Pulmonary Manifestations of Systemic Disease: Fellow Case Report Poster - Pulmonary Manifestations of Lung Disease |

A Case of Submassive Hemoptysis and Rapidly Progressing Respiratory Failure FREE TO VIEW

Dhaval Thakkar, MD; James Dargin, MD; Timothy Liesching, MD
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Lahey Clinic, Waltham, MA

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):1050A. doi:10.1016/j.chest.2016.08.1157
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SESSION TITLE: Fellow Case Report Poster - Pulmonary Manifestations of Lung Disease

SESSION TYPE: Affiliate Case Report Poster

PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM

INTRODUCTION: Diffuse alveolar hemorrhage (DAH) is a rare (incidence of 2-5%), but potentially lethal complication in systemic lupus erythematosus (SLE) with a mortality rate of 40-92%. Due to the rarity of this condition, treatment is based on case series and reports. Here we discuss the successful treatment of a patient with DAH and severe hypoxemia as the initial presentation of SLE.

CASE PRESENTATION: A 43 year-old female with no significant past medical history presented with sudden onset of submassive hemoptysis and dyspnea. Exam revealed a room air oxygen saturation of 85% and diffuse inspiratory crackles. Chest radiograph showed bilateral lower lobe infiltrates (Figure 1). Her condition rapidly deteriorated and she required intubation for severe hypoxemia. Lung protective ventilation was initiated and the PaO2 was 167 despite an FiO2 of 1.0 and PEEP of 18 cmH2O. Sequential lavage during bronchoscopy confirmed DAH. Serological studies, anti-DNA, anti-Smith, SSA, RNP, SMRNP complex, Anti-chromatin, and SCL-70 returned positive for a pattern suggesting SLE. She was treated with pulse methylprednisolone 1 gram daily for 2 days and tapered down to prednisone 60mg daily, cyclophosphamide 1580 mg for one dose, and five sessions of plasmapharesis. Her clinical condition and chest radiograph rapidly improved (Figure 2) and the patient was extubated after six days and discharged home on hospital day sixteen.

DISCUSSION: DAH is a rare, but potentially lethal complication of SLE. The condition primarily affects young women, and up to 60% will require mechanical ventilation. When DAH is the first manifestation of SLE, the diagnosis and appropriate treatment may be delayed. Despite the rapid onset of severe hypoxemia in this case, prompt diagnosis and aggressive treatment with lung protective ventilation, high dose corticosteroids, cyclophosphamide, and plasmapharesis resulted in dramatic clinical improvement.

CONCLUSIONS: DAH is a rare, but potentially life-threatening condition that may be the first manifestation of SLE. A high index of suspicion of SLE in young women with DAH and severe hypoxemia may help with rapid diagnosis and appropriate treatment.

Reference #1: Zamora et al. Diffuse Alveolar Hemorrhage and Systemic Lupus Erythematosus. Medicine 1997; 76(3): 192-202.

Reference #2: Schwab et al. Pulmonary Alveolar Hemorrhage in Systemic Lupus Erythematosus. Seminars in Arthritis and Rheumatism 1993; 123(1):8-15.

Reference #3: Badsha et al. Pulmonary Hemorrhage in Systemic Lupus Erythematosus. Seminars in Arthritis and Rheumatism 2003; 33(6): 414-421.

DISCLOSURE: The following authors have nothing to disclose: Dhaval Thakkar, James Dargin, Timothy Liesching

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