IgA Nephropathy (IgAN) is the most common cause of primary glomerulonephritis worldwide. Despite the histopathologic hallmark of mesangial IgA deposition, IgAN is a heterogenous autoimmune disease not only in terms of clinical presentation but also in long-term disease progression. The pathogenesis of the disease is complex and includes the generation of circulating IgA immune complexes with chemical and biological characteristics that favor mesangial deposition and reaction to mesangial under-glycosylated IgA1 accumulation, which leads to tissue injury with glomerulosclerosis and interstitial fibrosis. Patients with proteinuria over 1 g, hypertension, and impaired renal function at diagnosis are considered to be at high risk for disease progression and end-stage kidney disease (ESKD). Glucocorticoids have been the mainstay of treatment for these patients for years, but without long-term benefit for renal function and accompanied by several adverse events. A better understanding of the pathophysiology of IgAN in recent years has led to the development of several new therapeutic agents. In this review, we summarize the current therapeutic approach for patients with IgAN as well as all novel investigational agents.

Purpose: The association between COVID-19 and hypercoagulability is well established. This is a case of a patient with systemic lupus erythematosus (SLE) who developed unilateral renal vein thrombosis following COVID-19, the third case described in the international literature so far. Methods: Clinical, laboratory characteristics and outcomes of the patient were described in detail. Literature review was performed on MEDLINE database via Pubmed. Search items included COVID-19, renal infarction, and renal thrombosis. A total of fifty-three cases were located. Of these, only two patients had renal vein thrombosis but none of them carried a diagnosis of SLE. However, six cases have been published so far involving SLE patients in whom thromboembolic events developed following COVID-19, but none of them experienced renal vein thrombosis. Conclusion: The present case adds a new piece to the emerging puzzle of COVID-19 associated hypercoagulability, especially among patients with autoimmune diseases.

Early diagnosis and initiation of appropriate immunosuppressive treatment remain the cornerstone of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis at the cost of significant toxicity. In this report, we present a case of a 69-year-old female who presented with advanced renal insufficiency and evidence of pulmonary hemorrhage and was MPO–ANCA-positive with a clinical phenotype of granulomatosis with polyangiitis. Organ involvement included rapidly progressive glomerulonephritis (GN), along with extrarenal manifestations (skin, upper and lower respiratory system involvement, and onset of saddle-nose deformity). Kidney biopsy established the diagnosis of pauci-immune crescentic, sclerotic GN. She received therapy with glucocorticoids and cyclophosphamide, mainly due to life-threatening extra-renal manifestations, such as pulmonary hemorrhage. She avoided vasculitis-related death but she developed severe therapy-related toxicity, resulting in the discontinuation of immunosuppressive therapy. Continuous re-evaluation of patients with ANCA-associated vasculitis in terms of response to immunosuppressive therapy and treatment-related toxicity is crucial for their management.

Objectives: Vaccination against SARS-CoV-2 has been vital in alleviating the spread of the recent pandemic. We aimed to estimate the frequency and type of adverse events related to SARS-CoV-2 vaccine in patients with lupus nephritis (LN), and assess its impact, if any, on the risk of subsequent reactivation of nephritis. Methods: This was a retrospective, multicenter study which included patients with biopsy-proven LN, who had received at least one vaccine dose. Patients who ended up with end-stage kidney disease (ESKD) prior to vaccination or were diagnosed with LN after vaccination were excluded. Adverse events, systemic or local, COVID-19 outcomes (full recovery, death, or long COVID-19), outcome of LN (remission, refractory disease, relapse, ESKD or death), demographics, laboratory measurements, and immunosuppressive regimens were recorded. Results: Sixty-seven patients were included. The median age was 33 (20–46) years. Induction therapy for LN was administered to 92.5% of patients and 74.6% received maintenance therapy. Of these, 94.02% were in remission at vaccination. The BNT162b2 mRNA vaccine was administered in 97.01% of cases, with mild systemic adverse symptoms in 28.35% (myalgias 17.91%, headache 13.43%, arthralgias 13.43%, and fever 10.44%) and local adverse effects in 35.82% (pain 25.37%, swelling 13.43%). Overall, among patients in remission upon vaccination, two (3.17%) experienced a LN relapse within 5.75 (±0.25) months, while 75% of those with active disease at vaccination achieved remission within 21 (±2) months. Conclusions: SARS-CoV-2 vaccination appears safe for LN patients without serious adverse events occurring, and there is no significant impact in the clinical course of the disease.