Low-dose Prednisolone Research Articles

Structured and prompt treatment of early arthritis in clinical practice leverages window of opportunity and leads to excellent clinical outcomes: an innovativeretrospective cohort study.

With this work, we evaluated the impact of the Lisbon Early ARthritis cliNic (LEARN) on untreated inflammatory arthritis clinical and patient-reported outcomes. A retrospective cohort study enrolled patients in LEARN since its inception. Patients were followed for 12months and treated to achieve disease remission. Clinical, structural, and quality of life outcomes were assessed. The early arthritis module of the Portuguese Rheumatic Diseases Registry (Reuma.pt) is described. We assessed 292 patients between 2015 and 2022. Mean symptom duration and DAS-28-4V-ESR at baseline were 6.2 ± 3.5months and 5.6 ± 1.3, respectively. Rheumatoid arthritis (56.4%; 40.1% seropositive) and psoriatic arthritis (12.4%) were the most common diagnoses. Most patients were treated with methotrexate (75.3%) combined with low-dose oral prednisolone (88.1%). At 12months, a mean ΔDAS28-4V-ESR improvement of 2.3 ± 0.4 was registered, with 29.5% and 48.9% of patients achieving remission (DAS28-4V-ESR < 2.6) or low disease activity (DAS28-4V-ESR < 3.2), respectively. Among RA patients only, these figures were 20.6% and 46.6%, respectively. A clinically meaningful functional improvement was observed in 72.1% of the patients. Structural progression was limited, affecting only 16.1% of the patients. Fatigue, anxiety, depression, and quality of life also improved substantially, translated by improvements in FACIT, HADS, EQ5D, and SF-36 scores. A structured, dedicated approach to patients with early arthritis resulted in good clinical, structural, and functional outcomes. Furthermore, our findings suggest the window of opportunity for early intervention may have implications for mental health and global well-being. Key Points • Patient assessment is facilitated by reliable electronic clinical records, such as the early arthritis module of the Rheumatic Diseases Portuguese Register (Reuma.pt) which we describe here for the first time. • Inflammatory arthritis was confirmed in the majority of patients observed, but the time to first appointment was above the recommended. • Prompt start of conventional therapy allowed significant disease activity improvement and remission to be achieved in about one-third of the patients. • Key patient-reported outcomes elucidate disease impact and confirm the benefit of early treatment initiation, suggesting a window of opportunity also for mental health and global well-being.

Suppression of Aseptic Inflammation Reduces the Severity of Remodeling of the Pulmonary Artery Branches and Improves Progressing of Experimental Chronic Thromboembolic Pulmonary Hypertension

Chronic thromboembolic pulmonary hypertension (CTEPH) is a complication of pulmonary embolism, characterized by increased pressure in the pulmonary artery and impaired lysis of thromboemboli. Previously, the presence of aseptic inflammation in CTEPH was identified in the wall of the pulmonary artery branches and perivascularly. However, the role of this inflammation in the CTEPH formation is unknown. The aim of the work was to study the effect of aseptic inflammation on the CTEPH formation and progression. The experiments were performed on 54 male rats. The CTEPH model was reproduced by repeated intravenous administration of partially biodegradable microspheres (MS). Immediately after the last administration of MS, all animals were divided into groups: control CTEPH (c.CTEPH) – saline solution was administered intramuscularly (i/m) for 6 weeks; low dose of prednisolone (LD) – prednisolone was administered i/m at a dose of 1.5 mg/kg; high dose (HD) – prednisolone was administered i/m at a dose of 6 mg/kg; healthy animals. After 6 weeks, the following was performed: treadmill test, TTE, cardiac catheterization with manometry, and histological examination of the lungs. In a separate series of experiments, the severity of inflammatory infiltration of the vascular wall and perivascular zone was assessed by immunohistochemical studies (IHC). In the LD group, there was the decreasing of hypertrophy index (HI) and the percentage of collagen fibers in the vascular wall compared to c.CTEPH. There was a significantly greater reduction in HI compared to HD. In the HD group, there was positive effect on the percentage of collagen fibers in the vascular wall, this parameter did not significantly differ from healthy animals. According to IHC data, prednisolone in low dose effectively suppressed inflammatory infiltration of the vascular wall and perivascular space. The results of the study revealed the ability of prednisolone, by suppressing aseptic inflammation, to reduce the severity of remodeling of the pulmonary artery branches.

Design and Rationale of MYOFLAME-19 RCT: MYOcardial protection to reduce inFLAMmatory heart disease due to COVID-19 Infection using CMR Endpoints

Cardiac symptoms due to postacute inflammatory cardiac involvement affect a broad segment of previously well people with only mild acute COVID-19 illness and without overt structural heart disease. Cardiac magnetic resonance (CMR) imaging can identify the underlying subclinical disease process, which is associated with chronic cardiac symptoms. Specific therapy directed at reducing postacute cardiac inflammatory involvement prior to development of myocardial injury and impairment is missing. Prospective multicentre randomised placebo-controlled study of myocardial protection therapy (combined immunosuppressive/antiremodelling) of low-dose prednisolone and losartan. Consecutive symptomatic individuals with a prior COVID-19 infection, no preexisting significant comorbidities or structural heart disease, undergo standardised assessments with questionnaires, CMR imaging and cardiopulmonary exercise testing (CPET). Eligible participants fulfilling the criteria of subclinical Post-COVID inflammatory involvement on baseline CMR examination are randomised to treatment with either verum or placebo for a total of 16 weeks (W16). Participants and investigators remain blinded to the group allocation throughout the study duration. The primary efficacy endpoint is the absolute change of left ventricular ejection fraction (LVEF) to baseline at W16, measured by CMR, between the verum treatment and placebo group by absolute difference, using unpaired t-test confirmatively at the 5% significance level. Secondary endpoints include assessment of changes of symptoms, CMR parameters, and CPET after W16, and frequency of major adverse cardiac events after 1 year. Safety data will be analysed for frequency, severity and types of adverse events (AEs) for all treatment groups. The proportion of AEs related to the contrast agent gadobutrol will also be analysed. A calculated sample size is a total of 280 participants (accounting for 8% drop-out), randomised in 1:1 fashion to 140 in the verum and 140 placebo group. Myoflame-19 study will examine the efficacy of a myocardial protection therapy in symptomatic participants with post-COVID inflammatory cardiac involvement determined by CMR. The aim of the intervention is to reduce the symptoms and inflammatory myocardial injury, to improve exercise tolerance and preclude the development of cardiac impairment. NCT05619653.

The Pathomechanism and Current Treatments for Chronic Interstitial Cystitis and Bladder Pain Syndrome.

Interstitial cystitis/bladder pain syndrome (IC/BPS) is a chronic and debilitating condition characterized by symptoms such as bladder pain, frequent urination, and nocturia. Pain is typically perceived in the lower abdomen, pelvic floor, or urethra, causing significant discomfort and impacting quality of life. Due to the similarity of its symptoms with those of overactive bladder and acute bacterial cystitis, patients often face misdiagnosis and delayed appropriate treatment. Hunner's (HIC) and non-Hunner's IC (NHIC), each with distinct clinical presentations, urothelial dysfunction, chronic inflammation, and central sensitization and thus multimodal symptomatic treatment approaches, may be the most common pathogeneses of IC/BPS. Treatment of IC/BPS should involve identifying the different clinical phenotypes and underlying pathophysiology causing clinical symptoms and developing strategies tailored to the patient's needs. This review discusses the roles of urine biomarkers, bladder inflammation, and glycosaminoglycans in the pathogenesis of IC/BPS. Various bladder treatment modalities are explored, including glycosaminoglycan replenishment, botulinum toxin A injection, platelet-rich plasma injection, low-energy shock waves, immunosuppression, and low-dose oral prednisolone. Pelvic floor muscle physiotherapy and bladder therapy combined with psychiatric consultation can help alleviate psychological stress and enhance the quality of life of patients with IC/BPS. Elucidating the pathological mechanisms and exploring diverse treatment options would help advance the care of individuals suffering from this challenging bladder condition.

Clinical characteristics and risk factors for lupus flares in sub-Saharan Africa-retrospective cross-sectional study.

Systemic lupus erythematosus (SLE) is an autoimmune disease with a variable course with unpredictable flares. Identifying predictors of these flares is essential for monitoring and timely hospital care. To characterize the prevalence of flares within the first five years of SLE diagnosis and determine the clinical and immunological characteristics associated with flare development among patients attending the Rheumatology Clinic at Tikur Anbesa Specialized Hospital (TASH) and Lancet General Hospital. A multicenter, cross-sectional study was conducted from May 2023 to November 2023 at TASH and Lancet General Hospital. The data was collected from electronic medical records and analyzed using SPSS version 26. Logistic regressions were used to determine factors associated with lupus flare. Most patients with SLE were female (95.4%). The most common clinical presentations were musculoskeletal (71.8%), cutaneous (55%), and constitutional (22%). Almost half (44.3%) of the patients had comorbidity illness. Positive ANA test was found in 96.5% of the patients, whereas only 55% had positive anti-dsDNA test. The prevalence of SLE flare in the first five years of SLE diagnosis was 38.9%, and most flares occurred within the first year of diagnosis. Patients with the following characteristics were more likely to have flare-ups: younger age at diagnosis (less than 25years old), initial presentation with vasculitis, renal flare, and being on low-dose prednisolone. The most common clinical presentations were musculoskeletal, dermatologic, and constitutional manifestations. Age < 25years at diagnosis, initial clinical presentation with renal manifestation, and being on low-dose prednisolone were predictors of SLE flare. Key Points • This study found a significant gender disparity, with 95% female. • Nearly 39% of patients experienced an SLE flare within the first five years of diagnosis. • Over three-quarters (77%) of flares occurred within the first year of diagnosis. • Age less than 25years, initial presentation with vasculitis, renal involvement, and being on low-dose prednisolone were identified as predictors of flares.

Does high hepatic bioavailability enhance the effect of oral compared to subcutaneous glucocorticoids?

Glucocorticoids (GC) are important in the treatment of autoinflammatory disorders. Oral prednisolone ≤5 mg/day can be effective, but such doses are at or even below physiological daily endogenous GC production. We hypothesised that their immunosuppressive effect might be explained by high hepatic bioavailability of oral GC, exposing the liver to supraphysiological GC via the portal circulation. We tested this by comparing the effect of oral versus subcutaneous low-dose prednisolone, on erythrocyte sedimentation rate (ESR). Patients with rheumatoid arthritis or psoriatic arthritis, elevated ESR (≥30 mm/h) and no current or recent GC therapy were eligible. In a pilot study (n=5), 5 mg/day oral prednisolone decreased ESR significantly, suggesting a sample size of 10 patients for a randomised, non-blinded crossover trial. Patients received 5 mg/day prednisolone for 2 periods of 4 days: one treatment period orally and one subcutaneously with a 10-day washout period between treatments. ESR was measured before (day 1 and 15) and after (day 5 and 19) each treatment course. 10 patients were included. ESR decreased after both oral and subcutaneous prednisolone, by -5.6 (20.9) and -5.8 (3.0) mm/h, respectively (p=0.98). The treatment order had no effect on the outcome. . Short-term oral low-dose GC therapy is not more effective than parental GC in decreasing ESR, arguing against therapeutic high hepatic bioavailability effects. More likely, systemic concentration peaks following administration explain why oral physiological steroid doses are clinically effective.

Diagnostic Value of Short Course Low-dose Prednisolone in Patients with Clinically Suspected Seronegative Inflammatory Arthritis - A Retrospective Study.

We aim to establish the utility of a trial of low-dose systemic glucocorticoid therapy in the assessment of new clinically suspected inflammatory arthritis patients. We retrospectively identified patients from a private rheumatology practice in Melbourne, Australia between January 1st, 2019, and December 31st, 2021, who presented with clinically suspected inflammatory arthritis and subsequently underwent a trial of low-dose prednisolone (15 mg daily weaned over three weeks in 5 mg increments). We excluded patients with known autoimmune/ inflammatory disorders or concurrent immunosuppression at presentation. We collected basic participant demographic details and clinical details of their presentation, glucocorticoid response, investigations, and treatment. We recruited 177 participants with a median age of 52, and 69.5% were female gender. The median symptom time to presentation was 12 months. Hands were the most affected joint in 63.3% and 85% had bilateral disease. Among the participants, 29.4% had synovitis on clinical review and 75.7% had imaging performed as part of the initial assessment. At presentation, the median CRP was 11 and the median ESR was 16. 79.7% of the cohort experienced significant improvement in their arthritis symptoms from low-dose glucocorticoids and 83.6% of the cohort required long-term immunosuppression for an underlying inflammatory condition. Of those who responded to glucocorticoids, 92.1% were diagnosed with an inflammatory condition. Rheumatoid arthritis was the most common overall diagnosis in 28%. An initial trial of low-dose glucocorticoids in undifferentiated arthritis patients is useful in predicting the diagnosis of inflammatory arthritis. It is also a predictor of further long-term steroid-sparing therapy.

Anti-sulfatide antibody associated GBS with headache and abdominal pain: a case report

BackgroundGuillain‒Barre syndrome (GBS) is an acute inflammatory peripheral neuropathy caused by autoimmunity. Gangliosides and sulfatides are important components of peripheral nerves. Anti-sulfatide antibody-mediated complement is associated with acute sensorimotor peripheral neuropathy in GBS, which is characterized by pain and paresthesias.Case presentationThe child was a 7-year-old girl with headache and abdominal pain, followed by limb numbness and pain. Cranial imaging showed ventricular dilatation, peripheral nerve function conduction examination showed polyradiculopathy, and cerebrospinal fluid tests showed normal cell counts but elevated protein levels, all of which led to the diagnosis of GBS. After treatment with intravenous immunoglobulin (400 mg/kg × 5 days), the symptoms did not improve, and muscle strength progressively worsened, accompanied by paroxysmal complexion flushing, heart rate fluctuation, hyperhidrosis, and a progressive increase in cerebrospinal fluid protein (up to 3780.1 mg/L). On the basis of these findings combined with serum anti-sulfatide IgM positivity, anti-sulfatide antibody-related GBS was considered, and treatment with low-dose prednisolone (1 mg/kg/d) led to symptom improvement.ConclusionsAnti-sulfatide antibody-associated GBS is associated with small fiber peripheral neuropathy. The main manifestations are pain, paresthesias and autonomic dysfunction. In addition to the dysfunction of spinal nerve root absorption caused by increased cerebrospinal fluid protein, autonomic dysfunction may be involved in pain. When the therapeutic effect of immunoglobulin is not satisfactory, a low dose and short course of corticosteroids can be considered, and the prognosis is good.

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.

Nonspecific inflammatory pseudotumor of the maxillary and temporal fossa: a study of seven cases

ObjectiveInflammatory pseudotumor (IPT) is a rare, locally aggressive, benign neoplasm of unknown etiology. Because of its aggressive clinical behavior and locally destructive or infiltrative features, it may be mistaken for a malignant tumor. Approximately 5–44% of extrapulmonary IPT occur in the head and neck, primarily affecting the orbit. Study DesignBetween 2008 and 2021, our hospital received referrals for seven patients (three men and four women, aged 42–73 years) with pain, swelling, mass, and trismus. Computed tomography, magnetic resonance imaging, and biopsy were performed on all patients to diagnose IPT. ResultsOf the seven patients, four received low-dose prednisolone (PSL), one underwent surgery, and two were left untreated. The IPT disappeared in one of the two untreated cases, whereas it improved and later deteriorated in the other. The surgical patient had no recurrence. Low-dose PSL was effective in two patients; however, high-dose PSL and immunosuppressants were required in the remaining two cases owing to infiltration into each orbit or brain region. ConclusionsLow-dose PSL treatment was applicable in IPT cases affecting the maxillary to temporal fossa region, wherein symptoms did not improve without treatment. However, when low-dose prednisolone was ineffective, high-dose prednisolone and immunosuppressants were required.

Remitting seronegative symmetrical synovitis with pitting edema syndrome: case report of an atypical presentation of a rare syndrome and literature review

BackgroundRemitting seronegative symmetrical synovitis with pitting edema (RS3PE) is a rare syndrome. The following case shows an atypical course of the disease with successful treatment. In addition, the accompanied review highlights current findings in the pathogenesis and treatment. Clinicians should be aware of the differential diagnosis of RS3PE syndrome.Case presentationA 67-year-old female patient with recurrent, asymmetric, and painful swelling of both hands with pitting edema, predominantly affecting the dorsal right hand, presented at our in-patient clinic. Over the years of her disease, first diagnosed as rheumatoid arthritis and then psoriatic arthritis, prednisolone treatment had the most favorable effects over various disease-modifying antirheumatic drugs.Subsequent diagnostic evaluation confirmed RS3PE syndrome, a rare inflammatory disorder primarily affecting the elderly population. Manifesting as symmetrical joint inflammation of small joints with pitting edema, RS3PE syndrome typically onsets suddenly and may be accompanied by systemic symptoms like fever, fatigue, and weight loss. Although the precise etiology remains enigmatic, both the innate and the adaptive immune system seem to play a pathogenic role. Treatment is conventionally based on prednisolone, which effectively mitigates symptoms.Ultimately, RS3PE was diagnosed in the context of psoriatic arthritis without dermatological or nail involvement. Given the unusual presentation marked by female gender, asymmetry, and prolonged and extensive disease with various prior treatments and in the context of psoriatic arthritis, a tumor necrosis factor alpha inhibitor was initiated in addition to low-dose prednisolone resulting in clinical remission for the first time.ConclusionsIn conclusion, the aforementioned atypical manifestation highlights the significance of including RS3PE syndrome as a potential differential diagnosis, particularly in instances where specific diagnostic criteria for rheumatoid arthritis, polymyalgia rheumatica, or psoriatic arthritis are absent. RS3PE responds well to the administration of prednisolone. In refractory cases, a therapeutic trial with tumor necrosis factor alpha inhibitors can be conducted.

#410 Novel renal pathology and treatment of TIN with monoclonal plasma cells invasion in a patient with Sjögren's syndrome and IgA monoclonal gammopathy

Abstract Background and Aims Patients with Sjögren's syndrome (SS) may occasionally present with monoclonal gammopathy of undetermined significance (MGUS), of the rare IgA type. Additionally, they may present with tubulointerstitial nephritis (TIN) infiltrated by polyclonal plasmacytes and lymphocytes. Previous reports indicate the development of IgA-type MGUS and TIN with monoclonal plasma cells in some SS patients, which are considered distinct from monoclonal gammopathy of renal significance (MGRS) because there is no evidence that monoclonal gammopathy affects nephropathy. Although treatment with prednisolone (PSL) has shown beneficial effects, the influence of monoclonal plasma cells on nephropathy remains unclear and a standardized treatment approach is lacking. Here we present a new renal pathological insight through a case of such a patient as described above, who underwent renal biopsy before and after PSL treatment. The aim is to propose a treatment strategy based on the findings of the second renal biopsy. Method and Results (Case Presentation) A 75-year-old Japanese man with primary complaint of dry mouth was diagnosed with SS by lip biopsy and anti-Ro/SSA and La/SSB antibody tests. Monoclonal proteins were found in both blood and urine. After immunoelectrophoresis, bone marrow puncture, and 18F-FDG PET/CT, we identified it as IgA kappa-type MGUS. At the same time, he developed renal dysfunction and was admitted to our hospital (week 1, Fig. 1) for a renal biopsy. The biopsy revealed severe and diffuse TIN infiltrated by lymphocytes with abundant interstitial plasma cell invasion (Fig. 2a). Minimal changes were observed in the glomeruli. Periodic acid–Schiff staining revealed Dutcher bodies in the nucleus of plasma cells. Immunohistochemical staining for immunoglobulins and light chains showed predominantly positive results for IgA and kappa (Fig. 2b). Monoclonality was confirmed by in situ hybridization. The infiltrates were positive for CD 45, CD 79a, and CD 138, partially positive for CD 19 and CD 20, and negative for CD 5, CD 10, and CD 23. Congo-red staining showed no evidence of amyloid. Immunofluorescence and electron microscopy revealed no detectable immunological deposits in the glomeruli and tubules. Mutations in the myeloid differentiation primary response gene 88 were not detected in the kidney. We diagnosed the condition as acute TIN by lymphocytes with IgA kappa-positive monoclonal plasma cells, distinct from MGRS. Considering the lack of evidence from previous reports on the impact of monoclonal plasma cells on nephropathy, we decided to treat the patient primarily for TIN with SS, initiating PSL at 30 mg/day (0.5 mg/kg/day). Renal function improved rapidly with the treatment, allowing for a gradual reduction in the dose of PSL; however, a low dose of PSL (10 mg/day) was required to prevent relapse. As a complication of PSL, the patient eventually developed a lumbar compression fracture, prompting us to evaluate the effect of PSL on renal function by a renal re-biopsy. The re-biopsy revealed significant recovery of TIN by lymphocytes (Fig. 2c). Other findings, including abundant interstitial plasma cells invasion were comparable to the initial biopsy. We diagnosed the condition as recovered TIN by lymphocytes with IgA kappa-positive monoclonal plasma cells. We concluded that TIN by lymphocytes, rather than monoclonal plasma cell invasion, primarily affected the nephropathy, and PSL mainly improved the former. The Nudix hydrolase 15 codon139 in blood was identified as Cys/Cys, indicating a high risk of severe adverse reactions to azathioprine in Asians. Therefore, we decided to concurrently administer mycophenolate mofetil, proven effective in the treatment of TIN with SS. Conclusion In the management patients with SS and IgA monoclonal gammopathy, the focus should be on TIN by lymphocytes rather than monoclonal plasma cell invasion. This case, considering similar cases, suggests that it should be defined as a new disease category. We demonstrate new renal pathology and treatment insights based on renal re-biopsy in a patient treated with PSL.

#1273 Rapidly progressive glomerulonephritis with anti-neutrophil cytoplasmic antibodies, in a patient on golimumab

Abstract Background 73-year-old Chinese lady presented with fever, gross hematuria, and dysuria of 1-week duration. She has seronegative arthritis and was treated with human anti-tumor necrosis factor alpha monoclonal antibody (TNF-alpha inhibitor), golimumab injection 50 mg monthly, as she had previous adverse reaction with sulfasalazine and methotrexate. She suffered from severe osteoporosis and was treated with oral alendronic acid. At presentation, her blood pressure was 172/76 mmHg and pulse rate 100 beats per minute. The highest recorded temperature was 38.1 degrees Celsius. Clinical examination was unremarkable. She was diagnosed with rapidly progressive glomerulonephritis (RPGN). Creatinine at presentation was 252 µmol/L, estimated glomerular filtration rate (eGFR) 16 mL/min/1.73 m2. Two months ago, her creatinine was 68 µmol/L, eGFR 66 mL/min/1.73 m2. Urinalysis showed dysmorphic red blood cells of more than 2250 per high power field, few white blood cells, and no epithelial cells. Urine protein/creatine ratio (UPCR) was 2.3 g/g and serum albumin level was 30 g/L. ANA was borderline raised at 1:160 titer with homogeneous pattern. Anti-double stranded DNA (anti-dsDNA) was indeterminate at 29.34 IU. Anti-myeloperoxidase (MPO) antibody was positive at 162 RU/mL (Normal &amp;lt; 20 RU/mL). Anti-proteinase-3 (PR3) antibody was negative. Serum complement levels (C3 and C4) were normal. Screening for hepatitis B, hepatitis C, and HIV was negative. Ultrasound imaging of the kidneys showed raised renal echoes and resistive index. Percutaneous kidney biopsy was performed. On light microscopy, 19 glomeruli were seen. Some displayed segmental increase in mesangial cells and expansion of mesangial matrix. 4 glomeruli showed cellular crescents (Fig. 1). Immunofluorescence showed dominant 2+ staining for Immunoglobulin A (IgA) in the mesangium and electron microscopy revealed electron dense deposits in the mesangial and para-mesangial region. An occasional tubuloreticular structure was found. Diagnosis of IgA nephropathy was made and Oxford classification was M0 E1 S0 T0 C1. Results Our patient presented with rapidly progressive glomerulonephritis (RPGN) with positive MPO antibody, as well as ANA and anti-dsDNA at indeterminate levels. TNF-alpha inhibitors may lead to autoantibody formation and kidney biopsy was the key in clinching the diagnosis for our patient. Diagnosis of IgA nephropathy was made on the basis of dominant mesangial IgA on immunofluorescence and ultrastructural electron dense deposits within the mesangial and para-mesangial area. To our best knowledge, there has been only one case of IgA nephropathy reported with golimumab that occurred after 3 years and with mild albuminuria and no decline in renal function. Golimumab was promptly discontinued for our patient and she was inducted with pulsed methylprednisolone and high dose oral prednisolone 1 mg/kg/day. As she had history of severe osteoporosis, oral mycophenolate mofetil at 750 mg twice a day was commenced as steroid sparing agent. Prednisolone dose was then tapered to 0.5 mg/kg/day. She achieved full clinical remission after 3 months of immunosuppressive therapy. MPO antibody level decreased to normal after 5 months. Arthritis activity remained quiescent and she was maintained on low dose prednisolone. Conclusion We report a case of IgA nephropathy with crescents associated with golimumab. Treatment was successful with withdrawal of drug, oral prednisolone and mycophenolate mofetil.

#1803 Is class II lupus nephritis as benign as one thinks?

Abstract Background and Aims The classification of Lupus Nephritis (LN) plays a pivotal role in determining prognosis and guiding treatment, aligning with ISN/RPS recommendations. KDIGO and EULAR advocate for immunosuppression in cases of proliferative LN (Class III/IV) and Class V with nephrotic syndrome, while “non-proliferative” instances (Class I and II) are advised conservative care. In a contrasting approach, GLOSEN recommends immunosuppression for Class II if persistent proteinuria exceeds 1g. ISN/RPS suggests incorporating activity and chronicity indexes to further refine the classification of LN. Concerns surrounding immunosuppression in LN Class II arise from its favorable long-term kidney survival and potential drug toxicity. However, the need for caution is underscored by a 30–50% class transformation rate. Limited research comparing the long-term prognosis of treated and untreated patients exists due to the gradual progression of the disease. The use of Activity (NIH-A) and Chronicity (NIH-C) indexes, particularly in the context of protocol biopsies, offers an opportunity to reassess the perceived benign nature of LN Class II. Method In this single-center investigation, patients diagnosed with LN belonging to Class II or II+V from 1999 to 2021 were examined. Biopsies were classified according to ISN/RPS criteria and evaluated using the NIH-A and NIH-C Indexes. Patients previously diagnosed with Class III/IV LN and those experiencing class transformation to a proliferative class were excluded. We present the findings in a descriptive manner. Results Sixteen patients, with a median age of 38.5 (12 female), were included. Histological findings from the initial biopsies revealed a NIH-A of 0, with NIH-C exceeding 0 in 31% (5/16) cases. Six patients exhibited Class II plus V, with varying NIH-C scores. The primary contributors to NIH-C in the initial biopsies were tubular atrophy and interstitial fibrosis. Ten patients underwent a second biopsy after an average time lapse of 39 months (7-101). The decision for most second biopsies was based on clinical judgment rather than a standardized protocol. One patient initially diagnosed with Class II+V displayed Class II on the second biopsy, and vice versa. In 70% of patients the NIH-C increased, primarily driven by glomerulosclerosis. The rise in NIH-C scores was observed in 3/6 patients with Class II+V and in all 4 patients with isolated Class II. Most patients received mycophenolate, and 3 were treated with low-dose prednisolone. The clinical and histologic characteristics of the 10 patients who underwent a second biopsy are outlined in Tables 1 and 2. Conclusion Our limited, biased study raises questions rather than providing definitive evidence. The consistent NIH-C rise challenges Class II LN perceived benign nature. It is plausible that not all LN Class II cases are equivalent and histological variances, such as mesangial proliferation, or its focal or diffuse distribution impact outcomes. Histological variations and treatment impact on LN Class II warrant further investigation.

Upfront Low-Dose Cytarabine with Prednisolone for Langerhans Cell Histiocytosis with Liver Dysfunction: A Ray of Hope

AbstractThe management of Langerhans cell histiocytosis (LCH) with accompanying liver dysfunction poses significant challenges, and this prompted the development of a modified low-dose cytarabine and prednisolone regimen. In this prospective observational study on children with LCH and liver dysfunction, four patients underwent induction and maintenance chemotherapy. The induction phase included 5 days of 100 mg/m2 cytarabine and 4 weeks of 40 mg/m2 daily prednisolone, with subsequent tapering. Maintenance included a regimen of 5 days of 100 mg/m2 cytarabine, along with oral prednisolone, repeated every 3 weeks. Complete disease resolution occurred after varying chemotherapy cycles. Three patients had liver transplants, and the chemotherapy resumed for 52 weeks after the transplant. In one child, chemotherapy was continued after reaching remission. In conclusion, a modified, less toxic low-dose cytarabine-based chemotherapy effectively managed LCH with liver dysfunction, with liver transplantation as a postremission treatment option.

#2460 Haemodialysis-related periarticular calcifications as cause of recurrent severe inflammatory state

Abstract Background and Aims End stage chronic kidney disease (CKD) and haemodialysis (HD) are associated with disturbances in mineral and bone turnover ultimately leading to chronic kidney disease–mineral and bone disorder (CKD-MBD), which affects more than 90 % of patients with stage 5D CKD. The primary changes observed in CKD–MBD involve elevated phosphorus levels, decreased calcium levels, insufficient serum vitamin D, and heightened release of parathyroid hormone from the parathyroid glands, leading to secondary hyperparathyroidism. CKD-MBD leads to vascular calcification, increasing cardiovascular risk. There is also a correlation between abnormal bone remodeling activity in CKD-MBD and the likelihood of calcification in soft tissues. In rare instances, profound calcification occurs in periarticular tissues, resulting in severe inflammation and manifesting as systemic disease with articular involvement clinically. The objective of this case report is to demonstrate a recurrent inflammatory condition caused by severe periarticular calcification in a patient on HD. Case report We present a case of a middle-aged white male diagnosed with hypertension, hypercholesterolemia, chronic obstructive pulmonary disease, hepatitis C, and end-stage renal disease caused by hydronephrosis. He was undergoing HD since July 2020. From summer 2021 the patient experienced recurrent fever and arthralgias in the hips, elbows, and shoulders. Frequent acute hospitalizations were needed due to recurring fevers and severe inflammatory response with markedly elevated acute phase reactants (CRP was repeatedly &amp;gt;200 mg/l), raising suspicion of infection. Laboratory and imaging tests however showed no clear signs of infection, and antibiotic therapy was ineffective. An x-ray of the right AC-joint showed multiple periarticular calcifications dorsally from the AC-joint. Later, due to severe pain and swelling an MRI-scan of right elbow joint was conducted, revealing an osteomyelitis-suspect finding of the olecranon, and olecranon bursitis. No bacteria were detected in the punctate of olecranon bursa. X-ray scans of the elbows revealed bilateral soft tissue calcification dorsally from the proximal ulna. The x-ray scan of the right shoulder revealed calcific expansion in the AC-joint area, leading to total destruction of the lateral part of the clavicle. MRI and CT scans showed severe periarticular calcification around AC- and glenohumeral joints without inflammatory involvement of the synovium. Bony erosions were observed due to the pressure of calcified tissue on the bone. Whole-body CT-scan showed no sites of infection but there was a periarticular calcification observed around the hip joints. A PET-CT scan of the whole body, neck, and upper extremities revealed no signs of malignancy or infections, but there was an increased uptake of 18F-fluorodeoxyglucose detected in several periarticular sites, indicating a multi-site inflammatory process resulting from severe periarticular calcifications. A dual-energy CT-scan of upper extremities was performed, excluding the accumulation of monosodium urate crystals. The cycle of multiple hospitalizations and antibacterial therapies was broken with the diagnosis of CKD and HD-related inflammatory periarticular calcification. Initially, the inflammation was addressed with high-dose glucocorticoids. However, the dose was successfully tapered during the first month of treatment, and the patient continued to do well on a maintenance dose of 5 mg/day of prednisolone. Subsequently, hospitalizations, antibiotics, or surgical procedures were no longer required. Conclusion Severe CKD and HD cause disturbances in mineral and bone metabolism, that can lead to periarticular calcification. Like in our presented case, multi-site calcifications may mimic infection or autoimmune rheumatoid conditions making the way to correct diagnosis cumbersome and costly. Imaging techniques help us distinguish periarticular deposits from inflammatory and infectious arthritis. In our case low-dose prednisolone effectively reduced inflammation, obviating hospitalizations and minimizing healthcare burden.

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