BackgroundFocal segmental glomerulosclerosis (FSGS) is characterized by the presence of glomerular damage on histopathological examination. The major defining symptom of FSGS is proteinuria, which indicates damage to the glomerular filtration barrier. Additionally, FSGS is the most common cause of primary nephrotic syndrome. However, in Saudi Arabia, there is a paucity of research on this topic. Therefore, this study was designed to examine the clinical features, laboratory findings, and presence of comorbidities in patients with FSGS to determine their effects on clinical outcomes. MethodsWe retrospectively analyzed the histopathological and clinical data of patients diagnosed with FSGS via biopsy at King Abdulaziz University Hospital, Jeddah, Saudi Arabia, during the period 1989-2020. Biopsy samples were labeled according to the Columbia classification as tip, perihilar, cellular, collapsing, or not otherwise specified (NOS). ResultsWe included 39 children and 21 adults. Males accounted for 54.1% of the sample. Hypertension was the most common comorbidity. Regarding FSGS subtypes, 60.9% of the lesions in the adult patients were collapsing lesions, followed by NOS (26.1%). In pediatric patients, 36.8% of the lesions were NOS, followed by collapsing lesions (28.9%). We also observed a very low rate of remission. In both age groups, the most common clinical presentation was nephrotic syndrome. ConclusionWe found a high prevalence of collapsing and NOS FSGS subtypes in both the adult and pediatric age groups. The most prevalent outcome was the persistence of nephrotic syndrome with low rates of remission.

Objectives. This review addresses the microscopic features of immunoglobulin A nephropathy (IgA nephropathy), its prognostic variables in children, and measures to which extent these features and variables differ from adults. Furthermore, it describes the extent of this disease process among children in Saudi Arabia and the rest of the Arab countries and compares it with the data from the West and the Far East. Method. All the original work described the histological features of pediatric IgA nephropathy, and studies involved in developing the prognostic classification of IgA nephropathy, Oxford Classification, were reviewed. Moreover, the studies describing the crescent prevalence and outcome in pediatric IgA nephropathy in addition to thrombotic microangiopathy association were studied. National studies describing the prevalence of pediatric IgA nephropathy and pediatric crescentic glomerulonephritis were tracked with an overview of the regional data from the rest of the Arab world. Results. IgA nephropathy in children showed more glomerular proliferative changes and less glomerular vascular and tubule-interstitial chronic injury compared to adults. The reference study that described the association between thrombotic microangiopathy and IgA nephropathy did not include the pediatric age group. Moreover, it was found that the data from the Middle East was not encountered in developing the original and updated IgA nephropathy Oxford Classification. Furthermore, the prevalence of IgA nephropathy in children is described in the regional literature, but its histological features were not well detailed. Finally, the percentage of crescentic glomerulonephritis (GN) due to IgA nephropathy is less in our country compared to the West and concords with the Far East findings. Conclusion. A well-designed regional study addressing IgA nephropathy in Middle East children with a focus on histological features, association with crescent, and thrombotic microangiopathy and challenging the validity of the updated IgA nephropathy Oxford Classification is recommended.

We report the case of an adolescent girl with frequent hospital admissions for severe eczematous skin rashes with recurrent epistaxis and chest infections. Investigations revealed persistent severely elevated serum total immunoglobulin E (IgE) levels but normal levels of other immunoglobulins, suggesting hyper-IgE syndrome. The first skin biopsy revealed superficial dermatophytic dermatitis (tinea corpora). Another biopsy performed after six months revealed a prominent basement membrane with dermal mucin, suggesting an underlying autoimmune disease. Her condition was complicated by proteinuria, hematuria, hypertension, and edema. A kidney biopsy revealed class IV lupus nephritis, according to the International Society of Nephrology/Renal Pathology Society (ISN/RPS). Based on the American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) criteria, she was diagnosed with systemic lupus erythematosus (SLE). She was first administered with intravenous pulse methylprednisolone (600 mg/m 2 ) for three consecutive days, followed by oral prednisolone (40 mg/m 2 ) daily, mycophenolate mofetil tablets (600 mg/m 2 /dose) twice daily, hydroxychloroquine (200 mg) once daily, and three classes of antihypertensive medications. She maintained normal renal functions with no lupus morbidity for 24 months, then rapidly progressed to end-stage kidney disease, and was then started on three to four sessions of regular hemodialysis per week.Hyper-IgE is known to be a marker of immune dysregulation as it facilitates the generation of immune complexes (ICs) that mediate lupus nephritis and juvenile SLE.Regardless of the different factors that are impacting the production of IgE, the present case illustrated that juvenile patients with SLE may have increased IgE levels, indicating that higher IgE levels might have a role in lupus pathogenesis and prognosis. The mechanisms regarding the increased levels of IgE in subjects with lupus need further investigation. Further studies are thus required to assess the incidence, prognosis, and possible new specific management for hyper-IgE in juvenile SLE.

The degree of interstitial fibrosis and tubular atrophy (IFTA) seen on kidney biopsy has long been used to judge the chronicity of kidney disease to predict renal disease outcomes and prognosis. It is an essential component incorporated in many renal disease prognostic classification systems on the native and renal allograft. The impact of increased body mass index on the body metabolism, and the human vascular system, including the functional unit of the kidney, the nephron, is well-addressed in the literature. In this study, we focus on evaluating the degree of IFTA concerning the patient's body mass index (BMI). MethodAll the specimens of nephrectomies performed in King Abdulaziz University Hospital for adults from January 2010 to February 2021 were evaluated for this study. A total of 125 cases were selected for the study. The glass slides were pulled and assessed for the degree of IFTA. The demographic data, and the patient's BMI, were collected from the hospital records. Results

Background Crescentic glomerulonephritis (CrGN) is a pathological description of rapidly progressive glomerulonephritis (RPGN). It is characterized by renal failure and is associated with a grave prognosis. This study aimed to investigate the clinical outcomes of patients diagnosed with crescentic glomerulonephritis at the King Abdulaziz University Hospital (KAUH) in Jeddah, Saudi Arabia. Method This retrospective study included patients with CrGN who underwent treatment at the nephrology department at KAUH from June 2021 to August 2022. We collected and analyzed data from 56 patients diagnosed with CrGN on the basis of renal biopsies between 2002 and 2015. Result The study included 17 cases of CrGN. The mean age of patients at the time of diagnosis was 18.06 ± 13.49 years. The distribution of histological findings showed that cellular crescents (94.1%) and interstitial fibrosis and tubular atrophy (IFTA) (76.5%) were the most commonly observed histological findings. The most common underlying etiology was lupus nephritis (41.2%). Regarding the lab results, the mean serum creatinine level at admission was 378.88 ± 273.27 μmol/L, proteinuria was 1.53 ± 1.23 and glomerular filtration rate (GFR) level was 36.94 ± 45.08 mL/min. The factors associated with poor renal outcome were IFTA (P=0.01), phosphate level before discharge, serum creatinine level before and after discharge (P=0.032), and GFR level after discharge (P=0.001). Conclusion Crescentic glomerulonephritis is an important cause of acute kidney injury due to its potential to result in severe glomerular injury. In our study, 12 out of 17 patients experienced poor renal outcomes, which were associated with a high risk of morbidity and mortality. Therefore, early detection and treatment of CrGN is crucial in order to manage the disease.