Occult solitary fibrous tumour of the pleura presenting as recurrent spontaneous pneumothorax.

A woman in her 30s, non-smoker, presented at the emergency department two times because of spontaneous pneumothorax. The first episode was treated with small bore catheter drainage, while during the second episode-occurring only 1 week later-thoracoscopic talcage was attempted. The postoperative course was characterised by slow clinical and radiological resolution, and recurrence 3 days after discharge. Eventually, multiportal video-assisted thoracoscopic exploration identified an interfissural solid mass. Resection and further work-up revealed the diagnosis of 'low-risk' solitary fibrous tumour (SFT) stage pT1N0M0. The interdisciplinary tumour board advised no adjuvant therapy. A CT thorax was scheduled in 1 year for follow-up. The patient was discharged without complications and has had no recurrences of pneumothorax at 6 months of follow-up. This report shows that SFT can easily be missed on initial presentation and should be considered in the differential diagnosis of pneumothorax, especially when frequently recurring.

Post-traumatic pleural effusion: Don't forget the chylothorax!

In this case we describe a 58-year-old male with bilateral pleural effusion after a blunt trauma to the back. A pleural puncture revealed a chylothorax. An additional computed tomography scan showed a vertebral fracture at level D8 with rupture of the nearby thoracic duct. Our patient could be treated with a conservative approach. This case highlights the importance of ruling out a chylothorax in any post-traumatic pleural effusion. Despite the low prevalence, we consider it a don't-miss diagnosis given the specific treatment requirements.

Severe Hypertriglyceridemia After Use of Bempedoic Acid.

Bempedoic is a new drug for the management of hypercholesterolemia, approved since 2020 by the EMA for use in Europe. In this case report, we describe a 65-year-old woman with sudden worsening of hypertriglyceridemia after the introduction of bempedoic acid. Triglyceride levels normalized quickly on withdrawal of the drug. With this case report, we want to reveal a possible association between bempedoic acid and the paradoxical occurrence of hypertriglyceridemia. Furthermore, we want to emphasize the limited evidence regarding the use of bempedoic acid in patients with pre-existing hypertriglyceridemia. LEARNING POINTS: Bempedoic acid is a new drug with a proven positive effect on LDL reduction and cardiovascular outcomes.Metabolic adverse events, especially hyperuricemia and gout, following the use of bempedoic acid are well documented.The current literature provides very limited evidence regarding the use of bempedoic acid in patients with pre-existing hypertriglyceridemia, so caution is advised regarding use of this drug this population.

Tissue is the issue: when a second biopsy reveals the true diagnosis.

We describe the case of a woman with minimal glomerular changes on initial kidney biopsy. On long-term follow-up, the patient developed nephrotic proteinuria and a second kidney biopsy was performed, which revealed focal segmental glomerulosclerosis (FSGS). Findings from electron microscopy (EM) examination suggested a genetic form of FSGS. Next-generation sequencing showed heterozygosity for a mutation in COL4A3. Collagen IV nephropathies can be linked to late-onset FSGS. By establishing a genetic cause of FSGS, immunosuppressive treatment can be avoided. This case emphasizes the importance of re-biopsy in cases of a non-explained rise in proteinuria. EM can be helpful in differentiating between primary and secondary FSGS and informing treatment strategies. In cases of adult-onset FSGS that cannot be categorized by clinical-pathological assessment, genetic testing should be considered.

Symmetric polyneuropathy after viral symptomatology - not always Guillain-Barré Syndrome.

Introduction: Guillain-Barré Syndrome usually presents with ascending symmetric polyneuropathy, typically preceded by a viral infection. Despite the low incidence, physicians will often include Guillain-Barré Syndrome in their differential diagnosis. However, another underlying cause of polyneuropathy known as ANCA-associated vasculitis (AAV) is even more rare than Guillain-Barré Syndrome and therefore is usually overlooked. AAV has a broad spectrum of symptomatology and sometimes presents only with neurological complaints. If treated inappropriately, AAV can be lethal.Case report: In this case report, we describe a 72-year-old man presenting with complaints of symmetric polyneuropathy and paresis of both legs, initially diagnosed as Guillain-Barré Syndrome. During his treatment with intravenous immunoglobulins, he developed acute renal failure. Further investigations showed ANCA positive pauci-immune acute glomerulonephritis. This, in combination with eosinophilia and sinusitis, led to a final diagnosis of Eosinophilic Granulomatosis with Polyangiitis EGPA (Churg-Strauss disease). Induction therapy was initiated using glucocorticoids, cyclophosphamide and temporary plasmapheresis, followed by maintenance therapy with azathioprine complicated by bone marrow suppression. Azathioprine was discontinued and monotherapy with low-dose glucocorticoids was continued with the recovery of bone marrow function, good clinical condition and no relapse of vasculitis at 14 months follow-up.Conclusion: Physicians should be aware of the possible presentations of AAV. When suspected, indirect immunofluorescence assay for ANCA should be performed. When AAV is diagnosed, induction therapy should be administered as soon as possible, followed by maintenance therapy and careful follow-up, as patients are at risk for opportunistic infections, bone marrow toxicity or relapse.

Case report: successful treatment of membranous lupus nephritis with belimumab in an African female immigrant.

We describe the case of a 26-year-old African female who was treated successfully with belimumab in a case of severe membranous lupus nephritis and retinal vasculitis, resistant to first line therapy. She presented initially with chronic dacryoadenitis and screening showed nephrotic-range proteinuria. Biopsy of the kidney confirmed the diagnosis of membranous lupus nephritis. Clinical features (joint pain, dacryoadenitis, retinal vasculitis and lupus nephritis) in combination with serology (positive anti-double-stranded DNA (ds-DNA) antibodies, hypocomplementemia) confirmed the diagnosis of systemic lupus erythematosus (SLE). Treatment was immediately initiated with glucocorticosteroids (GCS), mycophenolate mofetil (MMF) and hydroxychloroquine sulphate (Plaquenil®). Tacrolimus was associated but no effect was observed with the proteinuria remaining in the nephrotic range and secondary effects of the glucocorticosteroids becoming a real concern. The patient was started on add-on belimumab with quasi-immediate effect on the proteinuria, making it possible to decrease the dosage of the other immunosuppressants and gradually stop them, even the GCS. The patient is currently in complete remission after 3 years of treatment with belimumab. We were able to stop immunosuppressive treatment but will keep her on antimalarial treatment as the most recent guidelines in treatment of SLE recommend.

Anticoagulation with fondaparinux for hemodiafiltration in patients with heparin-induced thrombocytopenia: dose-finding study and safety evaluation.

The optimal anticoagulation regimen for hemodialysis (HD) in patients with heparin-induced thrombocytopenia (HIT) has not been defined. Hemodiafiltration (HDF) adds a large convective component to HD, thereby changing the pharmacokinetics of most anticoagulants. Data on coagulation regimens for HDF are scant. We therefore aimed to study the feasibility, effectiveness, tolerability, and pharmacokinetics of fondaparinux anticoagulation in HDF. This was a prospective observational dose-finding study. Patients were started on fondaparinux at a dose of 0.05 mg/kg postdialysis body weight. Per protocol dose escalation was performed when significant clotting was observed and reduced when the anti-Xa activity postdialysis exceeded 0.4 IU/mL. Dose adjustments were made by steps of 0.01 mg/kg postdialysis weight. Anti-Xa activity was measured using a chromogenic method calibrated with low-molecular-weight heparin and validated against fondaparinux-calibrated anti-Xa activity. Four patients with HIT were followed for 160 sessions in total. At the end of the dose titration study, three patients ended at a maintenance dose of 0.03 mg/kg and one patient at 0.04 mg/kg of fondaparinux. Significant bleeding attributable to fondaparinux did not occur. The occurrence of clotting increased parallel to the reduction of fondaparinux dose, from 0/53 and 0/15 sessions at the higher doses (0.04 and 0.05 mg/kg) to 3/75 (4%) at 0.03 mg/kg and 1/17 (6%) at 0.02 mg/kg. Fondaparinux may be safely used and provides adequate anticoagulation for HDF in patients with HIT. We recommend to adjust dosage of fondaparinux to body weight and to initiate therapy at a dose of 0.03 mg/kg to prevent accumulation. Dose titration can be achieved by targeting postdialysis anti-Xa activity.