Low-dose Steroids Research Articles

OA02 Blood, Sweat and Steroids

Abstract Introduction This 79 year old gentleman was referred to the Rheumatology department with arthralgia, myalgia, erythema nodosum, an ESR elevated at 106 mm/h and IgA 6.45 g/L. His GP commenced him on 30 mg Prednisolone once a day for one week. His symptoms and signs improved significantly with treatment, prior to his first Rheumatology review. Case description He initially presented to his GP with pain and a skin rash in his groin which was thought to be fungal and treated as such. He then developed erythematous tender lumps on his right proximal forearm and left hand which were in keeping with erythema nodosum. He had a background history of ischaemic heart disease, hypertension, hyperlipidaemia, squamous cell carcinoma and an elevated BMI. Once the initial course of prednisolone stopped he experienced recurrence of the skin rash, in addition to night sweats, dizziness and weight loss. Inflammatory markers rose. CT scan of chest, abdomen and pelvis, done to exclude malignancy, was normal. PET CT while off steroid showed uptake in the descending aorta, not in keeping with large vessel vasculitis, and generalised bone marrow uptake consistent with an inflammatory process. Prednisolone was re-commenced. Bloods including HIV, Hepatitis, B12, CMV, EBV, Syphilis, ANA, ANCA and Cryoglobulins were normal. Skin biopsy showed non-specific inflammatory change. Bone marrow biopsy showed myeloid hyperplasia and haemophagocytosis. He required two further inpatient admissions due to shortness of breath, anaemia and skin rash, which coincided with steroid reduction. He responded to an increased dose of steroid and antibiotics. Genetic testing then identified a gene for UBA1, a pathogenic variant causing VEXAS syndrome. Clinical features were in keeping – skin lesions, sweats, elevated inflammatory markers, macrocytic anaemia and steroid responsivity. He was commenced on Tocilizumab and responded well. Bloods normalised and prednisolone was weaned. Neutropenia resulted in stopping Tocilizumab and later Anakinra and Sarilumab. Half dose once monthly Tocilizumab and low dose steroid are now successfully treating his disease. Discussion This gentleman had symptoms and signs in keeping with an auto-inflammatory condition. However, it took a number of specialties to investigate him due to blood results and imaging not naturally leading to a diagnosis. Initial differentials included sarcoid and polyarteritis nodosa. The chest and joints were not involved, serum ACE, calcium and CT Angio were normal so these were excluded. PET CT was done to ensure this was not an atypical large vessel vasculitis and the abnormal bone marrow uptake led us to discuss with haematology. At that time he had a pancytopenia with poor reticulocyte response and so haematology carried out a bone marrow biopsy. The bone marrow biopsy result showed a myeloid shift felt to be reactive and there was a degree of haemophagocytosis but he did not meet diagnostic criteria for haemophagocytic lymphohistiocytosis. Still’s disease was queried but he was not pyrexic, there was no arthritis, no hepatomegaly or splenomegaly and there was neutropenia rather than a neutrophilia. Skin biopsy, while not diagnostic, did query a vasculitic process. Therefore it was decided, despite persisting diagnostic uncertainty that he should be treated with Tocilizumb, which did allow steroid reduction. Eventually genetic testing was sent looking for periodic fevers and that is when the UBA1 gene was identified and the diagnosis of VEXAS syndrome made. Subcutaneous Tocilizumab did cause leucopenia and he suffered from recurrent infections. He had also been trialled on Anakinra and Sarilumab but both of these caused neutropenia. We have been able to switch to intravenous Tocilizumab in order to titrate the dose, and he is now on a small dose of 2 mg/kg 4 weekly which has allowed inflammation to settle without causing a drop in white cell count. Key learning points • There is little guidance on treatment of VEXAS syndrome. This gentleman has been difficult to treat due to drug side effects, and so this case raises the issue of few treatment options in VEXAS. It seems little is known about the disease and so we quickly come to a treatment conundrum when side effects prohibit the use of standard therapies. More research is required in to this condition and its management. • This case also raises the importance of working closely with other specialties. Where there is multi-system involvement, multi-disciplinary working is invaluable. It brings other perspectives and breadth of knowledge to the case, increasing the likelihood of making the correct diagnosis. • This case also reminds us of the value of genetic testing. Where it seems there is an inflammatory syndrome but results don’t seem to add up, we should put our patients forward for testing. • At the BSR case based conference I would like to hear other cases of treating VEXAS and what difficulties and wisdom my colleagues have to share. I would also like to hear if there are other treatment strategies others are aware of.

Severe cytopenia after chimeric antigen receptor-T cell driven by large granular lymphocytes and responsive to steroids.

Immune effector cell-associated hematotoxicity (ICAHT) is a common toxicity associated with an important morbidity after chimeric antigen receptor (CAR)-T-cell therapy. Multiple factors seem to be involved in the development of severe ICAHT, making its management difficult. Here, we report three cases of severe ICAHT after axicabtagene-ciloleucel (axi-cel) for diffuse large B-cell lymphoma showing an expansion of large granular lymphocyte in the bone marrow with a CD3/CD57-positive non-CAR-T immunophenotype. We show that it is possible to treat them with low-dose steroids, obtaining a striking resolution of cytopenias with no deleterious impact on the underlying malignancy.

Carotidynia - An Unexplored Entity in Otolaryngology Practice: Three Case Reports & Review of Literature.

Carotidynia is transient perivascular inflammation of the carotid artery. It is a rare condition of head and neck associated with atypical neck pain, often unilateral. Patients with carotidynia often presents with atypical symptoms that makes the diagnosis of this rare entity difficult. In this article, we report a case series of 3 patients that presents with variable symptoms along with different investigative modalities and treatment approaches. Due to rare entity, this condition is often misdiagnosed or necessitates several visits to various specialties before a diagnosis is reached. Thorough clinical examination along with radiology is must to reach to a diagnosis. Patient should be counselled regarding the benign nature of the disease that can be easily controlled by low dose steroids.

Custard apple seeds (Annona squamosa)-induced bilateral toxic keratopathy

Custard apple seeds are a natural remedy for head lice infestation in some parts of India. We report a case of a patient with accidental bilateral ocular exposure to custard apple seeds powder. The patient developed toxic bilateral keratopathy, which was treated with topical antibiotics, lubricants, and low-dose steroids. The patient responded well to the treatment. This report is intended to educate the patients regarding the potential toxic effects of custard apple seeds and increase the awareness of public on traditional harmful practices in this regard.

Managing RS3PE: Diagnostic approach, initial steroid response, and long-term management with DMARDs

Background. RS3PE, or Remitting Seronegative Symmetrical Synovitis with Pitting Edema, is a unique and relatively rare form of inflammatory arthritis that predominantly affects older adults. It is distinguished by its hallmark features: symmetrical swelling of the joints, significant pitting edema in the extremities, and a negative serological profile for common markers associated with rheumatoid arthritis, such as rheumatoid factor (RA factor) and anti-citrullinated protein antibodies (anti-CCP). This condition is often characterized by a remitting course, meaning that symptoms may improve or resolve over time, particularly with appropriate treatment. The condition usually affects older adults and can sometimes be associated with other underlying health issues, such as malignancies or infections. The “remitting” aspect of RS3PE refers to the fact that the symptoms often improve or resolve over time, particularly with appropriate treatment, which usually involves corticosteroids or other anti-inflammatory medications. Case report. This case study details the clinical presentation, diagnosis, and management of a 56-year-old female patient with known hypertension who presented with bilateral dorsal hand swelling, pain, and early morning stiffness over a 15-day period. The patient’s initial examination revealed bilateral pitting edema and active arthritis in the proximal interphalangeal joints, with significant warmth and tenderness. Laboratory tests showed elevated ESR and CRP, normocytic normochromic anemia, and negative rheumatoid factor and anti-CCP antibodies. Imaging studies, including X-ray, ultrasound, and mammogram, showed no erosive changes or malignancy, while a Pap smear and serum CA-125 levels were within normal limits. The clinical picture, combined with the exclusion of other conditions, supported the diagnosis of RS3PE. Treatment commenced with intravenous low-dose dexamethasone, which led to significant symptomatic improvement. This was followed by a transition to oral prednisolone, and the patient initially showed complete resolution of symptoms. However, recurrence of symptoms occurred upon discharge, prompting the introduction of steroid-sparing agents: methotrexate and hydroxychloroquine, alongside continued oral steroid treatment. Over a two-month follow-up period, the patient experienced no signs of relapse. This case fits the clinical and investigative criteria for RS3PE. The patient showed symptomatic improvement after treatment with low-dose steroids.

REMITTING SERONEGATIVE SYMMETRICAL SYNOVITIS WITH PITTING EDEMA (RS3PE) IN A 75-YEAR-OLD WOMAN: A CASE REPORT

Remitting seronegative symmetrical synovitis with pitting edema (RS3PE) is a rare inflammatory condition seen in elderly people. The disease is a syndrome characterized by sudden-onset swelling and pitting edema, holding hands and feet, especially in the distal extremities. RS3PE may be an alone disease or associated with malignancy, inflammatory rheumatic diseases or infections. The pathophysiology is not very clear. Its prevalence is not well known. Due to the presence in the elderly, especially polymyaljia rheumatica (PMR) and late-onset rheumatoid arthritis (LORA) is involved in the differential diagnosis. There are several criteria defined for diagnosis. Especially cases of RS3PE that are not associated with malignancy respond well to low-dose steroids. Although the relationship with cancer is known, it is not clear which patients should be screened for cancer. Our aim in this article is to present a patient with RS3PE and to remind physicians dealing with the geriatric population of the existence of RS3PE and to emphasize its importance.

Immune profiling-based targeting of pathogenic T cells with ustekinumab in ANCA-associated glomerulonephritis

Antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis is a life-threatening autoimmune disease that often results in kidney failure caused by crescentic glomerulonephritis (GN). To date, treatment of most patients with ANCA-GN relies on non-specific immunosuppressive agents, which may have serious adverse effects and be only partially effective. Here, using spatial and single-cell transcriptome analysis, we characterize inflammatory niches in kidney samples from 34 patients with ANCA-GN and identify proinflammatory, cytokine-producing CD4+ and CD8+ T cells as a pathogenic signature. We then utilize these transcriptomic profiles for digital pharmacology and identify ustekinumab, a monoclonal antibody targeting IL-12 and IL-23, as the strongest therapeutic drug to use. Moreover, four patients with relapsing ANCA-GN are treated with ustekinumab in combination with low-dose cyclophosphamide and steroids, with ustekinumab given subcutaneously (90 mg) at weeks 0, 4, 12, and 24. Patients are followed up for 26 weeks to find this treatment well-tolerated and inducing clinical responses, including improved kidney function and Birmingham Vasculitis Activity Score, in all ANCA-GN patients. Our findings thus suggest that targeting of pathogenic T cells in ANCA-GN patients with ustekinumab might represent a potential approach and warrants further investigation in clinical trials.

Sarcoidosis and Cancer: The Role of the Granulomatous Reaction as a Double-Edged Sword.

Background/Objectives: The relationship between sarcoidosis and the occurrence of neoplasia deserves to be investigated, but this relation has been observed in different and heterogeneous populations, leading to conflicting data. To clarify the causal relationship between these two diseases, different risk factors (e.g., smoking), concurrent comorbidities, corticosteroid therapy, and metastasis development-as an expression of cancer aggressiveness-were investigated. Methods: In a retrospective study on 287 sarcoidosis outpatients at the Pneumological Department of the Gemelli Foundation (Rome, Italy) between 2000 and 2024, the diagnosis of cancer was recorded in 36 subjects (12.5%). Results: The reciprocal timeline of the diseases showed three different scenarios: (1) cancer preceding sarcoidosis or sarcoid-like reactions (63.8%); (2) cancer arising after sarcoidosis diagnosis (8.3%); and (3) sarcoidosis accompanying the onset of malignancy (27.8%). Only two subjects with sarcoidosis and cancer showed metastasis, and one of them was affected by lymphoma. Conclusions: These data suggest that granulomatous inflammation due to sarcoidosis may assume an ambivalent role as a "double-edged sword", according to the M1/M2 macrophage polarization model: it represents a protective shield, preventing the formation of metastasis through the induction of immune surveillance against cancer while, on the other hand, it can be a risk factor for carcinogenesis due to the persistence of a chronic active inflammatory status. Low-dose steroid treatment was administered in only 31.6% of the cancer-sarcoidosis subjects for less than six months to control inflammation activity, with no promotive effect on carcinogenesis observed.

Predictors of survival for severe alcoholic hepatitis patients treated with low volume centrifugal plasma exchange and low dose steroid

Predictors of survival for severe alcoholic hepatitis patients treated with low volume centrifugal plasma exchange and low dose steroid

Isolated Omental Panniculitis

Isolated Omental Panniculitis is a very uncommon intra-abdominal disease, causing non-specific inflammatory symptoms, which only involves omentum without involving other organs like bowel, fat necrosis and pancreatitis. It is mostly seen in adults. The exact etiology of IAP is still obscure. Also, Modern diagnostic tools like CT & MRI were also ineffective to make the diagnosis. Different treatment modalities including conservative medical management with Anti-inflammatory drugs like low dose steroid along with NSAIDS and surgical excision of mass both were tried in past. In this article we are reporting an isolated omental panniculitis patient who presented to emergency with acute abdominal pain & was treated with laparoscopic omental segment resection.

DIPG-42. PHASE 1/2 CLINICAL TRIAL OF OMBIPEPIMUT-S IN JAPANESE PEDIATRIC PATIENTS WITH RECURRENT/REFRACTORY DIFFUSE INTRINSIC PONTINE GLIOMA, GLIOBLASTOMA, OR HIGH-GRADE GLIOMA

Abstract BACKGROUND Pediatric patients with recurred/relapsed diffuse intrinsic pontine glioma (DIPG), glioblastoma, or high-grade gliomas (HGGs) have a dismal prognosis. The present study investigated the impacts of 1) the absolute count of Wilms tumor 1 (WT1)-specific cytotoxic T cells (CTLs) at the initiation of WT-1 specific peptide vaccine (Ombipepimut-S) consisting of a killer peptide—adegramotide, a helper peptide—nelatimotide, and adjuvant MontanideTM ISA 51 VG and of 2) cumulative steroid dose on patients’ survival. We aimed to explore the optimal timing for initiating this cancer immunotherapy for them. METHODS The absolute counts of WT1-specific CTLs and lymphocytes were examined at the initiation of vaccination. This first-in-child phase 1/2 study investigated survival (overall survival [OS] and progression-free survival [PFS]), as well as cumulative steroid dose until day 28 after vaccination initiation and immune responses according to the Kaplan-Meier method and receiver-operating curve (ROC) analysis, respectively. RESULTS 18 patients (median age: 8.9 years [range: 2.8–18.7]), 11 (61.1%), 5 (27.8%), and 2 (11.1%) had DIPG, glioblastoma, and HGG, respectively) were intradermally injected with ombipepimut-S. WT1-specific immune responders accounted for 70.6% of patients. Median OS was significantly longer (P = 0.024) in WT1-specific immune responders (9.9 months [6.0−33.4]) than in nonresponders (4.9 months [2.2−16.5]). Under a low cumulative steroid dose (< 0.66 mg/kg body weight), ombipepimut-S significantly extended both OS (P=0.0015) and PFS (P=0.007). Immune responders tended to show the higher absolute counts of WT1-specific CTLs (P=0.091) and lymphocytes (P=0.087) at the initiation of vaccination. CONCLUSIONS Ombipepimut-S exhibited antitumor activity, and this cancer immunotherapy appeared to be preferably initiated when the patient’s immunity is relatively preserved immediately after the completion of standard therapy for primary disease or before the tumor recurs or relapses.

Centrifugal technique of plasma exchange and low-dose steroid to treat very severe alcoholic hepatitis patients: A retrospective analysis

Centrifugal technique of plasma exchange and low-dose steroid to treat very severe alcoholic hepatitis patients: A retrospective analysis

Safety and efficacy of low dose versus high dose corticosteroid for graft versus host disease (GVHD): A systematic review.

e24047 Background: Graft-versus-host disease (GVHD) is an immune-mediated complication of allogeneic hematopoietic stem cell transplantations. Current standard dose of corticosteroids used by the most hospitals for GVHD patients is 1-2 mg/kg/day. The prednisone dose used in the treatment of a GVHD has been a matter of debate. In this systematic review, we aimed to compare low dose prednisone to high/standard dose prednisone in terms of multiple prognostic outcomes of the patients undergoing allogenic hematopoietic stem-cell transplantations. Methods: We searched the databases PubMed, Cochrane library, and clinicaltrials.gov for all studies investigating the efficacy and safety of low dose and high dose steroid for GVHD. We set the search strategy incorporating the search terms, Graft-versus-host disease," "low dose cortico-steroid," and "high dose corticosteroid", and operated using the Boolean operators ‘AND’, and ‘OR’. Thus, retrieved articles were then exported on an Excel sheet, and duplicates were removed. After selecting the study based on inclusion criteria, data on study characteristics and biomarker description was extracted on a pre-determined data extraction table on the Microsoft Excel. Results: The search revealed 547 studies and 3 studies that met the eligibility criteria of this review have been included.During relapse, chronic GVHD, mortality, and malignancy outcomes were generally similar between doses, low-dose prednisone showed benefits for leukemia-free, relapse-free, and overall survival. Overall, the randomized trial by Chang et al. found lower risks of HZV and pulmonary infections with low-dose prednisone compared to standard-dose. (Table 1) Other infections were similar between groups. Conclusions: Low-dose prednisone showed similar efficacy to standard-dose for most GVHD outcomes (relapse risk, mortality), with potential benefits on survival, reduced HZV/pulmonary infections, and reduced adverse effect of higher cumulative dose of corticosteroids. [Table: see text]

#2475 Glomerulonephritis associated with anti -TNF-α therapy

Abstract Background and Aims Biologic agents like tumor necrosis factor α inhibitors/ anti-TNF-α/, IL6 and CD80/26 are widely used in many rheumatologic diseases but these may also elicit effects on renal function. These drugs, as well as rheumatic diseases themselves, can cause autoimmune renal disorders. Though effective on the main inflammatory process the incidence of kidney injury associated with their administration is increasing. Various forms of glomerulopathies have been described in connection with biological therapy - proliferative lupus nephritis, oligo immune necrotizing crescentic glomerulonephritis, membranous glomerulonephritis, IgA nephropathy and others. Method We report the clinical and pathologic findings in two patients with Rheumatoid arthritis and Ankylosing spondylitis on biologic therapy with an anti-TNF-α agent. During the treatment they presented with nephritic syndrome requiring kidney histology. Results Case 1: The first patient is a 52-year-old man diagnosed with ankylosing spondylitis, HLA-B27 positive, 15 years before admission. Four years before admission treatment with Golimumab /anti-TNF-α/ has been initiated. During the follow-up period urine tests revealed proteinuria and hematuria, which required more detailed tests. The patient presented with clinical features of nephritic syndrome, marked lower extremity edema, hematuria, nephrotic range proteinuria, impaired hypertension control. Kidney biopsy revealed membranoproliferative glomerulonephritis. It was assumed that kidney injury was most likely associated with Golimumab therapy. Its administration was discontinued, and therapy was started with pulses of Methylprednisolone 500 mg i.v. in three consecutive days, Cyclophosphamide 500 mg i.v., repeated the second and third month, followed by low steroid doses 0,5 mg/kg with gradual tapering. Mycophenolate Mofetil 1500 mg was started from the third month. General condition and laboratory results improved considerably. The occurrence of stiffness and pain in the sacroiliac joints several months after stopping biologic treatment necessitated the inclusion of Sulfasalazine to the therapy. Initiation of therapy with another class of biologic agent, Anti-IL-17 monoclonal antibody, was discussed with the follow-up rheumatologists. Case 2: The second patient is a 66-year-old woman, with a history of hypertension and essential thrombocythemia, who was diagnosed with seropositive rheumatoid arthritis 3 years before admission, treated with Adalimumab /anti-TNF-α/. Follow-up examinations revealed low-grade proteinuria, hematuria and impaired renal function. Kidney biopsy revealed IgA nephropathy. Following the discontinuation of Adalimumab, the patient was treated with pulses of Methylprednisolone 500 mg i.v. in three consecutive days, Cyclophosphamide 500 mg i.v., repeated the second and third month, followed by low steroid doses 0,5 mg/kg with gradual tapering. Kidney function significantly improved at the third month of treatment and proteinuria was gradually reduced. Initiation of Rituximab /antiCD20/ treatment was discussed with rheumatologists. Conclusion Treatment with biological drugs can cause immune-mediated glomerular disorder/IGD/. These events are rare, and the causative effects of a specific drug is hard to establish. When a patient is suspected of having an IGD, kidney biopsy should be performed for the exact diagnosis. The drug should be discontinued, and the treatment for the new-onset renal disorder should be promptly started. A rechallenge test of the same or a similar drug should be avoided, whereas switching to a different therapy is a reasonable option.

#2095 Genetic kidney disease in pregnancy – experience of tertiary referral hospital

Abstract Background and Aims Genetic kidney diseases (GKD) are rarely seen in pregnancy and some diagnosis will only be made during gestation, due to a high proportion of chronic kidney diseases (CKD) of unknown etiology and recent advances in genetic testing. Genetic counseling and, when possible, preimplantation genetic testing (PGT) should be offered pre-pregnancy to this specific CKD population. Method Retrospective analysis of maternal, obstetric and perinatal outcomes of pregnant women with GKD surveilled at Hospital Santa Maria, at the nephro-obstetric clinic from 2011 to 2023. Results We evaluated 34 pregnancies in 28 women, mean maternal age 30±6 years-old [21-37], 89% Caucasian. Molecular genetic testing was performed pre-pregnancy, during and post-pregnancy in 12/19, 2/19 and 5/19 patients, respectively. At baseline, mean serum creatinine (SCr) and proteinuria (Pr) was 1.0±0.9 mg/dL and 222±516 mg/g, with CKD stage 1/2/3/4/5 in 27/2/2/1/2 gestations and chronic hypertension (HTN) in 11/34 pregnancies. Only 1/28 patients performed PGT, while 1/28 patient declined it. De novo or increased Pr occurred in 10/34 gestations and de novo or worsening HTN in 5/34 gestations. Deterioration of renal function occurred in one patient (CKD3) without recovery and one patient (CKD5) induced dialysis 4 months post-pregnancy. Nephrotic Pr developed in 4/34 gestations. One patient (FSGS APOL1) was treated with tacrolimus and low dose steroids. The patient with TSC re-started sirolimus on week 22 to treat severe fetal ventricular rhabdomyoma. Miscarriage occurred in 2 pregnancies. The C section rate was 21,8%. There were 7 preterm deliveries of which 3 before 32 weeks of pregnancy, mainly due to severe preeclampsia. Mean gestational age at delivery was 37,6 ± 4 weeks (26-41), mean birth weight 2756 ± 831 g (560-3835). Eight newborns were admitted to neonatal ICU due prematurity, fetal TSC, and feeding difficulties. Pulmonary hemorrhage and sepsis caused the death of 2 newborns. We found 1 maternal death, due to rupture of a cerebral aneurism 1 month postpartum (PKD patient). Conclusion Our cohort was very heterogenous regarding CKD etiologies, but generally with good kidney function, which allowed overall good obstetric outcomes. Still, maternal, obstetric and fetal complications, namely HTN, Pr, PE and prematurity had an increased incidence compared to pregnancy in healthy patients. This particular population should be approached by an experienced multidisciplinary team in CKD pregnancy care to optimize outcome.

Sirolimus is effective and safe in childhood relapsed-refractory autoimmune cytopenias: A multicentre study.

Autoimmune cytopenias are a heterogeneous group of disorders characterized by immune-mediated destruction of haematopoietic cell lines. Effective and well-tolerated treatment options for relapsed-refractory immune cytopenias are limited. In this study, the aim was to evaluate the efficacy and safety of sirolimus in this disease group within the paediatric age group. The study enrolled patients in the paediatric age group who used sirolimus with a diagnosis of immune cytopenia between December 2010 and December 2020, followed at six centres in Turkey. Of the 17 patients, five (29.4%) were treated for autoimmune haemolytic anaemia (AIHA), six (35.2%) for immune thrombocytopenic purpura (ITP) and six (35.2%) for Evans syndrome (ES). The mean response time was 2.7 months (range, 0-9 months). Complete response (CR) and partial response (PR) were obtained in 13 of 17 patients (76.4%) and nonresponse (NR) in four patients (23.5%). Among the 13 patients who achieved CR, three of them were NR in the follow-up and two of them had remission with low-dose steroid and sirolimus. Thus, overall response rate (ORR) was achieved in 12 of 17 patients (70.5%). In conclusion, sirolimus may be an effective and safe option in paediatric patients with relapsed-refractory immune cytopenia.

E030 Osteonecrosis of the spine in antiphospholipid antibody syndrome

Abstract Background/Aims Osteonecrosis (ON) is a well-known complication of several rheumatic diseases, affecting the hip, knee, shoulder, or ankle. The involvement of spine and other sites with ON has been reported with catastrophic APS, although the nature of this association is not completely clear. In antiphospholipid syndrome, thrombosis of blood vessels is considered a possible mechanism, along with other risk factors, such as active disease, thrombocytopenia, glucocorticoid use and associated autoimmune diseases, making the pathophysiology multifactorial. We report a case of a 66-year-old male with APS who developed bone infarcts/osteonecrosis after withdrawal of anticoagulation for APS, which was necessitated by a bleeding complication and introduction of steroids for immune thrombocytopenia. Methods A 66-year-old male came to us with history of unprovoked deep vein thrombosis (DVT) for which he was on antiplatelet and anticoagulation for over 20 years. He was diagnosed to have high titre anti cardiolipin antibody (ACA)(186 U/ml) without other autoimmune conditions. He had an episode of bladder hematoma in August 2022, needing his anticoagulation to be suspended temporarily. He was also found to have profound thrombocytopenia with raised inflammatory markers. Considering it to be immune mediated he was started on high dose oral corticosteroids and was later maintained with azathioprine while the oral prednisolone dose was gradually brought down to 10 mg daily in about 3 months’ time. His thrombocytopenia improved progressively however he developed an unprovoked DVT in October 2022 and was restarted on anticoagulation. He continued to have low dose steroids for about 6 months. Meanwhile, he started having a new onset of low back pain in December 2022. An MRI of the whole spine showed multilevel osteonecrosis with haemorrhagic cavities in a few vertebral bodies. Results This case highlights three interesting points. The first is the atypical presentation of the osteonecrosis involving vertebral bodies. Osteonecrotic lesions in the spine of a patient with catastrophic primary antiphospholipid syndrome has been reported. Our case signifies that although vertebral bodies are an uncommon site for avascular necrosis in patients with APS, it is not rare. Secondly, the pathogenesis of ON is complex. Whether ACA are directly responsible for this condition or whether they are the bystanders remains controversial. ACA have shown to be an independent risk factor for developing ON in many studies. Thirdly, steroids are known to be an important risk factor in patients presenting with osteonecrosis. The vascular compromise could be attributed to a steroid induced hypercoagulative state, abnormal lipid metabolism, or fat embolism. Conclusion ON in APS is unusual and its underlying pathophysiology remains multifactorial. However, when complicated by the high ACA levels, active disease, difficult anticoagulation (bleeding tendencies), thrombocytopenia or prolonged use of steroids, it can lead to hypercoagulable state causing micro thrombosis and avascularisation. Disclosure M. Shah: None. K. Putchakayala: None.

Efficacy of lignocaine nebulization in patients with COVID-19 respiratory infection: An exploratory randomized double-blinded controlled trial.

Coronavirus disease (COVID-19)-related pneumonia is proposed to be an inflammatory process. The treatment currently includes supportive therapy and low-dose steroids. Anti-inflammatory drugs have been proposed to prevent cytokine storms and improve oxygenation in such cases. The study aimed to assess the efficacy of nebulized lignocaine in COVID-19 patients with pneumonia. This was an exploratory randomized double-blinded control trial conducted in COVID-19 patients with respiratory failure requiring oxygen therapy either by face mask or non-invasive mechanical ventilation. Patients included were of the age of more than 18 years of either gender. The patients were randomized to receive either lignocaine or distilled water nebulization. The outcomes assessed were PaO2/FiO2 ratio, hemodynamics, respiratory parameters, and sequential organ failure score (SOFA). The two groups were comparable concerning demographic variables. The PaO2/FiO2 were significantly higher in the lignocaine group from day 2 onward. The SPO2 was significantly higher on day 3 in the lignocaine group and thereafter there was no significant difference. Other hemodynamic, respiratory parameters, and SOFA scores showed no difference in both the groups. Lignocaine nebulization improved oxygenation in COVID-19 patients and can be used as adjunctive therapy along with other supportive medications.

Decreasing the steroid rapidly may help to improve the clinical outcomes of patients with intestinal steroid-refractory acute graft-versus-host disease receiving basiliximab treatment.

Intestinal steroid refractory acute graft-versus-host disease (SR-aGVHD) is the major cause of mortality in allogeneic hematopoietic stem cell transplantation (allo-HSCT). This retrospective cohort study aimed to identify the relationship between different steroid decreasing velocity and therapeutic response in patients with intestinal SR-aGVHD receiving basiliximab treatment, and also aimed to propose a reasonable steroid decreasing regimen for these patients. The median time for steroid dose decreasing to the 50% of initial dose and decreasing to the low-dose steroid for patients achieving ORR was 5 days and 12 days, respectively, which was both shorter than patients without achieving ORR. The ORR, NRM and survival in rapid and medium steroid decreasing group were all better than slow group. The cumulative incidence of ORR at any time was 90.4%, 78.1% and 62.3%, respectively, in rapid, medium, and slow group. The cumulative incidence of NRM at 1 year after basiliximab treatment was 18.7% (95% CI 11.3%-26.1%), 22.8% (95% CI 14.2%-31.4%) and 32.8% (95% CI 24.1%-41.5%), respectively, in rapid, medium, and slow group. The probability of OS at 1 year after basiliximab treatment was 76.9% (95% CI 68.9%-84.9%), 72.7% (95% CI 63.7%-81.7%), and 62.3% (95% CI 53.5%-71.1%), respectively, in rapid, medium, and slow group. Hence, it was helpful to decrease steroid to the 50% of initial dose ≤ 5 days and to the low-dose steroid ≤ 12 days after basiliximab treatment for intestinal SR-aGVHD patients, which may also be the reasonable steroid decrease protocol for these patients.

Clinical spectrum in microbiologically proven Demodex blepharokeratoconjunctivitis: An observational study.

To study the demographic, clinical, and microbiological profile of Demodex-related blepharokeratoconjunctivitis (BKC) at a tertiary eye care hospital. This retrospective observational study was conducted from January 2016 to September 2022. It included 83 patients with microbiologically proven Demodex BKC who presented to the cornea department of our tertiary care eye center. The clinical, microbiological, and demographic data of the 83 cases were analyzed. Of the 83 cases, 57 (68.67%) were younger than 40 years, and 25 (30.12%) were below 20. Most patients presented with a good visual acuity of 20/40 or better (93 eyes; 84.55%). The disease was unilateral in 55 patients and bilateral in 28. Cylindrical dandruff was the predominant presentation noted in 61 eyes (54.95%), followed by corneal scarring in 47 eyes (42.34%) and corneal vascularization in 40 eyes (36.04%). On light microscopy, 87.95% of the positive samples were identified as Demodex folliculorum , 7.23% as Demodex brevis , and 6.02% remained unidentified. Tea tree oil and lid scrubs eradicated the disease in most patients clinically (75/83, 90.36%). The spectrum of BKC includes both lid signs and corneal involvement. It can be a cause of recurrent BKC and detection of the mite by microscopic evaluation of the lashes can confirm the diagnosis. In most cases, the tea tree oil can effectively manage this condition. However, low doses of topical steroids are needed to control the inflammation in patients with corneal involvement.