There is a scarceness of information on the central nervous system effects of common variable immunodeficiency (CVID). A 30-year-old woman with a history of recurrent upper respiratory infections, vitiligo, and immune thrombocytopenic purpura presented with right-sided numbness. Magnetic resonance imaging (MRI) of the thoracic spine revealed a signal hyperintensity. MRI of the brain demonstrated FLAIR hyperintensity in the right middle frontal gyrus. Cerebral spinal fluid was unremarkable. Serum immunoglobulins revealed hypogammaglobulinemia. Endobronchial and subsequent mediastinum biopsies were all negative for sarcoidosis and malignancy. No infectious etiology was found. She was treated with glucocorticoids and intravenous immunoglobulin (IVIG) replacement therapy for CVID-associated myelitis. Follow-up MRI showed improvement; however, her numbness persisted despite these treatments, which led to an outside physician adding methotrexate for their suspicion of sarcoidosis. Her symptoms remained stable for two years, but when the methotrexate dose was weaned, her numbness worsened. Upon review, the treatment team refuted the diagnosis of sarcoidosis but continued treatment with prednisone, IVIG, and methotrexate for CVID-associated myelitis, from which her symptoms have stabilized. Here, we discuss CVID-associated neurological complications, its similarities to sarcoidosis, and a literature review with treatment regimens and outcomes.
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are small-to-medium-vessel vasculitis, which includes granulomatosis with polyangiitis, microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis. MPA predominantly affects the kidneys and lungs. Subarachnoid hemorrhage (SAH), a life-threatening condition, rarely occurs with AAV. In this case, we present a 67-year-old female who presented with a sudden-onset headache after a recent diagnosis of ANCA-associated renal vasculitis. Kidney biopsy revealed pauci-immune glomerulonephritis, and serum was positive for ANCA along with myeloperoxidase antibody. A computed tomography scan of the head revealed both SAH and intraparenchymal hemorrhage. The patient was managed medically for SAH and intraparenchymal hemorrhage. ANCA vasculitis was treated with steroids and rituximab, and the patient showed improvement.
Granulomatosis with polyangiitis (GPA) is an autoimmune, necrotizing granulomatous disease that affects small- and medium-sized blood vessels. Aspergilloma is a fungal mass of Aspergillus and usually found in the preexisting cavity in lung parenchyma. Surgical resection is the mainstay of treatment of aspergilloma. In this article, we present a case of a 70-year-old male with GPA and aspergilloma who presented with massive, life-threatening hemoptysis. Further workup with a chest computed tomography (CT) pulmonary angiogram demonstrated a pulmonary artery pseudoaneurysm along the wall of the lung cavity which was emergently managed with embolization and required monitoring in the medical intensive care unit. This case report alerts clinicians to maintain a high level of suspicion for an aneurysm if the degree of hemoptysis is higher than expected.
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