BACKGROUND Posterior reversible encephalopathy syndrome (PRES) is a neuro-radiological disorder characterized by seizures, headache, visual problems, and vasogenic edema visible on imaging scans. While it is most frequently linked to hypertensive crises, eclampsia, autoimmune conditions, and immunosuppressive drugs, its association with post-streptococcal glomerulonephritis (PSGN) is rare, particularly in adults. Typically, PRES associated with PSGN occurs in children and can develop even when blood pressure is mildly elevated. CASE REPORT We describe the case of a 20-year-old Sudanese man who presented with a sudden, severe headache, 2 episodes of generalized tonic-clonic seizures, and complete painless bilateral vision loss. Examination showed mild hypertension, periorbital swelling, and pedal edema. Laboratory tests indicated acute kidney injury, nephritic-range proteinuria, microscopic hematuria, low complement C3, and markedly elevated anti-streptolysin (ASO) titers, supporting a diagnosis of PSGN. Brain imaging revealed bilateral parieto-occipital vasogenic edema consistent with PRES. The patient was treated with oral antihypertensives, anticonvulsants, and a short course of steroids. Clinical improvement occurred within 24 h, with progressive vision restoration by day 3 and full recovery by day 5. Renal function normalized within 2 weeks, and no neurological deficits remained. CONCLUSIONS This case highlights an uncommon presentation of PRES secondary to PSGN in a young adult with mild-to-moderate blood pressure elevation. It emphasizes the importance of early recognition of PRES in patients with recent streptococcal infection and kidney dysfunction, even if they do not have severe hypertension. Rapid diagnosis and prompt treatment are crucial to achieve full neurological recovery and prevent potential complications.

This study developed a deep learning model for automated choroid plexus (ChP) segmentation and examined its relationship with systemic inflammation and processing speed and attention deficits (PSAD) in SLE patients without major neuropsychiatric syndromes. In this multicenter retrospective study, 137 SLE patients without major neuropsychiatric syndromes and 159 healthy controls (HCs) were enrolled. The Swin-UNETR model was trained for ChP segmentation on 3D T1-weighted MR images. SLE patients were classified as with processing speed and attention deficits (SLE-PSAD, n = 43) or intact processing speed and attention (SLE-IPSA, n = 94). Clinical, laboratory, and imaging data were compared among groups. Correlation, mediation, and LASSO regression analyses were performed. Swin-UNETR achieved high segmentation accuracy (median DSC = 0.89 internal, 0.82 external, P < 0.001). ChP volume was significantly greater in SLE-PSAD patients than in SLE-IPSA patients and HCs (P < 0.001) and positively correlated with systemic inflammation index (SII, r = 0.34, P < 0.001). Bayesian logistic regression identified increased ChP volume (aOR = 2.57), elevated SII (aOR = 2.47), and low complement component 3 (C3, aOR = 0.47) as independent PSAD risk factors. ChP volume mediated 39.2% of the SII-PSAD relationship (P < 0.001). LASSO regression identified a minimal three-biomarker model (ChP volume + C3 + SII) with excellent discriminative ability (AUC = 0.79 training, 0.77 validation). Enlarged ChP volume is a biomarker and mediator of PSAD in SLE patients. The Swin-UNETR model enables accurate ChP quantification, and the three-biomarker panel provides a practical tool for SLE-PSAD risk stratification.

Systemic lupus erythematosus (SLE) uncommonly presents with ocular manifestations as the initial feature. Uveal effusion syndrome (UES) is typically described in eyes with nanophthalmos and scleral abnormalities. This case is noteworthy for two reasons. First, the patient had normal axial length and no marked diffuse scleral thickening on ultrasound biomicroscopy(UBM), with a massive annular uveal/suprachoroidal effusion resembling a type III UES-like phenotype; however, concurrent active SLE, hypoalbuminaemia and polyserositis suggested a secondary systemic trigger. Second, ocular recovery appeared to accelerate after periocular corticosteroid therapy, despite inadequate response to systemic immunosuppression. A 38-year-old woman presented with three days of bilateral ocular pain and visual deterioration. Initial examination revealed intraocular pressure of 38.7 mmHg in the right eye and 32.7 mmHg in the left, shallow anterior chambers, and axial length of 21.85mm bilaterally. Bilateral angle-closure glaucoma was diagnosed, and laser peripheral iridotomy was performed along with topical antiglaucoma agents and corticosteroids. One week later, systemic symptoms including fever and polyarthralgia developed. Laboratory tests showed strongly positive autoimmune markers (ANA: antinuclear antibody, ANSA༚anti-nucleosome antibody, ARPA༚anti-ribosomal P protein antibody), low complement levels (C3༚complement 3, C4༚complement 4). Inflammatory markers were elevated (CRP༚C-reactive protein 84.08mg/L, ESR༚erythrocyte sedimentation rate 83mm/h), severe hypoalbuminaemia (27.4g/L). Imaging revealed pleural, pericardial, and peritoneal effusions. Active SLE with polyserositis and lupus nephritis was diagnosed. Intravenous methylprednisolone, cyclophosphamide, hydroxychloroquine and telitacicept were initiated. However, ultrasonography showed progressive 360-degree annular choroidal detachment with anterior displacement of the lens-iris diaphragm. Bilateral periocular triamcinolone acetonide 20mg was administered, followed by periocular dexamethasone 5mg. Choroidal detachment rapidly resolved, anterior chamber depth increased from 1.57mm to 2.93mm, and the retina reattached spontaneously. One month later, best-corrected visual acuity improved from 20/500 to 20/50 in both eyes, and intraocular pressure normalised. This case illustrates that systemic serous leakage in SLE can cause massive suprachoroidal effusion with secondary angle-closure glaucoma, even in eyes with normal axial length. Periocular corticosteroid therapy may serve as a useful adjunct by increasing local ocular drug exposure and promoting fluid resorption.

This prospective study evaluated first-trimester markers in pregnancies with isolated and combined forms of fetal growth disorders and gestational diabetes mellitus (GDM). Among 1869 screened women, the analysis included 83 controls, 55 GDM, 22 isolated intrauterine growth restriction (iIUGR), and 33 isolated large-for-gestational-age (iLGA) cases, with GDM subgroups stratified by fetal growth (GDM with normal fetal weight, GDM + IUGR, and GDM + LGA). First-trimester clinical and routine biochemical parameters were recorded, and serum concentrations of 80 proteins were measured using targeted LC-MRM-MS proteomics. Different trajectories emerged: IUGR phenotypes showed low PAPP-A/PlGF and high TSH (p < 0.01), indicating early placental insufficiency, while macrosomia showed opposite trends. GDM + IUGR represented the most severe “double hit” phenotype (lowest PlGF, earliest delivery), whereas GDM + LGA showed increased umbilical artery resistance despite excessive growth, suggesting endothelial dysfunction. Targeted proteomics revealed characteristic signatures: iIUGR featured low complement (C4A|C4B) and IGF proteins (IGFALS, IGFBP3) versus GDM and iLGA (p < 0.001); GDM + IUGR showed elevated PZP and CD5L versus iIUGR (p < 0.05); GDM + LGA was marked by high C4BPA and low RBP4, SERPINA7 versus iLGA (p < 0.05). Complement and IGF pathways were consistently implicated. Machine learning achieved 77% sensitivity for IUGR prediction using clinical parameters and 88% sensitivity for LGA prediction using proteomic data. These findings demonstrate that fetal growth disorders represent pathophysiologically unique entities detectable in the first trimester, enabling early risk stratification and personalized management.

Introduction Confirming systemic autoimmune rheumatic diseases in children is a challenge. Many diseases lack validated classification criteria, so we use criteria developed for adults. Case description A 13-year-old girl with an episode of parotitis, persistent fatigue, cervical lymphadenopathy, splenomegaly, malar rash, erythematous papules, photosensitivity, intermittent fever (≤ 38.5°C) without infection, prior arthralgia, and then arthritis of many joints. No clinical or subjective sicca symptoms. Bacterial, viral infections (human immunodeficiency virus, Epstein-Barr virus, cytomegalovirus, parvovirus B19), and toxoplasmosis were excluded. Laboratory tests revealed: leukopenia – 2.9 × 10³/μl, anemia – Hb 10.9 g/dl with positive DAT, [a]hypergammaglobulinemia; low C3, C4 complement, positive rheumatoid factor, antibodies to citrullinated protein antigens (–), anti-nuclear antibodies (ANA) (HEp-2) high-titer 1 : 10,240 (homogeneous) and 1 : 320 (speckled); anti-dsDNA elevated (393.2 IU/ml); anti-Ro60/Ro52 high (quantitative method); anti-SSB/La and AMA-M2 (+++; semiquantitative method); negative antiphospholipid antibodies, circulating immune complexes present. Based on the cervical lymph node biopsy, a proliferative process was excluded – reactive lymphoid hyperplasia. The patient was diagnosed with juvenile systemic lupus erythematosus (28 points European Alliance of Associations for Rheumatology/American College of Rheumatology classification criteria for SLE). Sjögren’s disease (SjD) diagnosis supported despite no minor salivary gland biopsy: typical Abs (anti-Ro60, anti-AMA-M2), SGUS score 2, recurrent sialadenitis. The SLEDAI 2K indicate high SLE activity (13 pts). Treatment with megadoses of glucocorticosteroid (GC) – methylprednisolone, maintenance therapy with oral GC (prednisone), methotrexate and hydroxychloroquine were introduced. Child lupus low disease activity was achieved. In 2024, GC was discontinued. The only clinical symptom was a discrete malar rash, peripheral blood counts, urinalysis were in norm, ANA titer 1 : 5,120 homogenous; 1 : 320 speckled pattern. Continued low C4 complement component and high Ro60 and Ro52 antibody concentrations without sicca symptoms. Conclusions Features of SLE appear to be identical to the adult form, but this is less obvious in children with SjD, especially with the absence of dryness symptoms. Recurrent parotitis, cutaneous manifestations, and similar laboratory abnormalities complicate the diagnostic process. Prior infections may act as a triggering factor, potentially explaining some atypical symptoms for both diseases (elevation of inflammatory markers). Treatment involves the same groups of drugs, and as in the case described, improvement was achieved in most domains.

Introduction Systemic lupus erythematosus (SLE) is a chronic, multi-organ inflammatory disease of autoimmune origin. In some cases, SLE may be the first symptom of cancer. Paraneoplastic syndromes in rheumatology manifest as symptoms of inflammation of joints, muscles, and blood vessels caused by cancer, but not directly related to tumour invasion or the presence of metastases. Systemic lupus erythematosus as a paraneoplastic syndrome was observed in patients with lymphoma, breast cancer, lung cancer, and, rarely, gastrointestinal cancer. Case description We present the case of a 69-year-old man who was admitted to the rheumatology clinic in September 2025 with an inflammatory rash on his face, neck, upper limbs and back, as well as oral ulcers, arthritis and weight loss. Laboratory tests revealed pancytopenia, low complement C3 and C4, positive antinuclear antibodies, and positive anti-ribosomal protein P. The patient fulfilled the 2019 European Alliance of Associations for Rheumatology/American College of Rheumatology classification criteria for SLE. Thoracic, abdominal and pelvic computed tomography scans showed pleural effusion, a single enlarged mediastinal lymph node, irregular thickening of the splenic flexure of the colon, and splenomegaly. Colonoscopy revealed a polypoid lesion in the distal transverse colon and descending colon, measuring approximately 3 cm; histopathological examination showed a tubular adenoma of the large intestine with high-grade dysplasia and a focus of invasion (adenocarcinoma not otherwise specified [NOS], low grade). The treatment consisted of glucocorticosteroids in high doses and hydroxychloroquine. As a result of the treatment, the skin and mucosal lesions, as well as arthritis, significantly decreased. In December 2025, a partial colon resection was performed. Since the operation, there have been no signs of SLE activity. Conclusions Any systemic connective tissue disease, including SLE, can be a paraneoplastic syndrome. Particular attention should be given to the possibility of cancer in cases involving severe skin lesions, general symptoms, and older age at the onset.

Introduction Atypical hemolytic uremic syndrome (aHUS) is a rare, progressive, often genetic disease that can be fatal if untreated. It may manifest with proteinuria, hematuria without acute kidney injury (AKI). It frequently leads to refractory hypertension, AKI and myocardial infarction or encephalopathy. We present a case of a 51-year-old patient, treated twice in the Clinic of Internal Medicine, Nephrology and Dialysis Therapy of the Military Institute of Medicine – National Research Institute, with refractory hypertension and AKI, who developed a-HUS, in the course of hypertensive nephropathy with renal failure. Case description A 51-year-old patient presented to the Emergency Department with a 1-month history of abdominal pain and intermittent vomiting for 5 months. On admission, the patient was in good general condition but in hypertensive crisis (blood pressure: 248/145 mmHg, heart rate: 121 bpm) requiring intravenous urapidil infusion. Severe renal failure was identified with a creatinine level of 5.7 mg/dl, urea 140 mg/dl and hypokalemia K+ 2.2 mmol/l. Further more, normocytic anaemia, thrombocytopenia, proteinuria and hematuria were observed. A multidrug antihypertensive regimen was initiated. The patient developed atrial fibrillation during hospitalisation, managed with amiodarone; echocardiography showed features of left ventricular hypertrophy, and head computed tomography showed diffuse white matter hypodensity. Labs indicated secondary hyperparathyroidism. A right kidney biopsy was performed, complicated by retroperitoneal hematoma with subsequent hypotension and anemization, however without signs of active bleeding. Amoxicillin with clavulanic acid was included in addition to 3 units of packed red blood cells. After radiologic consultation, angiography with additional renal artery embolization. The 50% hemolytic complement (CH50) and a disintegrin-like and metalloprotease with thrombospondin type 1 motif 13 (ADAMTS13) activity levels are not without abnormalities. Genetic screening for aHUS was initiated. Renal biopsy histopathology revealed arterionephrosclerosis, pointing to a hypertensive aetiology. Intravenous iron and folic acid supplementation were started for the mixed-aetiology anaemia. A month later readmission was due to high renal parameters, and acidosis was identified, along with anaemia and thrombocytopenia. Low complement C3 level 77 mg/dl. Genetic testing confirmed aHUS. However, the patient was not qualified for ravulizumab therapy by the National Commission. Conclusions The aHUS is a diagnostically challenging disease due to non-specific symptoms and multi-organ manifestations. This case illustrates one of the mechanisms in which aHUS may develop in the course of severe hypertension, which, in the setting of aHUS, was particularly refractory to treatment.

This study describes baseline demographics, clinical characteristics and treatments of patients diagnosed with systemic lupus erythematosus (SLE) at the time of registration in the registries of the Lupus Federated Data Network (LupusNet). Data were collected from five SLE registries in four global regions: APLC (Asia Pacific), FORWARD (North America), RELESSER (Europe), GLADEL 2.0 and Almenara (Latin America). LupusNet uses a federated data network approach and a privacy-by-design method, where only aggregated results are shared. Demographics, disease activity (based on Physician Global Assessment (PGA) and Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) scores), accumulated damage (Systemic Lupus International Collaborating Clinics Damage Index) and treatment use data were mapped and harmonised using the Observational Medical Outcomes Partnership Common Data Model V.5.4. A total of 10 370 patients were included in the analysis; of those, 3908 patients were in Asia Pacific, 3066 in North America, 1806 in Europe and 1590 in Latin America. The majority of patients were female (91%); the median (IQR) duration from SLE diagnosis to registry entry ranged from 5 (1-12) to 12 (6-19) years. Heterogeneity in disease activity was observed across registries based on mean (SD) SLEDAI-2K total (3.0 (4.1)-7.0 (7.4)) and PGA (0.4 (0.5)-1.2 (1.0)) scores. Across registries, SLE features most common were related to serologic activity (low complement and increased DNA binding (19%-47%)); other common clinical features included proteinuria and arthritis (4%-37%). Cataracts (4%-9%) were the most common characteristic related to accumulated damage. Glucocorticoids, antimalarials and immunosuppressants were the most commonly used treatments, with variations in their proportions observed across registries. These findings demonstrate significant heterogeneity in clinical characteristics and treatment patterns across the registries in LupusNet. By recognising regional differences, findings from LupusNet may help guide treatment approaches in SLE and optimise patient outcomes.

Systemic sclerosis (SSc) and renal involvement most frequently present as scleroderma renal crisis (SRC). Immune complex-mediated glomerulonephritis (GN) is an exceptionally rare renal manifestation in SSc and may complicate timely diagnosis and management. We report the case of a 40-year-old woman with a history of hypertension and Raynaud's phenomenon who presented with progressive dyspnea, bilateral lower limb edema, and oliguria. Laboratory investigations demonstrated rapidly deteriorating renal function (serum urea/creatinine - 71/6.44 mg/dL rising to 82/7.35 mg/dL within 24 h), nephrotic-range proteinuria (urine protein-creatinine ratio ≈ 11.6), hematuria (25-30 red blood cells/high-power field), and borderline low complement C3 (80.3 mg/dL). Autoimmune analysis revealed a speckled antinuclear antibody pattern (1:100) with Scl-70 positivity. Ultrasound showed enlarged kidneys with increased cortical echogenicity, and two-dimensional echocardiography confirmed moderate pulmonary arterial hypertension. Renal biopsy demonstrated diffuse proliferative GN with neutrophilic exudation, immunoglobulin G (IgG) and C3 immune complex deposition on immunofluorescence, and acute tubular injury, without thrombotic microangiopathy, thereby excluding SRC. The patient underwent hemodialysis and was initiated on low-dose corticosteroids. She was lost to follow-up after discharge. This case underscores an atypical and rare renal presentation of SSc, emphasizing the necessity of considering alternative diagnoses when renal dysfunction occurs without features of SRC. Early renal biopsy is essential to guide appropriate and individualized therapy in such cases.

Tjalma syndrome, also known as pseudo-pseudo Meigs syndrome, is a rare complication of systemic lupus erythematosus (SLE) characterized by ascites, pleural effusion, and elevated CA-125 levels in the absence of malignancy. We report a case of a 22-year-old woman with SLE presenting with vulvar swelling, severe polyserositis, nephritis, and progressive respiratory failure. SLE was confirmed by strongly positive anti-dsDNA with low complement levels. Despite immunosuppressive and supportive therapy, the patient's condition rapidly worsened, and she died due to hyperkalemia-induced ventricular fibrillation. This case highlights the diagnostic challenges of Tjalma syndrome, the need for thorough exclusion of malignancy, and the importance of early immunosuppression in preventing fatal outcomes.

Serological Stratification by Anti-Ro52 and Anti-Ro60 Profiles Reveals Distinct Systemic Phenotypes in Primary Sjögren's Disease.

Background: IgG4-related disease (IgG4-RD) is a multisystem fibroinflammatory condition capable of affecting nearly any organ. Renal involvement, although recognized, remains uncommon and frequently leads to diagnostic uncertainty because of overlap with autoimmune, infectious, and lymphoproliferative disorders. Case Presentation: A 34-year-old woman presented with a three-year course of intermittent painful purpuric plaques, chronic cough, Raynaud-like digital pallor, and episodic arthralgia. Examination revealed generalized non-tender lymphadenopathy. Laboratory studies showed anemia, eosinophilia, low complement levels, elevated IgG, and renal dysfunction. Autoimmune markers were negative. Imaging identified right-sided bronchiectatic changes and axillary lymphadenopathy. Skin biopsy showed leukocytoclastic vasculitis, while lymph node histology suggested reactive plasma cell-rich changes. A markedly raised serum IgG4 level (&gt;53 g/L) prompted kidney biopsy, which demonstrated storiform fibrosis and abundant IgG4-positive plasma cells, confirming IgG4-related tubulointerstitial nephritis. Discussion &amp; Conclusion: The combination of renal, cutaneous, pulmonary, and lymph node abnormalities, together with serological and histopathological findings, established the diagnosis of systemic IgG4-RD and illustrated its ability to mimic a broad differential diagnosis. Prednisolone therapy resulted in clinical and biochemical improvement, including normalization of renal function and improvement in complement levels. The patient remains in remission at 1 year.

Severe infusion-related reactions to rituximab are rare; we aim to extend our knowledge about them in children, focusing on rituximab-induced serum sickness (RISS) and anaphylaxis. We conducted a monocentric retrospective study on children and adolescents who received rituximab. Patients were defined as having RISS if they had fever and at least rash and/or arthralgia, 1 to 30 days following infusion, and without another diagnosis to explain symptoms. Anaphylaxis was defined according to the diagnostic criteria proposed by the World Allergy Organization. 1534 rituximab infusions in 391 patients were analyzed. Seven patients developed RISS; all received rituximab for an autoimmune disease, including four for immune thrombocytopenia (ITP). Six patients had fever, rash, and arthralgia. C-reactive protein or sedimentation rate was increased in all patients, and complement was decreased in 83%. Evolution was favorable within a few days with corticosteroids and/or intravenous immunoglobulins. Rituximab was reinfused in one patient, which resulted in an immediate anaphylactoid reaction. Lower doses of rituximab were less likely to induce RISS. RISS was associated with a greater chance of achieving ITP remission. Seven patients developed anaphylaxis; five successfully received further infusions using desensitization protocols. RISS in children is a severe complication of rituximab infusion. Our study suggests that it may be more frequent in individuals treated for autoimmune conditions, especially ITP. The classical triad of fever, rash, and arthralgia appeared to be frequently present, and biological inflammation and/or low complement can further support the diagnosis. In contrast to anaphylaxis, where rituximab may be safely rechallenged upon desensitization protocol, treatment alternatives should be pursued in patients experiencing RISS, given the higher risk of severe RISS recurrence.

BACKGROUND: Erythrodermic psoriasis (EP) is a severe, systemic inflammatory dermatosis. While kidney involvement is rare, the massive cytokine release associated with EP can induce systemic complications. We report a rare case of EP complicated by the simultaneous onset of immune complex-mediated crescentic glomerulonephritis (GN) and atypical haemolytic uraemic syndrome (aHUS), highlighting a severe form of complement-mediated renal injury in the context of dermatological inflammation. CASE PRESENTATION: A 52-year-old male presented with a three-month history of diffuse erythrodermic psoriasis following herbal supplement ingestion, complicated by progressive anasarca and acute kidney injury. Laboratory findings showed nephrotic-range proteinuria, microscopic haematuria, thrombocytopaenia (platelets 41 × 10^9/L), microangiopathic haemolysis (elevated lactate dehydrogenase and schistocytes), and low complement C3 levels. Skin biopsy confirmed EP. Renal biopsy revealed diffuse endocapillary proliferation and crescent formation (20% of glomeruli) with immune complex deposition (granularIgG, IgA), alongside features of thrombotic microangiopathy (endothelial swelling, segmental double contours). The patient was successfully treated with corticosteroids, intravenous immunoglobulin, and the terminal complement inhibitor eculizumab, resulting in normalisation of haematological parameters and substantial renal recovery. CONCLUSIONS: This case underscores a critical pathogenic link between severe psoriasis-associated systemic inflammation, immune complex deposition, and complement-mediated microangiopathy. Early recognition of this overlap syndrome and prompt complement inhibition are crucial for preventing irreversible kidney disease.

C3 glomerulopathy is an ultra-rare kidney disease driven by dysregulation of the alternative complement pathway fluid phase C3 convertase. We report the case of a previously healthy 13-year-old girl who presented with concurrent nephrotic and nephritic syndrome and low complement C3. Her initial kidney biopsy surprisingly showed mesangioproliferative glomerulonephritis with dominating IgA deposits resulting in a diagnosis of IgA nephropathy. Despite standard immunosuppressive treatment with prednisone and mycophenolic acid plus angiotensin-converting enzyme inhibitors, she achieved only partial remission and subsequently relapsed, exhibiting severe, persistent C3 consumption (6-8mg/dl) and sustained C5b9 activation (1059ng/ml). A repeat biopsy 1.2years later established the definitive diagnosis of C3 glomerulonephritis (C3GN) with a membranoproliferative pattern. C3NEF antibodies were negative, and no pathological variant was detected in the genetic study. After 1year and 9months without response to immunosuppressive treatment, the C3 inhibitor pegcetacoplan was initiated. The patient achieved rapid and complete remission within 3months, marked by normalization of proteinuria, from 2747mg/day (urine protein-to-creatinine ratio [UPCR] 2.66mg/mg) pre-treatment to 19mg/d (UPCR 0.02mg/mg) and complement activation markers (from 1162ng/ml pre-treatment to C5b9 92ng/ml; reference value 30-150ng/ml). This case highlights the inherent diagnostic complexity of C3GN, suggesting the critical role of repeat biopsies in treatment-refractory, complement-dysregulated glomerulonephritis. It strongly supports the potential efficacy of C3 inhibition as a targeted therapeutic approach for patients with C3GN.

A woman in her 30s presented with generalised swelling and a right occipital-temporal headache. Examination showed grade 4 papilloedema, and magnetic resonance venography revealed thrombosis of the right transverse and sigmoid sinuses extending into the internal jugular vein. Laboratory evaluation demonstrated anaemia, thrombocytopenia, severe hypoalbuminaemia, low complement levels and strongly positive serology for systemic lupus erythematosus (SLE), with a negative antiphospholipid profile. Initial urinary studies showed non-significant proteinuria (<0.5 g/day), making the cause of hypoalbuminaemia uncertain. After albumin infusion, repeat testing revealed borderline nephrotic-range proteinuria (3.044 g/day), and renal biopsy confirmed ISN/RPS (International Society of Nephrology/Renal Pathology Society) class IV diffuse proliferative lupus nephritis. Her thrombosis was attributed to the combined prothrombotic effects of active SLE inflammation, proteinuria and marked hypoalbuminaemia. Treatment with immunosuppression, anticoagulation and supportive care led to clinical improvement. This case highlights the diagnostic challenges of atypical lupus nephritis and emphasises the value of reassessing proteinuria after albumin correction to ensure timely recognition and management.

Evans syndrome (ES), the coexistence of autoimmune hemolytic anemia and immune thrombocytopenia, can unmask systemic autoimmune disease. We report a 30-year-old woman who presented with fatigue, jaundice, pallor, and mucocutaneous bleeding. Laboratory evaluation demonstrated severe Coombs-positive hemolytic anemia (hemoglobin 5.8 g/dL, reticulocytosis, high LDH, undetectable haptoglobin) and marked thrombocytopenia, whereas immunologic testing revealed high-titer ANA, anti-dsDNA positivity, and low complement levels; infections, thrombotic microangiopathy, and malignancy were excluded. She was stabilized with transfusion and treated with high-dose corticosteroids and intravenous immunoglobulin, achieving a partial response. Persistent cytopenias prompted rituximab, after which blood counts normalized and hemolysis resolved without major infectious or thrombotic events. At 6 months, she remained in remission on maintenance therapy. This case emphasizes that ES can be the initial manifestation of systemic lupus erythematosus and supports early autoimmune evaluation and tailored immunosuppression to address both cytopenias and underlying disease.

Lupus nephritis is a severe manifestation of systemic lupus erythematosus (SLE) typically characterized by glomerular "full-house" immunofluorescence. However, non-lupus nephropathies may occasionally exhibit similar patterns, creating diagnostic uncertainty. Non-lupus full-house nephropathy (FHN) is a recently recognized entity in which a full-house immunofluorescence pattern occurs in the absence of serological or clinical evidence of SLE. We report a 4-year-old girl who presented with generalized edema, hematuria, hypertension, and nephrotic-range proteinuria. Laboratory evaluation revealed anemia, elevated serum creatinine, and low complement C3 levels but negative antinuclear antibody (ANA) and anti-double-stranded DNA (anti-dsDNA) tests. Renal biopsy demonstrated diffuse proliferative glomerulonephritis consistent with lupus nephritis Class IV, with full-house immunofluorescence positivity for IgG, IgA, IgM, C3, and C1q. In the absence of systemic or serologic lupus features, a diagnosis of non-lupus full-house nephropathy was made. The patient was treated with pulse methylprednisolone followed by oral prednisolone, monthly cyclophosphamide for 6 months, and maintenance therapy with azathioprine and low-dose steroids. Hydroxychloroquine and antihypertensive agents were added. She achieved clinical and biochemical remission on follow-up.

To analyse the clinical characteristics of 30 children with haemolytic uremic syndrome (HUS) to enhance understanding, improve early diagnosis and explore prognostic strategies. A retrospective analysis was conducted on 30 HUS cases admitted to the nephrology department of Beijing Children's Hospital from 1 September 2020 to 30 April 2023. Clinical data, including laboratory results (with emphasis on haptoglobin levels and complement factors), kidney biopsy findings, genetic testing, treatment regimens (detailed dosages and courses) and follow-up outcomes, were manually extracted and verified by researchers. Efficacy was evaluated by haematological and kidney function recovery, and follow-up assessed remission, recurrence and chronic kidney disease (CKD) progression, with clear differentiation between dialysis dependence and CKD Stage 5. Aetiologies included secondary HUS (11 cases, mainly systemic lupus erythematosus, diagnosed after excluding other subtypes), cobalamin C deficiency (4 cases) and atypical HUS (aHUS, 15 cases). Most (63.3%) were over 6 years old, with a female predominance (male:female ratio 1:1.5). Common manifestations included nephrotic syndrome (73.3%), acute kidney injury (40% at Stage 3) and low complement C3 (90%). Haptoglobin levels were decreased in 89.3% of tested cases. Secondary HUS showed lower platelet counts (60.5 × 109/L vs. 135.9 × 109/L) and higher lactate dehydrogenase levels (1078.4 vs. 784.8 U/L) than aHUS (p < 0.05), likely due to concurrent autoimmune-mediated tissue damage. Treatment included plasma exchange, complement C5 inhibitors (eculizumab/crovalimab with specified dosages) and disease-specific therapies, with 60% complete remission. Follow-up revealed three cases of CKD Stage 5: one patient with dialysis dependence for < 3 months, one patient with dialysis dependence for > 3 months and one patient who received kidney transplantation (one additional patient underwent kidney transplantation independently of CKD Stage 5). Childhood HUS in this cohort is dominated by aHUS and secondary types. Early etiological differentiation, comprehensive laboratory assessment and targeted therapy improve outcomes, with findings aligning with global data but showing a more pronounced female bias due to high SLE-related cases.

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