Chest
Volume 118, Issue 4, October 2000, Pages 1083-1090
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Clinical Investigations
DIFFUSE LUNG DISEASE
Alveolar Hemorrhage in Systemic Lupus Erythematosus: Presentation and Management

https://doi.org/10.1378/chest.118.4.1083Get rights and content

Aim:

To describe our experience with alveolarhemorrhage (AH) in systemic lupus erythematosus (SLE).

Methods:

Review of medical records and pertinent medicalliterature using MEDLINE and reference lists from retrievedpublications.

Patients:

Seven patients with SLEadmitted with episodes of AH (n = 11).

Results:

Sixpatients were female, and one was male. Mean age at the time of AH was31.1 years. Mean duration of SLE was 4.5 years. AH occurred within 3weeks of SLE onset in two patients. Recurrent AH was observed in fourpatients. Six patients were already receiving treatment for SLE at thetime of AH. All patients presented with dyspnea and new pulmonaryinfiltrates. Hemoptysis occurred in only 54%. All patients had BALwithin 48 h of presentation. Temperature ≥39°C(102.2°F) accompanied 82% of episodes. Glomerulonephritis wasthe most common nonpulmonary SLE manifestation (74%). Treatment withempiric IV antibiotics was initiated in 10 episodes. Initial treatmentincluded high-dose corticosteroids (prednisone, 1 to 3 mg/kg/d[n = 2]; or IV methylprednisolone, 1 g/d [n = 9], with orwithout oral cyclophosphamide, 2 to 3 mg/kg/d [n = 7]). Plasmapheresis (three to four sessions) was added in five episodes forpersistent AH. All patients survived.

Conclusions:

AHmay mimic pneumonia. Hemoptysis may not be evident. Infection must beaggressively excluded, especially since many patients with AH are already receiving immunosuppressive therapy. AH frequentlyrecurs despite ongoing immunosuppression. Although high mortality rateshave been reported with AH in SLE, we observed 100%survival.

Section snippets

Materials and Methods

We performed a retrospective review of the medical records of patients meeting the preliminary inclusion criteria. Cases of AHconfirmed by demonstration of a bloody return on BAL withhemosiderin-containing macrophages were selected.

It was predefined that patients would be excluded if they haddrug-induced lupus, coagulopathies (international normalized ratio>3.5 s in patients receiving warfarin, severe chronic liverdisease, end-stage renal disease receiving dialysis), pulmonary edemafrom

Results

Between January 1993 and July 1998, seven patients with 11episodes of pulmonary hemorrhage met criteria for inclusion. These 7patients represented 1.0% (7 of 663) of patients with SLE admitted orreferred to the Cleveland Clinic Foundation with a listed diagnosis of SLE. Eight of 11 episodes occurred in the spring (n = 4) and summer(n = 4). There was no statistically significant pattern of seasonaloccurrence.

Discussion

Tables 2, 3summarize the patient characteristics, clinical presentations, treatments, and outcomes from this series, compared with data frompreviously published reports. A central theme in the previouslypublished literature is the high mortality rate seen with AH from SLE. Our observed patient survival of 100% is in marked contrast to mostseries, but reinforces the findings by Schwab et al,8 thatAH is a survivable complication of SLE.

Similar to most reports, most of our patients were young

Conclusion

AH is a serious manifestation of pulmonary SLE that may occurearly or late in disease evolution. It is survivable, despite itsfrequently dramatic presentation. Extrapulmonary disease may be minimaland may be masked in patients who are already receivingimmunosuppression for other symptoms of SLE. Predictors of patients atrisk for this complication are unclear at this time. The capacity of AHto occur and recur despite ongoing immunosuppressive therapy isemphasized in this series. AH may present

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