Scielo RSS http://scielo.pt/rss.php?pid=2976-052620250002&lang=pt vol. 39 num. 2 lang. pt http://scielo.pt/img/en/fbpelogp.gif http://scielo.pt http://scielo.pt/scielo.php?script=sci_arttext&pid=S2976-05262025000200001&lng=pt&nrm=iso&tlng=pt http://scielo.pt/scielo.php?script=sci_arttext&pid=S2976-05262025000200003&lng=pt&nrm=iso&tlng=pt Abstract Introduction: Anti‑neutrophil cytoplasm antibody (ANCA)‑associated vasculitis (AAV) is a necrotizing disease affecting small blood vessels, commonly involving the kidneys, and associated with high morbidity and mortality. Methods: We conducted a descriptive cross‑sectional study at Hospital de São João in Porto, including patients aged 18 or older with biopsy‑proven kidney involvement from AAV, followed by a multidisciplinary team from December 2010 to June 2023. We analyzed demographic, clinical, and laboratory variables, kidney pathology findings, treatment regimens, and patient outcomes. Results: Fifty‑one patients were included, with a median age of 66 years (IQR, 56‑74) and a male predominance (61%). Most patients (78%) had myeloperoxidase (MPO)‑associated AAV. Chronic kidney disease (CKD) was present in 18% of cases, and 96% presented with rapidly progressive glomerulonephritis (RPGN). Hemodialysis was required in 29% of cases. Induction therapy consisted of cyclophosphamide and glucocorticoids in 86%, with a median cyclophosphamide dose of 6 g (IQR, 3.25‑8.25). After 2020, five patients received rituximab plus glucocorticoids. Plasma exchange was performed in 12 patients (nine with alveolar hemorrhage). Maintenance therapy included azathioprine in 32 patients and rituximab in six patients. Of patients requiring hemodialysis, 40% recovered kidney function. During follow‑up, 39% of patients progressed to end‑stage kidney disease, ten patients (20%) relapsed, and 17 died (33%). Conclusion: This study provides valuable insights into the epidemiology, treatment, and outcomes of AAV with kidney involvement in Portugal. Further studies are needed to optimize treatment strategies across more centers. http://scielo.pt/scielo.php?script=sci_arttext&pid=S2976-05262025000200009&lng=pt&nrm=iso&tlng=pt Abstract Hemodialysis imposes a significant psychological burden on people receiving this kidney therapy and their families. However, the role of Renal Psychologists remains underrecognized in the majority of nephrology centres worldwide, including in Portugal. This review article presents the rationale, development, and implementation of evidence‑informed psychological assessments and interventions organised in Portuguese dialysis centres. Overall, three resources - two patient‑reported outcome measures that aim to facilitate the triage of psychological distress and its sources in people on hemodialysis (Hemodialysis Distress Thermometer for Patients [HD‑DT]) and informal/family caregivers (Hemodialysis Distress Thermometer for Caregivers [HD‑DT‑C]), and an Internet‑mediated psychoeducational intervention (the Connected We St@nd programme) are presented and discussed as a means to optimise interdisciplinary collaborations and the expansion of professional psychological support services in (national and international) nephrology care settings. http://scielo.pt/scielo.php?script=sci_arttext&pid=S2976-05262025000200019&lng=pt&nrm=iso&tlng=pt João was prepared to receive another dialysis treatment, which had long since become a routine part of his life. By his side was Dr. Júlia Silva, his nephrologist, who was carefully adjusting the machine that performed the vital function his kidneys could no longer manage. The doctor was his constant support in his fight against chronic kidney disease and the reason why his relationship with Dr. Silva was built on mutual respect and trust. João was a quiet man who found comfort in his faith and acceptance of what life had destined for him. “You know, doctor, it was my belief in a higher power that gave me the strength to accept my illness”. Dr. Silva, with her rational and scientific mind, began to understand João’s perspective and to realize that it was influencing her own life. In the silence of the dialysis room the doctor and patient shared moments of encounter between science and spirituality, recognizing that life is made up of suffering and love, and that spiritual life opens space for hope, even in the most difficult times. João’s journey, supported by his unshakeable faith, showed Dr. Silva how powerful the invisible forces that guide and shape human beings are in our lives. http://scielo.pt/scielo.php?script=sci_arttext&pid=S2976-05262025000200028&lng=pt&nrm=iso&tlng=pt Abstract The current availability of multiple life support technologies, like chronic kidney support in an outpatient setting, frequently masks fragile conditions and complicates the process of decision‑making in end‑of‑life. To avoid disproportionate interventions that prolong suffering, adequacy of therapeutic effort (ATE) is a process that aligns therapeutic interventions with the patient’s clinical status, prognosis and wishes, respecting the natural course of life. In this paper, we present a case of a 44‑year‑old female with critical heart and kidney failure for which an ATE process was applied along the patient’s clinical course. In the end, we review the main barriers to ATE implementation. Training palliative care competencies among professionals and developing guidelines and juridical clarification sessions can be crucial pillars for the implementation of ATE in the Nephrology field. This topic is of particular importance, especially in a country like Portugal, where renal replacement therapy remains highly prevalent. http://scielo.pt/scielo.php?script=sci_arttext&pid=S2976-05262025000200031&lng=pt&nrm=iso&tlng=pt Abstract Autosomal dominant tubulointerstitial kidney disease (ADTKD) accounts for approximately 2%‑5% of monogenic causes of chronic kidney disease (CKD) and remains frequently underrecognized due to its heterogeneous clinical presentation. We report the case of a 37‑year‑old pregnant woman diagnosed with CKD, initially attributed to chronic pyelonephritis. She presented with bilateral renal cysts, electrolyte abnormalities, uterine malformation, and a family history of CKD. Genetic testing was pursued based on the phenotypic features, identifying a novel heterozygous HNF1B variant, c.785_786dup p.(Ala263Argfs*3), classified as likely pathogenic. Subsequent familial screening revealed that the patient’s mother, who was on hemodialysis for CKD of unknown etiology, carried the same mutation, leading to the reclassification of her CKD etiology as ADTKD related to HNF1B. This case highlights the clinical variability and diagnostic challenges of HNF1B‑related ADTKD, emphasizing the crucial role of genetic testing in identifying the etiology and improving disease understanding among nephrologists. http://scielo.pt/scielo.php?script=sci_arttext&pid=S2976-05262025000200034&lng=pt&nrm=iso&tlng=pt Abstract Systemic sclerosis is the collagenosis with the highest mortality rate, since multiorgan damage may occur, besides the characteristic skin involvement. The annual incidence is estimated to range from 8 to 56 cases per million inhabitants, with a predominance in women. Renal involvement occurs in approximately 50% of cases through various possible pathogenic mechanisms. We present the case of a 42‑year‑old woman undergoing immunosuppressive treatment for pulmonary involvement of systemic sclerosis with cyclophosphamide, who developed acute renal injury requiring dialysis. High titers of antineutrophil cytoplasmic antibodies (ANCA), with antibodies against myeloperoxidase, associated with a glomerular crescent histological pattern and a pauci‑immune pattern in immunofluorescence, indicated renal involvement by ANCA vasculitis in the context of high‑intensity immunosuppressive therapy for systemic sclerosis. After controlling infection and hemorrhagic complications related to the renal biopsy, pulse therapy with methylprednisolone and rituximab was initiated, leading to improved renal function and the discontinuation of dialysis therapy. The overlap between ANCA vasculitis and systemic sclerosis is challenging to recognize due to the rarity of this severe form of autoimmunity and the need to rule out other possible diagnoses in this context, such as scleroderma renal crisis. Special attention should be given to the response to the chosen immunosuppressive regimen, given the possibility of disease activation despite ongoing therapy. http://scielo.pt/scielo.php?script=sci_arttext&pid=S2976-05262025000200039&lng=pt&nrm=iso&tlng=pt Abstract Kidney supportive care (KSC) incorporates palliative care principles into nephrology, encompassing the full spectrum of kidney disease. Dialysis withdrawal, a component of kidney supportive care (KSC), presents a challenging, complex, and emotionally demanding therapeutic option. As patients experience increasing physical dependence on treatment, healthcare professionals may avoid or defer discussions regarding its withdrawal, making it more difficult for patients and their families to cope with decisions regarding end‑of‑life care. The ideal withdrawal involves a carefully considered, patient‑centered decision to minimize suffering and psychological discomfort for the family. This case report details the management of a patient with end‑stage chronic kidney disease on kidney replacement therapy whose dialysis was temporarily suspended due to an initial misdiagnosis of cancer. It underscores the critical importance of multidisciplinary collaboration and the challenges of complex therapeutic decisions such as dialysis withdrawal, especially in an inpatient setting. http://scielo.pt/scielo.php?script=sci_arttext&pid=S2976-05262025000200044&lng=pt&nrm=iso&tlng=pt Abstract Calciphylaxis is a rare life‑threatening vasculopathy resulting from calcium deposition in the arteriolar microvasculature of the deep dermis and subcutaneous adipose tissue, mainly occurring in end‑stage kidney disease (ESKD) treated with maintenance dialysis. Calciphylaxis in patients without ESKD is even rarer, which makes the diagnosis of calciphylaxis challenging in this population. In patients with any stage of kidney disease, there should be high clinical suspicion of calciphylaxis after the appearance of painful nodules, indurated plaques, dusky livedoid plaques, or nonblanching retiform purpura. We present a clinical case of a 75‑year‑old female with stable stage 5 chronic kidney disease who developed an ulcerated skin lesion on the posterior surface of the right lower limb, in whom calciphylaxis was not initially suspected because several common risk factors were not present, such as maintenance dialysis, altered calcium‑phosphate product or warfarin use. A biopsy‑proven diagnosis of calciphylaxis was made. Re‑epithelialization of the ulcerated lesions was achieved after the induction of haemodialysis, administering sodium thiosulfate in each dialysis session, wound care, and pain control through a multidisciplinary approach. Early identification and directed therapy initiation may be lifesaving in a disease that portends an ominous prognosis.