Abstract
Diffuse alveolar hemorrhage (DAH) is a life-threatening clinicopathologic condition caused by accumulation of intra-alveolar red blood cells (RBCs) after disruption of the alveolar-capillary basement membrane that is often seen as a complication of various diseases, but is rare in systemic sclerosis. A 46-year-old female with systemic sclerosis presented to the emergency department complaining of right-sided chest pain. Initially, her electrocardiogram and chest X-ray (CXR) were unremarkable; however, she progressively decompensated into acute respiratory failure resulting in intubation. Repeat CXR and computed tomography scan showed diffuse bilateral alveolar infiltrates and pleural effusions. Video bronchoscopy with bronchoalveolar lavage showed numerous RBCs, neutrophils, macrophages, and respiratory epithelial cells consistent with acute DAH. She was started on intravenous pulse-dosing Solu-Medrol 1 g daily for 5 days. One month later, the patient returned with intractable nausea and vomiting. Again, she went into acute respiratory distress with a PaO2 of 59 while on a 10-L non-rebreather mask. CXR revealed development of alveolar infiltrates in the right lung. A bronchoscopy with bronchoalveolar lavage again showed numerous RBCs and neutrophils along with staining positive for hemosiderin-laden macrophages. Systemic sclerosis with alveolar hemorrhage is a rare occurrence; however, most cases are single episodes of hemorrhage, whereas we present a case with 2 confirmed episodes within 30 days. Its life-threatening nature makes a systemic approach and aggressive treatment crucial to decreasing morbidity and mortality—making it a diagnosis that should not be overlooked, especially in patients with nonspecific symptoms.
Highlights
Diffuse alveolar hemorrhage (DAH) is a life-threatening clinicopathologic condition that results from an accumulation of intra-alveolar red blood cells after the disruption of the alveolar-capillary basement membrane
We discuss a patient with known overlap syndrome, consisting of systemic sclerosis and dermatomyositis, who presented with 2 consecutive episodes of DAH within 30 days
Diffuse alveolar hemorrhage can have a myriad of underlying etiologies including systemic vasculitides, rheumatic disease, anti-glomerular basement membrane disease, idiopathic pulmonary hemosiderosis, and systemic lupus erythematosus (SLE); reports of DAH secondary to systemic sclerosis with alveolar hemorrhage are rare since the first description by Kallenbach et al in 1977.2 All of the previous reports encased a single episode of hemorrhage, whereas here we present a case with confirmed 2 episodes of DAH within 30 days
Summary
Diffuse alveolar hemorrhage (DAH) is a life-threatening clinicopathologic condition that results from an accumulation of intra-alveolar red blood cells after the disruption of the alveolar-capillary basement membrane. Physical examination demonstrated multiple cushingoid features secondary to her chronic steroid use, along with digit contractures and shiny, tight-appearing digits On admission, her electrocardiogram and chest X-ray were unremarkable; the patient progressively decompensated and developed intractable nausea and vomiting. It was discovered that she had failed to start the outpatient IVIG therapy During this admission, similar to the previous admission, the patient went into acute respiratory distress with a PaO of 59 on an arterial blood gas while on a. A bronchoscopy with BAL again showed numerous red blood cells and neutrophils along with staining positive for hemosiderin-laden macrophages, confirming her second episode of DAH She was started on IVIG inpatient for her scleroderma crisis, which had to be discontinued after she developed tonic-clonic seizures shortly after the treatment. The patient has returned to the ED multiple times for cardiac and recurrent pyelonephritis episodes, but has not had a recurrent episode of DAH again
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