Lupus Nephritis (LN) still represents one of the most severe complications of Systemic Lupus Erythematosus (SLE) and a major risk factor for morbidity and mortality. However, over the last few years, several studies have paved the way for a deeper understanding of its pathogenetic mechanisms and more targeted treatments. This review aims to provide a comprehensive update on progress on several key aspects in this setting: pathogenetic mechanisms of LN, including new insight into the role of autoantibodies, complement, vitamin D deficiency, and interaction between infiltrating immune cells and kidney resident ones; the evolving role of renal biopsy and biomarkers, which may integrate information from renal histology; newly approved drugs such as voclosporin (VOC) and belimumab (BEL), allowing a more articulate strategy for induction therapy, and other promising phase III-immunosuppressive (IS) agents in the pipeline. Several adjunctive treatments aimed at reducing cardiovascular risk and progression of chronic renal damage, such as antiproteinuric agents, represent an important complement to IS therapy. Furthermore, non-pharmacological measures concerning general lifestyle and diet should also be adopted when managing LN. Integrating these therapeutic areas requires an effort towards a holistic and multidisciplinary approach. At the same time, the availability of an increasingly wider armamentarium may translate into improvements in patient’s renal outcomes over the next decades.

Autosomal dominant polycystic kidney disease (ADPKD) is a genetic kidney disease characterized by gradual kidney enlargement and progressive renal function loss. Pregnancy is a significant risk factor for adverse maternal and fetal outcomes in chronic kidney disease (CKD), regardless of the nephropathy. Women with ADPKD often face concerns about worsening their renal condition and passing the disease on to their offspring. Recent studies show better outcomes due to improved pregnancy surveillance and prior counseling. Risk factors for poor fetal and maternal outcomes include advanced maternal age, pre-existing hypertension, urinary infections, proteinuria, and renal failure. Collaboration between nephrologists and gynecologists is essential for addressing clinically significant concerns in pregnant women with ADPKD. Nephrologists should play an active role in assisting and supporting pregnant ADPKD patients as part of a multidisciplinary team.

Introduction: Autosomal dominant polycystic kidney disease (ADPKD) is the most common genetic kidney disease and the 4th leading cause of renal replacement therapy in the world. ADPKD is a systemic disorder as cysts may develop in several organs. Liver cysts are the most common extrarenal manifestations and are often incidentally detected. Even though cysts do not influence liver function, they can grow to a very great size and can significantly enlarge liver volume, causing structural distortion of the biliary tree and patient discomfort due to the mass effect. Nephrectomy is frequently considered in preparation for renal transplantation in patients with remarkable kidneys’ enlargement. There are currently no globally recognized clinical guidelines for nephrectomy. Although cysts do not normally affect liver function in ADPKD, after nephrectomy cases of liver fibrosis and Budd-Chiari have been reported. These are uncommon disorders due to the obstruction of the blood flow in the hepatic venous causing spleen and liver volume enlargement, portal hypertension, and hepatic cirrhosis. Case Presentation: We present a case of hepatic fibrosis with splenomegaly and severe pancytopenia as a tardive complication after bilateral nephrectomy in 47-year-old ADPKD patient. Conclusion: This finding underscores the critical significance of meticulously examining the anatomical relationship between polycystic kidneys and the liver before performing nephrectomy. Additionally, it highlights the importance of assessing liver involvement and associated complications. By integrating liver assessment into the criteria, we can significantly enhance patient care and improve the overall management of ADPKD before kidney transplantation.

Key Clinical Message: We present the case of a 41‐year‐old man patient diagnosed with solitary left kidney with few cysts. He has a family history of unilateral renal agenesis (URA) but no for autosomal dominant polycystic kidney disease (ADPKD). Genetic testing revealed PKD1 gene intron 11 heterozygous nucleotide variant c.2854‐23G>T, but no gene mutation implicated in URA. Just eight cases of ADPKD with one kidney have been recorded globally. PC1 and PC2 disruption, causing primary cilia malformation or absence resulting in relevant in the first embryonic development alteration. Cillia's crucial significance in many diseases will require more research.

Autosomal dominant polycystic kidney disease is a genetic disorder characterized by the progressive development of renal cysts, leading to end-stage renal disease in a significant proportion of affected individuals. While the disease affects both men and women, emerging evidence suggests that sex differences may influence the clinical presentation, progression, and response to treatment in autosomal dominant polycystic kidney disease patients. Based on our experience, women are diagnosed earlier and often experience more symptoms like chronic pain and microhematuria. However, disease progression tends to be slower. Hormonal therapy, controversial due to its impact on cyst growth, is important for contraception and conditions like polycystic ovary syndrome, endometriosis, and menopause. Copper intrauterine devices are the safest contraceptive option, while progestin-only methods are recommended for those who cannot take estrogen. This review aims to explore the implications of gender differences in autosomal dominant polycystic kidney disease, highlighting the need for personalized approaches to diagnosis, monitoring, and management. Emphasizing the integration of gender-based insights into clinical practice could ultimately lead to more tailored therapeutic strategies, improving patient outcomes in autosomal dominant polycystic kidney disease.