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Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis that frequently infiltrates the peri-kidney space ("hairy kidney" appearance), kidney pelvis and proximal ureters, leading to obstructive uropathy. Here, we analyzed the clinical characteristics, imaging findings and long-term kidney outcome of a large multicenter cohort comprising 195 consecutive patients with ECD. Retroperitoneal peri-kidney or peri-ureteral involvement was detected at diagnosis in 147 patients. Of them, 70 had hydronephrosis (bilateral in 47), and 16 with kidney atrophy (unilateral in 14). Kidney vascular peduncle infiltration was found in 60 patients, and kidney artery stenosis in 31. The estimated glomerular filtration rate (eGFR) at diagnosis was significantly lower in patients with than in those without peri-kidney involvement (median 74 vs. 98 mL/min/1.73 m

Objectives To describe characteristics, treatment, and outcome of isolated antineutrophil cytoplasm antibody (ANCA)-associated scleritis at diagnosis, in comparison with idiopathic scleritis with negative ANCA tests. Methods This retrospective case control multicentre study was performed within the French Vasculitis Study Group (FVSG) network and in three French tertiary ophthalmologic centres. Data of patients with scleritis without any systemic manifestation and with positive ANCA results were compared to those of a control group of patients with idiopathic scleritis with negative ANCA tests. Results 120 patients, including 38 patients with ANCA-associated scleritis and 82 control patients, diagnosed between January 2007 and April 2022, were included. Median follow-up was 28 months (IQR 10–60). Median age at diagnosis was 48 (IQR 33–60) and 75% were female subjects. Scleromalacia was more frequent in ANCA-positive patients (p = 0.027). 54% had associated ophthalmologic manifestations, without significant differences. ANCA-associated scleritis more frequently required systemic medications, including glucocorticoids (76% versus 34%, p 5 mg/L at diagnosis was the only significant risk factor of progression to systemic AAV (adjusted HR 5.85; 95%CI 1.10–31.01; p = 0.038). Conclusion Isolated ANCA-associated scleritis are mostly anterior scleritis with higher risk of scleromalacia than ANCA-negative idiopathic scleritis and are more often difficult-to-treat. One third of patients with PR3- or MPO-ANCA scleritis progressed to systemic AAV.

Immune checkpoint inhibitors (CPIs) have recently become a cornerstone for the treatment of different advanced cancers. These drugs have the ability to reactivate the immune system against tumour cells but can also trigger a myriad of side effects, termed immune-related adverse events (irAEs). Although there are numerous reports of CPI-related endocrinopathies, hypercalcaemia as a suspected irAE is not well documented. The mechanisms of CPI hypercalcaemia are not clearly established. However, in our review, four distinct causes emerged: endocrine disease-related, sarcoid-like granuloma, humoral hypercalcaemia due to parathyroid-related hormone and hyperprogressive disease following CPI initiation. Prompt recognition of hypercalcaemia and the institution of therapy can be lifesaving, affording the opportunity to address the underlying aetiology. In this review we discuss the incidence, diagnosis and management of immune-related hypercalcaemia in oncological patients receiving CPI agents.

Erdheim-Chester disease (ECD) is a rare form of L group histiocytosis, accounting for up to 1500 cases to date worldwide, which mainly affects men between their 5th and 7th decade of life. The most frequent manifestations are bone involvement, perirenal infiltration with an evocating appearance of “hairy kidneys”, and a “coated aorta” aspect. Lung involvement in ECD is less common and includes pleural infiltration and interstitial lung disease. Herein, we report the case of a 76-year-old woman with recurrent pleuropneumonia revealing ECD.