ABSTRACT
A woman in her 20s with no medical history presented with progressive abdominal distension, right-sided abdominal discomfort, fatigue and nausea. Examination showed multifocal lymphadenopathy and hepatomegaly with tense ascites. Investigations revealed a multisystem inflammatory condition characterised by elevated acute phase reactants, anaemia, thrombocytopenia, acute kidney injury, lymphocytic ascites, hypoalbuminaemia and hypergammaglobulinaemia. HIV and human herpes virus-8 tests were both negative. In the presence of elevated ANA and SS-A/Ro antibodies, the patient was suspected to be carrying a connective tissue disease, most likely systemic lupus erythematosus (SLE). Clinical and laboratory findings fulfilled the diagnostic criteria for SLE. However, lymph node biopsy showed interfollicular plasmacytosis, associated with high interleukin 6 (IL-6) and vascular endothelial growth factor titers, together hinting towards a rare diagnosis of multicentric Castleman's disease (MCD). As we investigated further, renal biopsy was consistent with thrombotic microangiopathy which has been previously reported in MCD. Furthermore, immune staining on the renal biopsy was negative for 'full-house' immunoglobulin and complement staining pattern, which is specific for lupus nephritis, helping us exclude SLE. In light of these new findings, the patient was started on anti-IL-6 therapy which provided a successful outcome.
Subject(s)
Castleman Disease , Lupus Erythematosus, Systemic , Ascites/complications , Castleman Disease/complications , Female , Humans , Lupus Erythematosus, Systemic/complications , Lupus Erythematosus, Systemic/diagnosis , Lupus Erythematosus, Systemic/pathology , Lymph Nodes/pathology , Vascular Endothelial Growth Factor A/therapeutic useABSTRACT
BACKGROUND: The presence of bifascicular block on electrocardiography suggests that otherwise-unexplained syncope may be due to complete heart block. European Society of Cardiology (ESC) recommends investigating it with electrophysiology study (EPS). PPM is indicated if high-degree atrioventricular block is inducible. Long term rhythm monitoring with implantable loop recorder (ILR) is recommended if EPS is negative. We evaluated adherence to these guidelines. METHODS: This is a single-center retrospective audit of adult patients with bifascicular block hospitalized for unexplained syncope between January 2018 and August 2019 under general medicine service. Patients with an alternative explanation for syncope were excluded. Guideline adherence was assessed by formal cardiology consult and whether EPS followed by ILR and/or PPM were offered. RESULTS: 65 out of 580 adult patients (11.2%) admitted to general medicine service for syncope had a bifascicular block; 29 (5%) were identified to have bifascicular block and unexplained syncope. Median age was 77 ±10 years; 9 (31%) were female, and 6 (20.7%) patients had at least one prior hospital visit for syncope at our academic medical center. Cardiology was consulted on 17 (58.6%) patients. Two patients were evaluated by EPS (1 refused) followed by ILR. Overall, 3 out of 29 patients (10.3%) received guideline-directed evaluation during the hospitalization based on ESC guidelines. None of the patients received empiric PPM during the index hospitalization. CONCLUSION: Among patients admitted to the general medicine service with unexplained syncope and bifascicular block, a minority (10.3%) underwent guideline-directed evaluation per ESC recommendations. Cardiology was consulted in 58.6% of cases.
Subject(s)
Clinical Audit , Guideline Adherence , Heart Block , Syncope , Aged , Aged, 80 and over , Female , Heart Block/physiopathology , Heart Block/therapy , Humans , Male , Middle Aged , Retrospective Studies , Syncope/etiology , Syncope/physiopathology , Syncope/therapyABSTRACT
A 43-year-old man with history of non-Hodgkins' lymphoma presented with unilateral eye swelling, pain and vision deficits which had been progressive over 2 months. Symptoms followed a presumed bacterial pneumonia 4 months prior. Imaging demonstrated retro-orbital soft tissue swelling with bony erosion concerning for a mass; surgical decompression was performed with histology confirming disseminated Blastomyces dermatitidis Symptoms responded rapidly to antifungal therapy with amphotericin followed by itraconazole. Orbital dissemination of blastomycosis is extremely rare; accurate diagnosis requires tissue biopsy to facilitate timely targeted therapy and minimise morbidity.
