Clinical characteristics and outcomes of diffuse alveolar hemorrhage in patients with systemic lupus erythematosus

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Abstract

Objective

To identify the clinical characteristics of diffuse alveolar hemorrhage (DAH) compared to other types of acute diffuse lung infiltration in SLE patients, and the factors associated with mortality in these patients.

Methods

We studied a retrospective cohort including SLE patients with acute diffuse lung infiltration on thoracic CT between January 2004 and August 2014. We divided them into 2 groups, a DAH and a non-DAH group, and compared the clinical characteristics and outcomes in the 2 groups. We also evaluated the risk factors for mortality in SLE patients with diffuse lung infiltration.

Results

Of 47 patients with diffuse lung infiltration, 24 patients (51.1%) satisfied the criteria for DAH and the remaining 23 patients (48.9%) were assigned to the non-DAH group. There were no significant differences between the demographic features of the 2 groups. However, decreased hemoglobin (OR = 3.46; 95% CI: 1.38–8.67; p < 0.01) and C4 (OR = 1.21; 95% CI: 1.03–1.42; p = 0.02) levels, and presence of hypoxia (OR = 23.09; 95% CI: 1.47–365.34; p = 0.03) at the time of diagnosis were associated with SLE-DAH. In addition, severe conditions requiring mechanical ventilation (OR = 64.61; 95% CI: 1.98–2112.02; p = 0.02) were associated with increased mortality, whereas DAH did not increase mortality compared with non-DAH in SLE patients with diffuse lung infiltration.

Conclusions

In SLE patients with acute diffuse lung infiltration, it is important to promptly evaluate the DAH when patients have low levels of hemoglobin or C4, and symptoms of hypoxia. Mortality is associated with severe conditions requiring mechanical ventilation rather than with DAH in patients with diffuse lung infiltration.

Introduction

Acute diffuse alveolar infiltration, a rare manifestation of systemic lupus erythematosus (SLE), is caused by various life-threatening conditions such as acute pneumonitis, diffuse lung infection, and diffuse alveolar hemorrhage (DAH) [1], [2], [3]. DAH is a rare but life-threatening complication of SLE [4]. In previous reports, the incidence rate of DAH in SLE patients varied from 0.5% to 5.4%, and the mortality rate varied from 0% to 95% [5], [6], [7], [8], [9], [10], [11], [12], [13].

DAH is characterized by acute diffuse lung infiltration on chest radiographic images, an abrupt drop of hemoglobin level and non-specific respiratory symptoms such as hemoptysis, dyspnea, cough, sputum, and hypoxia. Since the treatments for DAH and the other causes of acute diffuse alveolar infiltration are completely different, early recognition and prompt and adequate treatment offer the best chance of improving the survival of SLE-associated DAH (SLE-DAH) [10]; however, it is difficult to diagnose DAH early because of its abrupt onset and rapid progression, and the non-specific clinical symptoms and radiographic findings.

For early recognition of DAH as the cause of acute diffuse lung infiltration, it is important to identify defining clinical features. However, the clinical symptoms indicating DAH, such as cough and sputum are non-specific and can be present in many respiratory disorders; in addition, symptoms such as dyspnea, hypoxia, and hemoptysis can also develop in severe pneumonitis or lung infections. Moreover, the imaging findings in DAH-SLE are quite similar to those in other types of acute diffuse lung infiltration, and an abrupt drop in hemoglobin level can also occur in lupus flare or hemorrhages at other sites [14].

Thus, for early differential diagnosis of DAH in SLE patients we require more information. In addition, to improve outcomes the clinical characteristics associated with mortality in these patients need to be investigated. In this study, we aimed to identify the specific clinical features of DAH compared with other conditions leading to acute diffuse lung infiltration in SLE patients, and the factors associated with mortality in these patients.

Section snippets

Patient selection

The retrospective cohort of 47 patients with acute diffuse lung infiltration was selected from a total of 815 SLE patients who underwent thoracic CT scans between January 2004 and August 2014 in a single academic hospital. We classified these 47 patients into 2 groups as a DAH group and a non-DAH group. Inclusion criteria for the DAH group were (1) acute diffuse lung infiltration on thoracic CT scans, characterized by patchy ground-glass opacities without significant interlobular septal

Demographic, clinical features, and mortality of SLE-DAH patients

Of the 47 SLE patients with acute diffuse lung infiltration in thoracic CT scans, 24 patients (51%) satisfied the DAH criteria of this study. Hence, the rest of the 23 patients were classified as non-DAH patients (49%). The non-DAH group consisted of infectious conditions (n = 14) including cytomegalovirus (CMV), pneumocystis, influenza mycoplasma and non-specific pneumonia, and non-infectious conditions (n = 9) such as pneumonitis, interstitial lung disease, serositis, pulmonary edema, and

Discussion

Diffuse alveolar hemorrhage is a rare but catastrophic complication of SLE. The incidence of DAH ranges from 0.5% to 5.4% in the reported case series [5], [6], [8]. We assumed that the incidence of DAH among SLE patients might depend on the definition of DAH. The definition used in previous studies was not consistent, especially with regard to the decline in Hb level: the definition employed was usually 1–2 mg/dl but some studies did not have a fixed criterion for the reduction [5], [6], [8]. In

Conclusions

In SLE patients with acute diffuse lung infiltration, prompt evaluation of the DAH is needed when patients have low hemoglobin level, C4 level, and symptoms of hypoxia. Mortality is associated with severe conditions requiring mechanical ventilation rather than with DAH in patients with diffuse lung infiltration.

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