rituximab (Kikuzubam / PBO326)

Rituximab not superior to azathioprine for EGPA remission maintenance.

Onuora Sarah S

A Case of Parkinsonism in a Patient with Pemphigus Vulgaris after Rituximab Treatment.

Menteşoğlu Dilek D, Atakan Nilgün N

Diffuse alveolar hemorrhage and myositis complicating eosinophilic granulomatosis with polyangiitis: a case report and narrative review.

Subki Ahmed Hussein AH, Albeity Abdurahman A, Alghamdi Maha Khider MK, Sayes Najla M NM et al.

Eosinophilic granulomatosis with polyangiitis (EGPA) is an uncommon antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis characterized by asthma, eosinophilia, and multisystem involvement. Diffuse alveolar hemorrhage and myositis are both infrequent manifestations of EGPA, and their co-occurrence in the same patient has rarely been reported. A 46-year-old man with adult-onset asthma, chronic sinusitis, and marked peripheral eosinophilia was diagnosed with EGPA in 2015. Over six years of follow-up, he had recurrent pulmonary relapses with migratory infiltrates and developed gastrointestinal involvement, nondilated cardiomyopathy, coronary artery disease, myositis, and diffuse alveolar hemorrhage; he was positive for myeloperoxidase (MPO)-ANCA. Disease activity persisted despite high-dose glucocorticoids, azathioprine, cyclophosphamide, and mycophenolate mofetil. Because the disease was severe, organ-threatening, and predominantly vasculitic, an anti-interleukin-5 agent was not chosen for remission induction; instead, rituximab was started and induced sustained remission. This case illustrates that refractory, MPO-ANCA-positive EGPA may respond to B-cell depletion with rituximab. The randomized REOVAS trial did not show rituximab to be superior to conventional remission-induction therapy; however, it was not designed to establish equivalence with cyclophosphamide in severe disease, and the optimal strategy for severe refractory disease remains undefined. ANCA status, together with the pattern and severity of organ involvement, may help individualize treatment in this setting.

Shrinking Lung Syndrome with Favourable Response to Belimumab and Corticosteroids Alone.

Père Rémy R, Le Goueff Anouk A, Nguenang Marina M

Shrinking lung syndrome (SLS) is a rare complication of systemic lupus erythematosus. It is characterized by dyspnoea and a restrictive ventilatory defect, often associated with pleuritic chest pain. Given its rarity, there is no standardized treatment. Most published case reports describe a favourable outcome with corticosteroids alone or in combination with other immunosuppressive agents, such as cyclophosphamide or rituximab. Only a few cases treated with belimumab have been reported. We present the case of a 16-year-old female presenting with pleuritic chest pain, orthopnoea, and a restrictive ventilatory defect. Further investigations revealed positive antinuclear antibodies, a small left pleural effusion, and mild elevation of the left hemidiaphragm, findings consistent with SLS. Treatment with corticosteroids and belimumab was initiated, leading to significant clinical and biological improvement. This case highlights the potential role of belimumab as a first-line treatment for SLS in combination with corticosteroids alone. Shrinking lung syndrome is a rare condition with no standardized treatment.Most reported cases describe the use of corticosteroids in combination with azathioprine, mycophenolate mofetil or rituximab. Only a few cases have reported the use of belimumab.Belimumab may be considered as a first-line treatment option which could induce remission.