RESUMEN
Anti-melanoma differentiation-associated gene 5 (MDA 5) is one of the subtypes of dermatomyositis associated with rapidly progressive lung disease. MDA 5 carries a high mortality risk due to respiratory failure. The exact pathophysiology is unclear, but it is linked to genetic predisposition and viral triggers with the associated innate response and cytokine production like interleukins IL-1,6,18, tumor necrosis factor-alpha, and interferons. It is usually treated with anti-cytokines, high-dose steroids, immunosuppressants, and plasma exchange. Due to the atypical presentation and rapidity of the disease course, the diagnosis is often delayed. We report a 39-year-old female presenting with rapidly progressive lung disease secondary to an aggressive form of dermatomyositis.
RESUMEN
Hypertension causing thrombotic microangiopathy (TMA) is one of the several etiologies of TMA, which causes endothelial damage and thrombosis of microvasculature, leading to hemolytic anemia, thrombocytopenia, and ischemic organ damage. Renal microvasculature involvement leading to renal dysfunction is most frequently seen in TMA but the degree of dysfunction varies with etiology. If left untreated, TMA carries a risk of high mortality, so it is extremely important for early identification of the cause of TMA. Plasma exchange is a commonly used treatment modality for TMA; however, it is not always necessary. Hypertension-induced TMA can be safely treated with antihypertensives, without the need for invasive plasma exchange. We report a 37-year-old African American hypertensive lady presenting with hypertensive emergency and TMA with rapidly progressing renal dysfunction. The patient had improvement in her platelet count after her blood pressure was reduced in a controlled manner.
