IS Case 335: Alveolar hemorrhage secondary to systemic lupus erythematosus

Keith Forrest Dockery, MD

Imaging Sciences URMC 2009
Publication Date: 2009-09-15

History

Patient is a 33-year-old female with systemic lupus erythematosus (SLE) who presented to the emergency department (ED) with fever, cough, hemoptysis, anemia (HCT 1), thrombocytopenia (84K), acute renal failure, and clinical question of infection or lupus pneumonitis. She had recently self-discontinued steroid therapy for SLE.

Her hospital stay was complicated. A renal biopsy revealed mild mesangial proliferative lupus nephritis with superimposed focal ANCA-associated cresenteric glomerulonephritis and acute tubular necrosis.

Findings

Plain film of the chest taken while in the ED (Fig. 1) demonstrated diffuse bilateral airspace disease, predominately central, with relative peripheral clearing, which could represent atypical infection such as with fungal organisms versus edema, hemorrhage, or pneumonitis. A CT scan (Fig. 2) revealed bilateral, diffuse, asymmetric (right > left) alveolar opacification, with peripheral clearing. The appearance favored alveolar hemorrhage or atypical infection, such as viral or fungal etiology, versus lupus pneumonitis. The findings are not sufficiently specific to exclude a combination of infection and hemorrhage.

Post-treatment plain film of the chest one month later (Fig. 3) demonstrated near-complete resolution of pulmonary infiltrates, compatible with symptomatic recovery.

Diagnosis

Alveolar hemorrhage secondary to systemic lupus erythematosus

Discussion

The patient’s discharge diagnosis was alveolar hemorrhage secondary to systemic lupus erythematosus (SLE) flare. Alveolar hemorrhage secondary to rheumatologic etiologies is most frequent in SLE and systemic vasculitides. Alveolar hemorrhage may occur in patients with mild or aggressive forms of SLE. The frequency of alveolar hemorrhage in SLE is ~2-5% and it accounts for ~2-3% of hospital admissions: mortality ranges from ~20-90%, though survival is usually >50%. Fatal decline can be rapid, on the order of days from presentation. Therefore, rapid diagnosis and treatment is important.

The pleuro-pulmonary manifestations of SLE [4] are as follows:

1. Lupus pneumonitis

2. Lymphocytic interstitial pneumonia

3. Pulmonary hemorrhage

4. Pulmonary embolism associated with lupus anticoagulant

5. Pulmonary hypertension

6. Lupus pleuritis

7. Weakness of the diaphragm.

This patient was negative for serum lupus anticoagulant and negative for leukocytosis at presentation. No lymphadenopathy was identified on CT. The clinical presentation of alveolar hemorrhage may mimic primary pulmonary hemosiderosis, Goodpasture’s syndrome, or Wegener’s granulomatosis. Anti-GBM antibody was not mentioned in the clinical notes and the patient was p-ANCA positive, not c-ANCA positive. Bronchoaveolar lavage was not performed in this case, but can be helpful and would have been expected to reveal classic findings of blood and hemosiderin-containing macrophages.

Fever is present in 82% (other studies range from 83-100%) of patients with alveolar hemorrhage, but only 54% (other studies range from 25-100%) exhibit hemoptysis: our patient had both. Acute anemia ranges from 75-100% at presentation, determined as a rapid drop in hemoglobin: HCT was 16 at presentation in the current case.

The most common imaging pattern seen in one series was diffuse alveolar and interstitial infiltrative type at 82%, with 27% having pleural effusions. Infiltrates are typically perihilar or bibasilar. A minority had unilateral (lobar-type) findings in the same series.

Anemia and new pulmonary infiltrates are considered the most specific findings, while nonspecific findings include fever, cough, dyspnea, chest pain, and tachycardia.

References

  1. Haaga JR, Lanzieri CF, Gilkeson RC. Computed Tomography and Magnetic Resonance Imaging of the Whole Body. Vol 1, 4th ed., Mosby, 2003.
  2. Koh JH, Young S, Lee CK, et al. A case of diffuse alveolar hemorrhage in systemic lupus erythematosus: treatment with plasmapharesis. J Korean Rheum Assoc. 199 Jun;6(2): 185-191. [KoreaMed]
  3. Nguyen T, Martin MK, Indrikovs AJ. Plasmapheresis for diffuse alveolar hemorrhage in a patient with Wegener's granulomatosis: Case report and review of the literature. J Clin Apher. 2005 Dec;20(4):230-4. PMID: 16265628
  4. Pines A, Kaplinsky N, Olchovsky D, Rozenman J, Frankl O. Pleuro-pulmonary manifestations of systemic lupus erythematosus: clinical features of its subgroups. Prognostic and therapeutic implications. Chest. 1985 Jul;88(1):129-35. PMID: 3924488
  5. Santos-Ocampo AS, Mandell BF, Fessler BJ. Alveolar hemorrhage in systemic lupus erythematosus: presentation and management. Chest. 2000 Oct;118(4): 1083-90. PMID: 11035681

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