lupus with DAH

In this case, the patient presents with diffuse alveolar hemorrhage (DAH), most likely associated with a new diagnosis of systemic lupus erythematosus (SLE).

SLE is an autoimmune disorder of unknown causes that leads to the immune system attacking a variety of organs. SLE more commonly manifests in women between the ages of 20 to 40 years with a constellation of symptoms, including fatigue, joint pain and swelling, characteristic malar rash, pneumonitis, and glomerulonephritis. The patient in this question is presenting with multiple signs and symptoms associated with SLE, including anemia, pneumonitis, a urinalysis result suggestive of glomerulonephritis, and arthritis. The diagnosis of SLE would be confirmed by checking antinuclear antibodies, anti-double-stranded DNA, and antiphospholipid antibodies and performing serum protein electrophoresis.

On CT scans, the typical patterns of DAH are focal or diffuse areas of ground-glass opacity or consolidation, which are seen on this patient’s scans (Figures 1 and 2). For this patient with a high suspicion for SLE and concomitant DAH, the most appropriate next step among the options provided is bronchoscopy and BAL. After ruling out infection, the first-line therapy for DAH would be to start high-dose immunosuppressive therapy. If there is a lack of response to high-dose immunosuppression, this could indicate idiopathic DAH or another, nonimmune-mediated cause.

Heart catheterization helps to evaluate for coronary artery disease and measure hemodynamics of the left and right sides of the heart. In this case, the patient presents primarily with single organ failure (the lungs), is hemodynamically stable, and has a recent echocardiogram showing preserved function without significant wall motion or valvular disease. It is more likely that her hemoptysis is secondary to infection or inflammatory disorder than to heart failure. There is no indication for leftor right-sided heart catheterization at this time.

Surgical (or “open”) lung biopsy is indicated in idiopathic causes of interstitial lung disease for which infection, exposures, and immune-related causes have already been ruled out. That is not the case in this question, and bronchoscopy with BAL is more appropriate at this time.

Whole lung lavage does not have a clear role in diagnosis or treatment of DAH. Whole lung lavage does have a role in the treatment of protein alveolar proteinosis (PAP), a diffuse lung disease characterized by lipoproteinaceous material, which accumulates in the distal air spaces. Whole lung lavage, which often requires multiple liters of lavage for each lung, washes out this periodic acid-Schiff (PAS)-positive lipoproteinaceous material and allows air exchange across the alveoli. PAP is caused by reduced granulocyte-macrophage colony-stimulating factor levels, which is related to tobacco smoke, infections, or autoimmune disease. Patient clinical presentation is usually progressive shortness of breath with a nonproductive cough, no hemoptysis, and no extrapulmonary symptoms. Chest CT scans typically demonstrate ground-glass opacification in a homogeneous distribution (reverse batwing). Bronchoscopy is performed with BAL to rule out infection (such as Pneumocystis jirovecii, Nocardia, Mycobacteria, and fungi) and a characteristic milky appearance demonstrating PAS-positive macrophages at cytological examination. Treatment of PAS consists of avoiding toxins (tobacco smoke); treating any underlying infections; and, in autoimmune PAP-associated with moderate to severe lung disease, whole lung lavage. The patient in this question presents with a clinical picture suggestive of SLE and DAH, and evaluating for DAH and ruling out infectious causes of her acute hypoxemic respiratory failure should be the first priority via bronchoscopy and BAL.1234

Footnotes

  1. SEEK Questionnaires ↩

  2. Ameer MA, Chaudhry H, Mushtaq J, et al. An overview of systemic lupus erythematosus (SLE) pathogenesis, classification, and management. Cureus. 2022;14(10):e30330. PubMed ↩

  3. Medlin JL, Hansen KE, McCoy SS, et al. Pulmonary manifestations in late versus early systemic lupus erythematosus: a systematic review and meta-analysis. Semin Arthritis Rheum. 2018;48(2):198-204. PubMed ↩

  4. Park JA. Treatment of diffuse alveolar hemorrhage: controlling inflammation and obtaining rapid and effective hemostasis. Int J Mol Sci. 2021;22(2):793. PubMed ↩