IgG4-related disease (IgG4-RD) is a multi-organ inflammatory immune-mediated illness caused by IgG4-secreting plasma cells infiltrating the tissue. This condition usually affects elderly men. A 90-year-old Chinese male was diagnosed with IgG4-RD based on the new 2019 ACR/EULAR classification criteria, as he had multiple organ involvement. After receiving treatment with glucocorticoids, leflunomide, and gamma-globulin, the patient\'s clinical symptoms significantly improved, confirming the accuracy of the diagnosis. The patient had an 18-year medical history during which the disease progressively worsened due to delayed diagnosis and treatment. Although the relevant symptoms were alleviated with appropriate medication, the overall treatment process encountered challenges. Due to the patient\'s relative lack of adrenocortical function, he experienced symptoms such as nausea, exhaustion, and loss of appetite during the hormone reduction process. Therefore, timely intervention is especially crucial to address the side effects of hormone therapy.

A 55-year-old man was admitted to the hospital with vomiting, diarrhoea, and chest pain. Upon examination, he exhibited signs of increased inflammatory response, acute kidney injury, and thrombocytopenia, leading to a diagnosis of TAFRO syndrome, which was supported by the clinical evidence of generalized lymphadenopathy, pleural effusion, and hepatosplenomegaly. Despite receiving intensive multimodal immunosuppressive therapy, including glucocorticoid pulse therapy (methylprednisolone 1,000 mg/day), tocilizumab, and cyclosporine in the intensive care unit, the patient showed minimal response and succumbed to the disease on the seventh day of hospitalization. Histopathological analysis of the lymph nodes revealed idiopathic multicentric Castleman disease (iMCD)-like features, and Epstein-Barr virus-encoded RNA (EBER) in situ hybridization identified multiple EBER-positive cells. These findings highlight the elusive pathogenic mechanism of TAFRO syndrome and the potential resistance of some patients to standard treatments such as tocilizumab. The presence of EBER-positive cells in lymph nodes or bone marrow may serve as an indicator of disease severity and treatment resistance. Therefore, histopathological detection of EBER-positive cells may help predict responsiveness to conventional treatments, disease severity, and prognosis in patients with TAFRO syndrome.

UNASSIGNED: Immune checkpoint inhibitors (ICIs) are increasingly being used in the treatment of advanced metastatic and immunogenic cancers. However, these therapies could cause immune-related adverse events (irAEs), which require high-dose glucocorticoid administration.
UNASSIGNED: A 52-year-old man with metastatic renal cell carcinoma received ICI therapy. Two weeks later, he suffered from severe irAEs and received glucocorticoid therapy for 13 months. Twenty-one months after the initiation of glucocorticoid administration, he presented to us with bilateral hip pain and was diagnosed with bilateral osteonecrosis of the femoral head (ONFH).
UNASSIGNED: IrAEs associated with ICI therapy might be an emerging underlying disease of ONFH.

Immunoglobulin G4-related disease (IgG4-RD) is a systemic fibroinflammatory condition characterized by significant infiltration of immunoglobulin G4 (IgG4)-positive plasma cells within affected tissues, with or without elevated serum IgG4 levels. The prevalence of IgG4-RD remains largely undetermined due to diagnostic challenges, as the condition is frequently unrecognized or misdiagnosed. This report describes a case of a 63-year-old man who was ultimately diagnosed with this rare condition after an extensive two-year period of elusive symptoms. Initially presenting with intermittent body pains and fluctuating fever, his condition progressively evolved to include severe right orbital swelling with marked tenderness and ecchymosis, recurrent non-tender nodules on his arm, and diminished vision. A detailed review of his medical history prompted the consideration of IgG4-RD, leading to the measurement of serum human IgG4 levels, which were found to be significantly elevated at 1504 mg/L (normal range: 39.2-864 mg/L). Following his diagnosis, treatment with glucocorticoids (0.6 mg/kg for one month) was initiated, resulting in a positive clinical response. This case emphasizes the critical importance of considering less common conditions in the differential diagnosis of patients presenting with complex, multi-system symptoms.

BACKGROUND: Pamidronate is used for the treatment of hypercalcemia. However, a rare but potential adverse event of pamidronate treatment is hypocalcemia. This report describes an unusual case of severe, irreversible hypocalcemia after a single injection of pamidronate for the treatment of hypercalcemia due to glucocorticoid withdrawal in a dog.
METHODS: An 11-year-old castrated male Maltese dog presented with anorexia, vomiting, and diarrhea (day 0). The patient had calcinosis cutis throughout the body, calcification of intraabdominal organs, mild azotemia, and severe hypercalcemia. The severe calcification was attributed to long-term glucocorticoid administration, which was discontinued 1 month before presentation. Fluid therapy, diuretics, calcitonin, and a single intravenous injection of pamidronate were used for the treatment of hypercalcemia. On day 14, normocalcemia was achieved, but renal failure occurred. On day 20, severe and irreversible hypocalcemia occurred, and on day 42, the patient was euthanized at the owner\'s request because of worsened hypocalcemia and renal failure.
CONCLUSIONS: Although hypocalcemia is an extremely rare adverse event of bisphosphonate treatment, bisphosphonates like pamidronate can result in potentially life-threatening conditions according to the patient\'s underlying conditions. Therefore, the patient\'s condition should be closely monitored and any underlying conditions should be carefully evaluated before initiating the treatment for hypercalcemia using pamidronate.

OBJECTIVE: Cushing\'s syndrome (CS) is associated with severe cardiovascular (CV) morbidity and mortality. Cardiac magnetic resonance (CMR) is the non-invasive gold standard for assessing cardiac structure and function; however, few CMR studies explore cardiac remodeling in patients exposed to chronic glucocorticoid (GC) excess. We aimed to describe the CMR features directly attributable to previous GC exposure in patients with cured or treated endogenous CS.
METHODS: This was a prospective, multicentre, case-control study enrolling consecutive patients with cured or treated CS and patients harboring non-functioning adrenal incidentalomas (NFAI), comparable in terms of sex, age, CV risk factors, and BMI. All patients were in stable condition and had a minimum 24-month follow-up.
RESULTS: Sixteen patients with CS and 15 NFAI were enrolled. Indexed left ventricle (LV) end-systolic volume and LV mass were higher in patients with CS (p = 0.027; p = 0.013); similarly, indexed right ventricle (RV) end-diastolic and end-systolic volumes were higher in patients with CS compared to NFAI (p = 0.035; p = 0.006). Morphological alterations also affected cardiac function, as LV and RV ejection fractions decreased in patients with CS (p = 0.056; p = 0.044). CMR features were independent of metabolic status or other CV risk factors, with fasting glucose significantly lower in CS remission than NFAI (p < 0.001) and no differences in lipid levels or blood pressure.
CONCLUSIONS: CS is associated with biventricular cardiac structural and functional impairment at CMR, likely attributable to chronic exposure to cortisol excess independently of known traditional risk factors.

A 40-year-old Indian male presented with rash and abdominal pain, leading to a diagnosis of IgA vasculitis, a rare condition in adults. This systemic vasculitis involves IgA immune complex deposition, resulting in inflammation and tissue damage. Diagnosis relies on clinical features and biopsy findings, with management focused on symptom relief and addressing organ involvement. Long-term prognosis varies, emphasizing the importance of multidisciplinary care and patient education for optimal outcomes.

UNASSIGNED: Leber\'s idiopathic stellate neuroretinitis (LISN) is a rare disease characterized by disk edema, peripapillary and macular hard exudates, and often, the presence of vitreous cells. To enhance clinical understanding of the disease, a retrospective analysis was conducted on a patient diagnosed with LISN at our hospital, and discussions were held regarding its diagnosis and treatment.
UNASSIGNED: We reviewed the medical records of a 26-year-old male patient whose main complaint was a decrease in visual acuity of both eyes for 4 days, which had worsened over the last day. After systemic examination, fundus fluorescein angiography, and indocyanine green angiography, the patient was diagnosed with LISN in both eyes. After treatment with glucocorticoids, the patient\'s vision showed a significant improvement.
UNASSIGNED: Upon admission, the visual acuity of both eyes was: VOD 0.05, VOS 0.25. After 5 days of treatment, the visual acuity of both eyes was: VOD 0.25, VOS 0.4. After 1 month of follow-up, the visual acuity of both eyes was: VOD 0.4, VOS 0.6. After 5 months of follow-up, the patient\'s vision improved to VOD 0.6, VOS 0.8.
UNASSIGNED: The cause of LISN remains unidentified. It is essential to rule out diseases exhibiting similar clinical signs but possessing a clear etiology. The primary treatment approach involves glucocorticoid-based anti-inflammatory therapy, potentially supplemented with antibiotics, antivirals, vasodilators, and traditional Chinese medicine. This disease is usually self-limiting and generally carries a favorable prognosis.

Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection can trigger autoimmune inflammation in the liver, leading to acute autoimmune hepatitis (AIH). We herein report a case involving a 39-year-old woman with a 23-day history of yellow skin and urine. Using the revised original scoring system of the International AIH Group, we definitively diagnosed the patient with acute severe AIH (AS-AIH). She began treatment with 80 mg/day intravenous methylprednisolone, which was gradually reduced and followed by eventual transition to oral methylprednisolone. The patient finally achieved a biochemical response after 30 days of therapy, and liver transplantation was avoided. Clinicians should be aware that the onset of AS-AIH after SARS-CoV-2 infection differs from the onset of conventional AIH with respect to its clinical and pathological features. Early diagnosis and timely glucocorticoid treatment are crucial in improving outcomes.

UNASSIGNED: Eosinophilic cholangitis (EC) is an uncommon form of benign biliary obstruction. It frequently eludes accurate clinical diagnosis, leading to inappropriate treatment methods. It is our aspiration that this clinical report will impart comprehensive insights into EC and, specifically, the critical role of tomographic examination.
UNASSIGNED: A 34-year-old man was urgently admitted to the hospital due to excruciating abdominal distress persisting for several hours. Following a six-day course of anti-inflammatory therapy, his symptoms displayed marginal improvement, prompting his discharge. He returned to the hospital a month later for re-examination on doctor\'s orders. Based on the results of the re-examination, the patient refused steroid hormone shock therapy and subsequently underwent laparoscopic left-lateral hepatic lobectomy in order to confirm the diagnosis. The preoperative absolute counts of eosinophils in the peripheral blood were documented as 2.3 × 109/L, 3.06 × 109/L, and 1.50 × 109/L consecutively; concurrently, the corresponding percentages of eosinophils were quantified at levels of 21.90%, 30.70%, and 19.20%. The subsequent postoperative pathological assessment unveiled EC as the definitive diagnosis. The patient has since remained free from disease recurrence and is presently alive.
UNASSIGNED: When encountering a patient presenting with persistent elevation in absolute eosinophil count in peripheral blood, coupled with imaging manifestations suggestive of intrahepatic periductal inflammation, diagnosis of EC should be highly suspected. The most optimal diagnostic and therapeutic workflow for EC could entail CT-guided liver lesion biopsy, ensued by glucocorticoid pulse therapy, and finally, short-term monitoring utilizing CT or MRI (including T1WI, T2WI, DWI, CEMRI) techniques.