A RARE MANIFESTATION OF SYSTEMIC LUPUS ERYTHEMATOSUS (SLE): DIFFUSE ALVEOLAR HEMORRHAGE

Abstract

SESSION TITLE: Autoimmune Diseases Gone Wild: Rare Cases of Pulmonary ManifestationsSESSION TYPE: Rapid Fire Case ReportsPRESENTED ON: 10/18/2022 01:35 pm - 02:35 pmINTRODUCTION: Systemic lupus erythematosus (SLE) is an autoimmune multisystemic disease that can involve the lungs in up to 50-70% of cases, which include pleuritis, obliterative bronchiolitis, acute or chronic lupus pneumonitis, interstitial lung disease, diffuse alveolar hemorrhage (DAH), and other infrequent manifestations. Here we report a case of DAH associated with SLE.CASE PRESENTATION: A 39 -year-old female with Multiple Sclerosis and epilepsy presented a persistent fever of 105.1°F. Physical examination was significant for diffuse alopecia and inspiratory crackles in the lower lungs. Chest Xray revealed new bilateral patchy opacities and CT Angiogram of the chest revealed bilateral patchy ground-glass opacities. She was progressively hypoxemic and required intubation due to hypoxic and hypercapnic respiratory failure; endotracheal aspirates were blood tinged. Labs revealed a WBC of 3.9, Hb of 8.2 (from 10.0), platelets of 91, ANA 1:2560 with speckled pattern, Anti-RNP positive, and low complement levels. Due concerns for DAH in newly diagnosed SLE, a bronchoscopy was performed revealing bleeding without a definable source, persistent bloody sequential lavage and negative microbiology consistent with DAH. The patient was started on a 3-day course of IV 1 g methylprednisolone and Rituximab (RTX). She was weaned off of vasopressors, steroids were tapered, and she was extubated after nine days. Lung infiltrates resolved after 2 weeks of treatment.DISCUSSION: DAH is a rare, but devastating manifestation of SLE. Suggested pathophysiology is immune-mediated damage of the small blood vessels and alveolar spaces with histopathological findings of immune complex deposition that results in alveolar-capillary damage. The clinical picture of DAH is characterized by hemoptysis, an acute drop in hemoglobin, hypoxemic respiratory failure and diffuse pulmonary infiltrates. As seen with our patient, these nonspecific findings prove a diagnostic challenge and make it necessary to rule out infections, drug toxicity, pulmonary embolism and heart failure. A combination of careful history, physical examination, chest imaging, and bronchoscopy will provide a diagnosis of DAH. High-dose steroids and immunosuppression including cyclophosphamide, RTX, plasmapheresis, mycophenolate, azathioprine, and IVIG are current treatment options. Infections can trigger DAH therefore broad-spectrum antibiotics should be also considered if an infection is suspected.CONCLUSIONS: DAH should be included in the differential diagnosis for patients with lung parenchymal involvements. The mortality of DAH associated with SLE is high despite available treatments, therefore early diagnosis and prompt treatment are crucial. The mainstay of treatment is early administration of high-dose steroid, corticosteroid-sparing agents and supportive care.Reference #1: Aguilera-Pickens, G., Abud-Mendoza, C. Pulmonary manifestations in systemic lupus erythematosus: pleural involvement, acute pneumonitis, chronic interstitial lung disease and diffuse alveolar hemorrhage. Reumatologia Clinica (Engl Ed), (2018). 14(5):294-300. Doi:10.1016/j.reumae.201Reference #2: Virdi, R.P.S., Bashir, A., Shahzad, G., et al. Diffuse alveolar hemorrhage: a rare life-threatening condition in systemic lupus erythematosus. Case Reports in Pulmonology, (2012). 2012:836017-4. doi:10.1155/2012/836017.Reference #3: Al-Adhoubi, N. K., Bystrom, J. Systemic lupus erythematosus and diffuse alveolar hemorrhage, etiology and novel treatment strategies. Lupus, (2020). 29(4):355-363. Doi:10.1177/09612033209DISCLOSURES: No relevant relationships by Jonathan Ariyaratnamno disclosure on file for Carlos gonzalez;No relevant relationships by Ricardo LopezNo relevant relationships by Jasmine Sandhuno disclosure on file for Bahtiyar Toz; SESSION TITLE: Autoimmune Diseases Gone Wild: Rare Cases of Pulmonary Manifestations SESSION TYPE: Rapid Fire Case Reports PRESENTED ON: 10/18/2022 01:35 pm - 02:35 pm INTRODUCTION: Systemic lupus erythematosus (SLE) is an autoimmune multisystemic disease that can involve the lungs in up to 50-70% of cases, which include pleuritis, obliterative bronchiolitis, acute or chronic lupus pneumonitis, interstitial lung disease, diffuse alveolar hemorrhage (DAH), and other infrequent manifestations. Here we report a case of DAH associated with SLE. CASE PRESENTATION: A 39 -year-old female with Multiple Sclerosis and epilepsy presented a persistent fever of 105.1°F. Physical examination was significant for diffuse alopecia and inspiratory crackles in the lower lungs. Chest Xray revealed new bilateral patchy opacities and CT Angiogram of the chest revealed bilateral patchy ground-glass opacities. She was progressively hypoxemic and required intubation due to hypoxic and hypercapnic respiratory failure; endotracheal aspirates were blood tinged. Labs revealed a WBC of 3.9, Hb of 8.2 (from 10.0), platelets of 91, ANA 1:2560 with speckled pattern, Anti-RNP positive, and low complement levels. Due concerns for DAH in newly diagnosed SLE, a bronchoscopy was performed revealing bleeding without a definable source, persistent bloody sequential lavage and negative microbiology consistent with DAH. The patient was started on a 3-day course of IV 1 g methylprednisolone and Rituximab (RTX). She was weaned off of vasopressors, steroids were tapered, and she was extubated after nine days. Lung infiltrates resolved after 2 weeks of treatment. DISCUSSION: DAH is a rare, but devastating manifestation of SLE. Suggested pathophysiology is immune-mediated damage of the small blood vessels and alveolar spaces with histopathological findings of immune complex deposition that results in alveolar-capillary damage. The clinical picture of DAH is characterized by hemoptysis, an acute drop in hemoglobin, hypoxemic respiratory failure and diffuse pulmonary infiltrates. As seen with our patient, these nonspecific findings prove a diagnostic challenge and make it necessary to rule out infections, drug toxicity, pulmonary embolism and heart failure. A combination of careful history, physical examination, chest imaging, and bronchoscopy will provide a diagnosis of DAH. High-dose steroids and immunosuppression including cyclophosphamide, RTX, plasmapheresis, mycophenolate, azathioprine, and IVIG are current treatment options. Infections can trigger DAH therefore broad-spectrum antibiotics should be also considered if an infection is suspected. CONCLUSIONS: DAH should be included in the differential diagnosis for patients with lung parenchymal involvements. The mortality of DAH associated with SLE is high despite available treatments, therefore early diagnosis and prompt treatment are crucial. The mainstay of treatment is early administration of high-dose steroid, corticosteroid-sparing agents and supportive care. Reference #1: Aguilera-Pickens, G., Abud-Mendoza, C. Pulmonary manifestations in systemic lupus erythematosus: pleural involvement, acute pneumonitis, chronic interstitial lung disease and diffuse alveolar hemorrhage. Reumatologia Clinica (Engl Ed), (2018). 14(5):294-300. Doi:10.1016/j.reumae.201 Reference #2: Virdi, R.P.S., Bashir, A., Shahzad, G., et al. Diffuse alveolar hemorrhage: a rare life-threatening condition in systemic lupus erythematosus. Case Reports in Pulmonology, (2012). 2012:836017-4. doi:10.1155/2012/836017. Reference #3: Al-Adhoubi, N. K., Bystrom, J. Systemic lupus erythematosus and diffuse alveolar hemorrhage, etiology and novel treatment strategies. Lupus, (2020). 29(4):355-363. Doi:10.1177/09612033209 DISCLOSURES: No relevant relationships by Jonathan Ariyaratnam no disclosure on file for Carlos gonzalez; No relevant relationships by Ricardo Lopez No relevant relationships by Jasmine Sandhu no disclosure on file for Bahtiyar Toz;