Cardiac involvement, which may remain clinically silent, is a major determinant of prognosis in eosinophilic granulomatosis with polyangiitis, and remission induction generally requires glucocorticoids (GCs) combined with cyclophosphamide or rituximab. In contrast, treatment selection may be challenging in women wishing to preserve fertility because cyclophosphamide carries a risk of gonadal toxicity. We report the case of a 40-year-old woman with a history of asthma, eosinophilic chronic rhinosinusitis, and eosinophilic otitis media who developed postpartum-onset eosinophilic granulomatosis with polyangiitis and presented with neurological and cutaneous manifestations. Elevated cardiac troponin I levels and newly developed electrocardiographic abnormalities suggested subclinical myocardial injury despite the absence of cardiac symptoms. Intravenous methylprednisolone pulse therapy was initiated immediately. Because cardiac troponin I levels showed no early decline after GC initiation, and GC monotherapy was considered potentially insufficient to control eosinophil-mediated myocardial injury, additional eosinophil-targeted therapy was selected after shared decision-making that balanced fertility preservation with the need for prompt disease control. Benralizumab was chosen because of its potent eosinophil-depleting effect. Peripheral eosinophils rapidly decreased to zero, troponin I normalised by Day 26, and GCs were successfully discontinued within 5 months. To our knowledge, this is the first report of benralizumab used as part of a fertility-preserving remission-induction strategy to avoid cyclophosphamide in a woman with eosinophilic granulomatosis with polyangiitis and cardiac involvement. This approach may represent an individualised therapeutic option for carefully selected patients in whom fertility preservation is an important treatment consideration.
Juvenile Systemic Lupus Erythematosus (jSLE) is a rare disorder that presents with greater severity compared to adult Systemic Lupus Erythematosus. It typically manifests in adolescent females with constitutional symptoms, multisystem involvement, and a malar rash. In this case, a 9-year-old African American pre-pubertal girl presented with a progressive facial and scalp rash, weight loss, night sweats without fever, and abdominal pain following recent penicillin exposure. On laboratory results and clinical examination, leucopenia, auricular chondritis, palatal ulceration, and marked facial rash were found on physical exam and initial lab testing, alongside a negative infectious disease workup. Autoimmune workup demonstrated an elevated ANA and anti-dsDNA with low complement levels. Therefore, she met diagnostic criteria for jSLE. She improved rapidly with corticosteroids. Over the following year, she subsequently developed lupus nephritis. This case illustrates an atypical presentation of jSLE with early-onset prior to puberty and uncommon features, including chondritis, discoid lesions, and isolated mucocutaneous symptoms, with no associated joint pain or organ damage at initial presentation. Atypical jSLE presentations require high clinical suspicion to prevent delayed diagnosis and progression to complications such as lupus nephritis.
Pregnancy after anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody-positive dermatomyositis-associated interstitial lung disease is rarely described, and preconception management remains uncertain. A 29-year-old woman presented with fever, arthralgia, cough, characteristic Gottron's and inverse Gottron's papules, normal muscle enzyme levels, mild interstitial lung abnormalities on high-resolution computed tomography, and positivity for anti-melanoma differentiation-associated gene 5 antibody. Because of the potential risk of rapid pulmonary deterioration, she received prednisolone, a calcineurin inhibitor, and intravenous cyclophosphamide, followed by maintenance immunosuppression. Mycophenolate mofetil was discontinued as part of preconception planning 24 months after disease onset. After two biochemical pregnancies that ended before ultrasonographic confirmation of a gestational sac, a subsequent pregnancy was confirmed approximately 36 months after disease onset while she was receiving prednisolone 10 mg/day and tacrolimus. Pregnancy was not complicated by relapse of dermatomyositis or interstitial lung disease, hypertensive disorder, gestational diabetes, fetal growth restriction, or non-reassuring fetal status. She delivered a healthy female infant at 38 weeks and 3 days of gestation by vaginal delivery. At more than 4 years postpartum, she remained relapse-free, and the antibody remained negative. This case suggests that planned pregnancy may be feasible in carefully selected patients after sustained preconception disease control, discontinuation of teratogenic drugs, and continuation of pregnancy-compatible immunosuppression under multidisciplinary surveillance; serial anti-MDA5 antibody measurements may provide adjunctive information when interpreted alongside clinical, pulmonary function, and imaging assessments.
Chronic non-bacterial osteomyelitis/chronic recurrent multifocal osteomyelitis is an autoinflammatory, sterile bone disorder that predominantly affects children but can also occur in adults. The nonspecific nature of its presentation, as well as a relative lack of awareness of the condition, often results in significant diagnostic delay. In this article, we present the case of a female patient who began experiencing sternal pain at the age of 14, initially thought to be due to costochondritis but ultimately discovered to be secondary to chronic non-bacterial osteomyelitis/chronic recurrent multifocal osteomyelitis. We demonstrate her long and tortuous diagnostic journey, including key imaging and bone biopsy findings. We also report good clinical and radiographic response to tumour necrosis factor-α inhibitors, despite having to switch from adalimumab to etanercept and then to methotrexate due to side effects and anti-drug antibody production.
Sjögren's disease (SjD) is a systemic autoimmune disorder with glandular and extra-glandular manifestations. Pleural effusion is a rare complication of SjD among different types of pulmonary involvement. Herein, we report a case of primary anti-centromere antibody (ACA)-positive primary SjD with pleural effusion. A 79-year-old woman was admitted to our hospital with a 3-month history of leg oedema. She was diagnosed with primary SjD based on positive Schirmer and Saxon tests, and her anti-Sjögren syndrome-related antigen/Ro antibody test was negative, but ACA and anti-Sjögren syndrome-related antigen B/La tests were positive. The patient's symptoms promptly improved following prednisolone treatment. This is the first reported case of ACA-positive primary SjD complicated with pleural effusion, and the possible presence of pleural effusion should be considered in SjD, even in patients with ACA-positive primary SjD.
