A 32 year-old African American man with a history of asthma was admitted to the ICU with acute respiratory failure due to diffuse alveolar hemorrhage (DAH), diagnosed by bronchoscopy, requiring intubation and blood transfusions. Course was complicated by development of a pulmonary embolism (PE) and placement of IVC filter. He was discharged on prednisone with a diagnosis of idiopathic pulmonary hemosiderosis (IPH) based on lung biopsy.
Follow-up visit revealed his mother has rheumatoid arthritis and maternal aunt has SLE. Labs revealed anemia, lymphopenia, proteinuria with cellular casts, and +ANA (1:320). He had a negative dsDNA, Viper venom, anti-Sm, Lupus anticoagulant anti-phospholipid and anti-cardiolipin. He also was negative for anti-GBM, RF, ANCA, anti-TTG and anti-CCP. He established care with rheumatology and pulmonology, developed significant weight gain and required hospitalization for episodic hemoptysis associated with steroid tapers. He developed bilateral unprovoked deep venous thromboses in the upper extremities.
Six months later, he developed pleuritis establishing the diagnosis of systemic lupus erythematous. Treatment with hydroxychloroquine and azathioprine achieved remission of symptoms, and prednisone was tapered to 5 mg daily with associated fifty-pound weight loss and improved functional status.
Hemoptysis is a condition seen by hospitalists. Evaluation should include pulmonary causes, discriminating between alveolar, bronchial and capillary sources. DAH requires bronchoscopy for diagnosis, as the clinical picture of hemoptysis, anemia and diffuse radiographic pulmonary infiltrates is non-specific. Possible etiologies include immunologic (SLE, Goodpasture’s syndrome, vasculitis) and non-immunologic (IPH, coagulopathy, necrotizing pulmonary infections, malignancy, drugs). Serologic testing is necessary to distinguish these etiologies, and more than one process may be present, such as IPH and SLE in this patient.
Pulmonary manifestations occur in the majority of patients with SLE, especially men, ranging from mild asymptomatic pleuritis to severe and often fatal DAH. It is important to assess for SLE in patients with DAH, as SLE with pulmonary involvement is associated with increased mortality, and the patient should be started on high-dose corticosteroids and potentially immunologics as soon as possible.
SLE is a multi-organ disease requiring identification of 4 of 11 possible characteristics defined by the American College of Rheumatology for diagnosis. These criteria do not have to be present at the same time and often are identified over the course of months to years. Of the serologic markers, ANA has a high sensitivity and therefore its absence makes SLE less likely, but dsDNA and anti-Sm Ab have high specificity and thus do not rule out SLE in their absence. When less than 4 criteria have been met but there is clinical suspicion for SLE, the patient should be carefully monitored by evaluation of symptoms, physical exam findings and laboratory values at subsequent visits in order to obtain early control of arising symptoms and to prevent long-term organ damage.
Diffuse alveolar hemorrhage may be due to immunologic and non-immunologic conditions. Serologic evaluation in combination with other abnormal clinical or laboratory findings may lead to a diagnosis of an underlying autoimmune disease.
To cite this abstract:Sherrill M, Thangudu P. Sle or Not Sle? That Is the Question!. Abstract published at Hospital Medicine 2015, March 29-April 1, National Harbor, Md. Abstract 687. Journal of Hospital Medicine. 2015; 10 (suppl 2). https://www.shmabstracts.com/abstract/sle-or-not-sle-that-is-the-question/. Accessed July 23, 2019.