginning at one week of age, followed by pale stools and tea-colored urine. Physical examination revealed jaundice, hepatomegaly, and splenomegaly. Laboratory investigations showed elevated liver enzymes: aspartate aminotransferase (AST) 292 U/l, alanine transaminase (ALT) 187 U/l, gamma-glutamyl transferase (GGT) 312.7 U/l, and alkaline phosphatase (ALP) 882 U/l), along with elevated bilirubin level (total bilirubin 7.9 mg/dl and direct bilirubin 6 mg/dl). Cytomegalovirus (CMV) IgG was reactive. A two-phase abdominal ultrasound examination revealed a preprandial gallbladder size of 0.65 x 0.33 x 1.91 cm, a postprandial size of 0.37 x 0.29 x 0.73 cm. Liver biopsy revealed chronic hepatitis and mild fibrosis (F1). The patient was treated with methylprednisolone, which was gradually tapered off. Following steroid therapy, liver enzyme and bilirubin levels normalized, gallbladder size increased, and stool color darkened. Posttreatment gallbladder size showed an increase (preprandial 3.05 x 0.96 x 0.72 cm, postprandial 2.45 x 0.72 x 0.82 cm). Conclusions: Steroids may improve bile flow and prevent further liver damage. Targeting immune mechanisms could represent a promising approach for future cholestasis treatments.

This review provides a comprehensive overview of the surgical management of cataract in patients with uveitis associated with retinal autoimmune diseases. Cataract in uveitic eyes develop as a result of chronic intraocular inflammation and long-term steroid therapy and are one of the most common causes of vision loss. Cataract surgery in this patient group involves varying difficulties depending on systemic and ocular disease activity and accompanying ocular comorbidities. A detailed ophthalmologic and systemic evaluation should be performed before surgery, especially considering the status of the retina and optic nerve structures. Preoperative control of inflammation for at least three months is recognized as an important prognostic factor for reducing postoperative complications and enhancing surgical outcomes. Appropriate immunosuppressive therapy with local and systemic corticosteroids before, during, and after surgery plays a critical role in reducing postoperative inflammation and the risk of developing cystoid macular edema (CME). Phacoemulsification with intraocular lens implantation is the preferred surgical technique for most patients with uveitic cataract. Surgery in these cases is often challenging because of miotic pupil, posterior synechiae, iris atrophy, zonular weakness, and pupillary membranes. Therefore, the use of pupil dilators, iris manipulations, and additional procedures may be required. Patients should be closely monitored postoperatively for excessive inflammation, CME, increased intraocular pressure, hypotony, and other potential complications. Recent advances in surgical techniques, immunomodulatory therapies, and intraocular lens technologies have significantly improved the safety and visual outcomes of cataract surgery in patients with uveitis. However, an individualized approach should be adopted for each patient and it should be kept in mind that the risks of unforeseen complications are higher than those of traditional cataract surgery, and careful follow-up should be maintained after surgery.

Hepatitis-associated aplastic anemia (HAAA) is a rare but potentially life-threatening form of acquired aplastic anemia. Since 2022, our center has observed an increase in HAAA cases. This study examines pediatric HAAA cases to enhance understanding of its presentation, diagnosis, and treatment outcomes, aiming to guide future research and care protocols. A retrospective review was conducted on 5 pediatric HAAA patients treated between 2022 and 2023 at a tertiary children's hospital in the Midwestern United States. Data included clinical presentation, diagnostics, bone marrow and liver pathology, treatments, and clinical course. Immunohistochemical analysis was performed on liver biopsies. As a result, none of the 5 patients developed liver failure. One patient had a genetic mutation associated with an immune-mediated disease; other genetic tests were negative. Histopathology revealed consistent CD8 T-cell infiltration in the liver and bone marrow, with a median CD4/CD8 ratio of 0.5. The median interval from hepatitis onset to pancytopenia was 7 to 9 weeks, with a median follow-up of 2.5 years. Four patients developed severe aplastic anemia (sAA), and 1 had nonsevere aplastic anemia (NSAA). Steroid therapy was insufficient in 4 cases, necessitating antithymocyte globulin (ATG) and cyclosporine. Due to nonresponse, 4 patients required stem cell transplantation (SCT). HAAA can rapidly progress to sAA, highlighting the importance of early, aggressive intervention. Equine ATG and cyclosporine should be initiated promptly, but refractory cases often require SCT. Further research is essential to refine therapeutic strategies and improve outcomes.

Indian National Association for the Study of the Liver Position Statements on Prevention, Diagnosis, and Management of Hepatitis B Virus Infection in India.

Abstract Introduction Nemaline rod myopathy (NM), also called rod myopathy, is a rare muscle disease characterised by the presence of thread-like structures called nemaline rods in the muscle. The myopathy can be childhood-onset which is inheritable or adult-onset which is sporadic and acquired. Patients develop muscle weakness, hypotonia and reduced, even absent, reflexes. The muscle weakness can lead to respiratory muscle weakness and swallowing difficulty, posing a major cause of morbidity and mortality in such patients. We describe a case of sporadic late-onset nemaline myopathy (SLONM) secondary to inflammatory myositis. Case description A now 80-year-old Anglo-Indian lady was diagnosed with limited cutaneous systemic sclerosis (SSc) in her 60s with longstanding history of Raynaud’s phenomenon. She has anti-centromere antibodies, calcinosis, nailfold abnormality and reflux. Since 2014, she predominantly complained of exertional breathlessness and demonstrated persistently modestly elevated creatine kinase (CK). She had proximal muscle weakness, mild neck weakness but no bulbar weakness. Electromyography revealed peripheral sensorimotor polyneuropathy and myopathic changes in proximal muscles. MRI demonstrated atrophy of gluteus and hamstrings with fat replacement. Muscle biopsy was in keeping with polymyositis with focal lymphocytic infiltrate and diffuse sarcolemma MHC Class 1 upregulation. For many years, due to fear of side effects, she had declined steroid therapy. The breathlessness was extensively investigated. Pulmonary function showed mildly reduced DLCO. HRCT excluded SSc-related interstitial lung disease. Coronary angiography, right heart catheterisation and cardiac MRI did not show evidence of pulmonary hypertension although demonstrated mild coronary artery disease. Her breathlessness was attributed to possible respiratory muscle weakness secondary to SSc-related myositis. In 2022, following worsening symptoms and declining DLCO, she agreed to steroid therapy though the treatment was restricted due to side effects. She had evidence of axial weakness then. CK fell significantly with steroid but there was minimal subjective improvement. She did not tolerate methotrexate and mycophenolate mofetil. During 2024, her symptoms continued though her CK was within normal range, probably due to low muscle. Her troponin T was raised. Repeat muscle biopsy showed the presence of nemaline rods on Gomori trichrome staining with no inflammatory changes and only focal MHC Class 1 upregulation. The neurohistopathologist re-evaluated the muscle biopsy performed in 2014 and confirmed the absence of nemaline rods then. Our patient was subsequently found to have weak Mi-2 antibodies. She tested negative for HIV and there was no evidence of concurrent MGUS. Discussion The presence of atrophy does not suggest a further role of immunomodulatory therapy but SLONM can exist in the context of prior inflammatory processes - in this case due to longstanding SSc-related myositis. Rituximab treatment has recently been approved and our patient’s journey continues. Nemaline rod myopathy was first described in 1963 as a muscle disease in infancy; the adult-onset form was later described in 1966. Sporadic late-onset nemaline rod myopathy (SLONM) is an acquired muscle disease and extremely rare. The diagnosis can be challenging as the presence of nemaline rods can be limited and expert knowledge is required for pathological confirmation. The disease can be associated with MGUS and HIV. In non-MGUS-associated disease, patients tend to show slow progression in their muscle symptoms and be unresponsive to standard treatment. Other cases of SLONM are associated with various autoimmune conditions, including Sjögren’s disease. In a study of 49 patients with SLONM, 66.7% were found to have variable sarcolemmal HLA-ABC expression as a core feature of myositis and the predominant inflammatory cells were CD68+ cells. This study suggested inflammation may be involved in the pathogenesis of the disease. We believe our patient developed SLONM secondary to longstanding SSc-related inflammatory myositis. Key learning points • Sporadic late-onset nemaline myopathy (SLONM) is a rare acquired muscle disease characterised by proximal muscle weakness which can lead to respiratory muscle weakness. • SLONM can be associated with MGUS and HIV. In non-MGUS-associated SLONM, patients often progress slowly and tend not to respond to standard treatment for myositis. • In certain cases, nemaline rods can be observed secondary to inflammatory process. • Our case highlights the difficulty in reaching the diagnosis of SLONM and the value of repeating muscle biopsy.

Abstract Introduction A gentleman presented to hospital with a purulent cough, shortness of breath and fevers. He had recently been admitted to hospital and treated for a chest infection. He was re-admitted and treated as a hospital acquired pneumonia. Despite intensive antibiotic therapy, his condition continued to deteriorate. Eventually, he was diagnosed with granulomatosis with polyangiitis, however despite delayed treatment he continued to deteriorate and subsequently passed away. This case highlights how diagnosis bias delayed treatment and resulted in a poor outcome for this patient. Case description 64 year old gentleman presented to Rotherham Hospital with a purulent cough, shortness of breath and oxygen requirement. He had recently been admitted with a chest infection. Chest X Ray showed patchy consolidation, and he had raised inflammatory markers and AKI stage 1. He was therefore re-admitted and commenced on treatment for a hospital acquired infection with IV piperacillin/tazobactam. He was otherwise fit and well, and worked as a kitchen joiner. Despite 10 days of piperacillin/tazobactam, this gentleman had ongoing oxygen requirement and worsening renal function. Sputum cultures had come back as negative. In light of the worsening AKI, it was decided to test him for other possible causes for his respiratory distress. ANCA antibodies came back as positive. He was reviewed by Rheumatology and commenced on steroid therapy. Despite this, his condition continued to deteriorate and he had increasing oxygen requirements. Despite escalation to ITU, he continued to deteriorate and subsequently passed away. Discussion This case highlighted that it is important to consider alternative diagnosis when patients do not respond to initial treatment. In this case, this gentleman was fit and well. In theory if he had a hospital acquired pneumonia, it would be expected that he would start to improve quickly with broad spectrum antibiotics. However, it took 10 days to identify that he was not improving and this therefore delayed the initiation of the treatment required to improve his condition. Further, awareness of diagnosis bias was really significant in this case. As he was referred to the ward from AMU as a hospital acquired pneumonia, this is what he was treated as. It is very important to consider rarer diagnosis if patients are not responding as expected. Since this case, the ward where this happened now has bi-weekly board rounds with an MDT of staff to discuss results and diagnosis to have a wider discussion. This helps to initiate team discussion and wider thinking regarding diagnosis in order to “think outside the box.” Finally, awareness of rarer conditions is vital. Hence, doing talks at regional teaching regarding this case will help other resident doctors to be aware of conditions such as these, thus keeping them on their list of differentials. Key learning points • Common conditions are common, however if patients do not respond to treatment appropriately, it is important to think of differentials. • Having an awareness of rarer conditions such as granulomatosis with polyangiitis is essential in order to be considered on the list of differentials. • It is important to recognise diagnosis bias, and to involve the wider team to consider alternative differentials.

Visual Restoration in Rare Ocular Lyme Borreliosis Using Adjunctive Steroid and Antibiotic Therapy

Abstract Introduction This case series highlights the challenges in diagnosing late onset axial spondyloarthropathy in three patients who presented with musculoskeletal symptoms between the age of 45 to 65 and were initially diagnosed with polymyalgia rheumatica and remitting seronegative symmetrical synovitis with pitting edema (RS3PE). They were later found to have axial spondyloarthropathy and their treatment changed to biologic therapies with good response. Case description Case 1: A 51-year-old woman was diagnosed with PMR in December 2020 after presenting with shoulder and lower back pain and raised inflammatory markers. She was treated with prednisolone until April 2023. Her symptoms recurred, particularly affecting the lower back, hips, and thighs and MRI revealed acute-on-chronic right sacroiliitis and facet joint inflammation. Examination showed sacroiliac tenderness with a high BASDAI of 6.9 and spinal VAS of 9. She was started on adalimumab but later switched to secukinumab due to a hypersensitivity reaction, achieving good disease control (BASDAI 2.8, VAS 3). Case 2: A 69-year-old woman presented in April 2017 with bilateral shoulder and back pain and was treated for presumed PMR. Steroids were introduced and her response was atypical with improvement only after 7 days. There was suboptimal response seen with her CRP increasing and this led to an increase in her prednisolone dose. In March 2019, she underwent a PET CT scan which showed inflammation in her sacroiliac joint and this was confirmed on MRI scanning. HLA-B27 testing was positive and a diagnosis of ankylosing spondylitis was made in April 2019 (age 63). She was treated with adalimumab, then switched to etanercept due to primary failure, and is now stable on secukinumab (BASDAI 1.1, pain score 1/10). Case 3: A 63 year old lady was diagnosed with RS3PE in December 2019 following a sudden episode of illness with high inflammatory markers. She was a sportsperson and an avid horse rider. She was commenced on prednisolone but had struggled at lower doses with increased stiffness and a review in 2023 prompted MRI imaging which revealed evidence of bilateral symmetrical sacroiliitis. A diagnosis of ankylosing spondylitis was made (age 61). She commenced on anti-TNF therapy following trial of anti-inflammatories and is now established on adalimumab with good response. Discussion This case series highlights the challenges in diagnosing axial spondyloarthropathy (axSpA) particularly late onset axSpA and the clinical vigilance that axial spondyloarthropathy can present later in life and can mimic polymyalgia rheumatica (PMR). These three cases involve middle aged women (age 45-65) who presented with symptoms suggestive of PMR and RS3PE and were later found to have axSpA as the cause of their musculoskeletal symptoms. One patient was a sports person and an avid horse rider, and this may have played a role in her presentation at a later age as exercise classically improves inflammatory axial pain. All three patients were subjected to prednisolone therapy and two patients had prednisolone for several years. AxSpA and PMR are two conditions that share the same symptoms such as morning stiffness, fatigue, and pain in shoulder and pelvic girdles. PMR typically affects patients over 50, whereas axSpA is often thought to affect younger individuals. However, late-onset axSpA is under-recognized and can mimic PMR. Both conditions may show some response to corticosteroids, though PMR typically has a dramatic response. In axSpA, the response may be partial or temporary and patients with partial or suboptimal responses to steroid therapy should prompt re-evaluation of their symptoms for alternative diagnoses such as axSpA, haematological malignancies like myeloma or degenerative causes. Serological investigation with HLA-B27 and inflammatory markers may be useful and while HLA-B27 is not diagnostic on its own, its presence supports a diagnosis of axSpA especially in ambiguous presentations. ESR and CRP are often elevated in both PMR and axSpA, hence are not distinguishing features but may support the presence of ongoing inflammation. MRI imaging is invaluable in distinguishing axSpA from PMR when inflammatory axSpA is suspected. Key learning points 1. This case series highlights a diagnostic pitfall that has real consequences for patients - delayed treatment, ongoing inflammation, and potential long-term exposure and complications from prednisolone therapy. 2. It also challenges assumptions, especially the belief that axSpA is a disease of younger male individuals and that PMR only affects older adults, and emphasizes the dynamic nature of these diagnoses as they evolve with time and response to treatment. 3. Eliciting patients’ occupations, hobbies and the type of sports they do is crucial as individuals with axSpA who partake in greater levels of physical activity may have inflammatory pain which improves with activity and as a result presenting later in life and may be misdiagnosed as polymyalgia rheumatica. 4. This case series also highlights the diagnostic challenges of late onset axSpA and the importance of remaining vigilant and re-evaluating diagnoses if response to steroids are suboptimal or when standard treatments fail.

Abstract Introduction Antisynthetase Syndrome (ASS) is a rare autoimmune condition associated with interstitial lung disease (ILD), arthritis, and myositis, characterised by anti-aminoacyl-tRNA synthetase antibodies. ILD may be the first or only manifestation and can resemble infection making early diagnosis challenging. Anti-EJ antibodies are uncommon but strongly associated with ILD-dominant presentations. Our case follows a middle-aged woman with rapidly progressive ILD initially treated as infection. Her deterioration despite antibiotics alongside subsequent autoantibody profiling and radiological features led to the diagnosis of anti-EJ ASS. This case highlights the importance of prompt autoimmune screening and multidisciplinary input in atypical ILD. Case description A 54-year-old woman with type 2 diabetes, Raynaud’s phenomenon, and Charcot’s arthropathy presented with 4 weeks of progressive dyspnoea and pleuritic chest pain. CT pulmonary angiogram excluded pulmonary embolism but revealed diffuse ground-glass changes and peribronchovascular thickening, suggestive of atypical infection. She was treated with antibiotics and discharged with home oxygen. An outpatient high resolution CT scan was arranged. One month later, she re-presented with worsening dyspnoea, elevated inflammatory markers (CRP 136 mg/L), and hypoxaemia requiring 60l/min high-flow nasal oxygen therapy. Her admission chest radiograph revealed diffuse, bilateral air space opacification. She was admitted to ICCU and empirically treated for Pneumocystis jirovecii pneumonia with high-dose co-trimoxazole. Her HRCT scan revealed bilateral extensive lower lobe predominant peribronchial consolidation with diffuse ground glass changes in the upper lobes. With respiratory input, she was started on IV methylprednisolone 1g for 3 days for what was suspected to be a rapidly progressive interstitial lung disease/organising pneumonia. Significant improvement was seen in the subsequent 48 hours, and she was stepped down to oral prednisolone and low-flow oxygen therapy. Autoimmune testing revealed positive ANA, rheumatoid factor (81.9 IU/ml), anti-Ro52, and anti-EJ antibodies. Rheumatology, Radiology and Respiratory colleagues concluded a diagnosis of systemic autoimmune rheumatic disease–related ILD (SARD-ILD), consistent with anti-EJ antisynthetase syndrome. She was started on rituximab and cyclophosphamide for long-term immunosuppression. The patient continues to improve, with reduced oxygen requirements and no further hospitalisations. This case illustrates how rapidly progressive ILD can mimic infection, delaying accurate diagnosis. Early steroid therapy, autoimmune serology, and multidisciplinary collaboration proved key in identifying and managing this rare presentation. Discussion Organising pneumonia is a type of ILD that can mimic infectious pneumonia, presenting with cough, fever, and dyspnoea (Cordier, 2000). Diagnosis is based on radiological and histological findings and exclusion of other causes, such as infection and malignancy. In this case, organising pneumonia was suspected due to imaging features, clinical presentation, rapid deterioration and lack of response to antimicrobials. This patient’s serological findings confirmed suspicions that this was an autoimmune ILD. Antisynthetase syndrome (ASS) is characterised by antibodies against aminoacyl-tRNA synthetases, including anti-Jo-1, PL-7, PL-12, OJ, and EJ (Aggarwal et al., 2022). Anti-EJ is less common but is strongly associated with ILD as a primary or sole manifestation (Sawalha et al., 2020). Our patient lacked classic extrathoracic features such as arthritis or myositis, contributing to diagnostic uncertainty. The presence of anti-Ro52—frequently co-expressed in ASS—is associated with severe lung disease and worse prognosis (Yamasaki et al., 2017). Corticosteroids remain the first-line treatment for inflammatory ILD (Idiopathic Pulmonary Fibrosis guidelines, 2015). However, relapse rates are high in patients with anti-EJ antibodies treated with steroids alone. Combination immunosuppressive therapy, particularly with rituximab or cyclophosphamide, has shown better outcomes (Sawalha et al., 2020). Our patient responded favourably to this approach, highlighting the importance of early rheumatology input. This case supports the growing consensus that autoimmune serology and MDT assessment should be part of the early diagnostic workup in ILD with atypical features or rapid progression. With SARD-ILD comprising up to 30% of ILDs (Fischer et al., 2015), updated BSR guidelines and greater clinician awareness are needed to prevent missed or delayed diagnoses in complex cases. Key learning points • Antisynthetase syndrome should be suspected in patients with ILD and rapid deterioration despite appropriate infection management. • Anti-EJ antibodies are associated with ILD-dominant ASS, often without myositis or arthritis. • Co-existing anti-Ro52 is linked to more severe pulmonary involvement. • Imaging features mimicking COP warrant autoimmune screening if atypical or unresponsive. • High-dose corticosteroids are effective in acute inflammatory ILD flares. • Combination immunosuppressive therapy is preferred for long-term disease control. • Early MDT input (respiratory, radiology, rheumatology) is essential for accurate diagnosis. • National guidelines for SARD-ILD are needed to standardise care and improve outcomes. • Presentations mimicking infectious pneumonia warrant more in depth imaging and autoimmune screening if atypical or unresponsive.

Allogenic hematopoietic stem cell transplantation is compromised by transplant-related morbidity and mortality, particularly acute graft-versus-host disease (aGVHD). Early diagnosis or prediction of aGVHD is crucial for improving treatment outcomes. This study aimed to explore the utility of a biomarker panel for predicting and diagnosing aGVHD, as well as correlating biomarkers with aGVHD severity and response to treatment. In this prospective study of 83 pediatric patients undergoing allogeneic HSCT, patterns of seven biomarkers were followed starting from the day of stem cell infusion up to day 90 post-transplant. Patients with aGVHD exhibited significantly higher levels of IL-2Rα, IL-6, IL-8, and HGF at the onset of aGVHD. Furthermore, patients with refractory aGVHD demonstrated elevated levels of HGF on day 28 post-transplant. In aGVHD patients, the biomarkers exhibited a distinct pattern. With the exception of IL-6, all biomarkers significantly increased at the onset of aGVHD. Following treatment, most biomarker levels decreased, except IL-6, IL-8, and HGF, which remained elevated 28 days post therapy initiation. These three biomarkers were also significantly higher in steroid-refractory patients. In summary, IL-2Rα, IL-6, IL-8, and HGF show potential for aiding in the diagnosis of aGVHD. Additionally, HGF, IL-6, and IL-8 levels may serve as predictors of response to steroid therapy.

Abstract Introduction We present a case of a Caucasian female in her late 50s who developed aortitis secondary to underlying axial spondyloarthropathy. Despite multiple encounters with various specialists, the diagnosis was delayed due to longstanding misattribution of axial symptoms and atypical presentation characterised predominantly by cervical and sternoclavicular involvement. Case description A female in her 50s was re-referred to the rheumatology clinic by her GP with longstanding neck and groin discomfort that had progressively worsened over six months. On examination, she demonstrated synovitis of bilateral sternoclavicular joints, left knee and left ankle. Investigations revealed borderline rheumatoid factor positivity, negative anti-CCP antibodies, and negative HLA-B27. A sacroiliac joint X-ray was performed and reported as normal. Her past medical history was significant for a 20-year history of cervical spondylosis and fibromyalgia. Other conditions included migraine, asthma, iron deficiency anaemia, acne rosacea, recurrent urinary tract infections, and a liver haemangioma. Review of historic medical records revealed that she had been referred to rheumatology many years earlier for long-standing neck and lower back pain for which she had been under the care of the pain management team, receiving opioids and repeated trigger point injections in her neck and lower back, which had not provided optimal pain relief. Her symptoms were considered mechanical in nature at that time, and she was referred for physiotherapy. She was commenced on methotrexate for inflammatory arthritis. Despite clinical control of her peripheral arthritis, her inflammatory markers remained markedly elevated; at one point, her CRP reached 228 mg/L and ESR 122 mm/hr. Due to persistently elevated inflammatory markers, a PET scan was performed. This demonstrated intense increased uptake along the aorta—from the proximal ascending aorta to the distal abdominal aorta—consistent with large vessel vasculitis. Additionally, the scan showed intense bilateral uptake at the sacroiliac joints and the sternoclavicular joints. Discussion A detailed review of her history revealed significant axial symptoms that had persisted for decades. At first presentation, limitation of neck rotation and lateral flexion, along with mild lumbar forward flexion restriction, was noted. The sacroiliac joint X-ray was re-examined, and contrary to the initial report, evidence of sacroiliitis was identified. Diagnosis of cervical spondylosis was based on MRI without the necessary fat suppression to determine the presence of inflammation. Her elevated inflammatory markers, a reflection of active aortitis, were partially attributed to recurrent discontinuation of methotrexate because of repeated infections. During these periods off methotrexate, she required substantial steroid therapy to manage peripheral symptoms, which led to osteopenia. Additionally, the impact on her life was not insignificant; the longstanding and progressive nature of her disease had necessitated multiple career changes to accommodate her physical limitations. The PET scan results clarified that she had developed aortitis as a complication of longstanding axial spondyloarthropathy, which had previously gone unrecognised. She was commenced on high-dose prednisolone with a plan to commence biologic treatment. Key learning points Aortitis is a rare but serious complication of axial spondyloarthropathies, carrying a risk of cardiac complications and potentially life-threatening cardiovascular events. Early diagnosis and treatment is crucial, though inevitably more challenging in women with undiagnosed axial spondyloarthritis, which is now increasingly recognised. Axial spondyloarthritis may present atypically; rather than manifesting with predominant lower back pain, some patients, as in the case we present, may be troubled more by cervical and atypical peripheral symptoms. Sternoclavicular involvement, in particular, may represent an early and under-recognised feature of the disease. This case highlights the importance of requesting appropriate imaging (including the necessary protocols/sequences) and reviewing X-ray images rather than relying solely on radiology reports. Symptoms that do not respond to advanced pain management should be revisited and re-investigated if there is clinical suspicion of serious or systemic disease.

Vocal cord haemangiomas in adults are extremely rare. Management options include surgical resection, steroid therapy, ethanol injection, cryosurgery, radiation therapy and laser surgery. Transarterial embolisation has rarely been reported as a treatment for vocal cord haemangiomas in adults. This may be the first case in which an adult with a vocal cord haemangioma was successfully treated with transarterial embolisation. This patient, in the 40s, presented with complaints of a gradually progressive change in her voice over the past 6 months and was diagnosed with vocal cord haemangioma. She underwent transarterial embolisation, resulting in a significant reduction in the lesion size without any complications, which improved her voice. Superselective transarterial embolisation may be a safe and viable option for vocal cord haemangiomas in adults without major complications who prefer not to undergo surgical or laser excision for infantile-type haemangiomas that are resistant to conventional therapies.

Objective: There is no consensus regarding treatment modalities for idiopathic sudden sensorineural hearing loss. The aim of this study was to evaluate the clinical and audiological outcomes of three-dose versus five-dose intratympanic dexamethasone administration as an adjunct to systemic steroid therapy in patients with sudden sensorineural hearing loss. Methods: We retrospectively analyzed data from 30 patients diagnosed with sudden sensorineural hearing loss between 2022 and 2025 who received combined oral steroid therapy and intratympanic dexamethasone. Patients were divided into two groups: 20 received three intratympanic injections, and 10 received five injections. Pure tone audiometry (PTA) and speech discrimination scores (SDS) were measured before treatment and within 10 days after completion of therapy. Changes in audiometric outcomes were compared between the two groups. Results: The mean age was 47.3 years in the three-dose group and 48.6 years in the five-dose group. Tinnitus was present in all patients, and vertigo in 20%. PTA improvement was 16.1 ± 10.4 dB in the three-dose group and 14.7 ± 11.5 dB in the five-dose group (p &gt; 0.05). The mean SDS improvement was 10.7% ± 14.6 in the three-dose group and 6.9% ± 11.5 in the five-dose group (p &gt; 0.05). Overall PTA improvement rates were 28.23% and 23.28%, while SDS improvements were 30.94% and 43.81% for three- and five-dose groups, respectively. No statistically significant differences were observed between protocols. Conclusion: Both three- and five-dose intratympanic dexamethasone regimens, when added to systemic steroids, yielded significant hearing improvements in patients with sudden sensorineural hearing loss. However, increasing the number of injections did not confer additional benefit. These findings suggest that a lower-dose regimen maybe sufficient, reducing procedural risks and patient discomfort. Larger, randomized controlled trials are needed to establish standardized dosing protocols. Keywords: Sudden sensorineural hearing loss, intratympanic steroid, dexamethasone, pure tone audiometry, speech discrimination score