Pulmonary hemorrhage in systemic lupus erythematosus

doi:10.1016/j.semarthrit.2003.09.006

Seminars in Arthritis and Rheumatism

Volume 33, Issue 6, June 2004, Pages 414-421

https://doi.org/10.1016/j.semarthrit.2003.09.006 Get rights and content

Abstract

Objective

To determine the clinical features of our systemic lupus erythematosus (SLE) patients presenting with pulmonary hemorrhage (PH).

Methods

We reviewed the records of all SLE patients who had PH between 1994 and 2001, a total of 22.

Results

All patients had radiographic infiltrates. The mean drop in hemoglobin was 3.2 ±1.1 g/dL, hemoptysis occurred in 50%, the Dlco was increased in 10 of 11 patients (91%), and 11 of 14 patients who underwent bronchoscopy had positive findings. All received high-dose prednisolone and most also were given pulse methylprednisolone (MEP) and cyclophosphamide. All patients required care in the intensive/monitored care unit, 14 were intubated, 11 had plasmapheresis, and 8 died as a result of PH (mortality rate, 36%). SLEDAI and SLAM were able to indicate active disease in SLE patients with PH (median SLEDAI, 19 [interquartile range, 10 to 24] and mean SLAM, 16.1 ± 5.8). There was a statistically significant increase in the SLEDAI from 1 month prior to PH to the time of PH (P = .014), indicating that the patients were having a significant disease flare. The median SLEDAI and SLAM scores of patients who died were slightly higher than that of survivors.

Conclusion

PH in SLE patients occurred in those with severe, multiorgan involvement, with high SLEDAI scores. A high degree of suspicion should be maintained in lupus patients with active disease and unexplained infiltrates on chest radiographs and dropping red cell indices, even in the absence of hemoptysis. Early aggressive management with high-dose steroids and intravenous pulses of cyclophosphamide is advocated and may explain recent trends of improved survival. Plasmapheresis may be useful for the acutely ill patient who does not respond to the above measures but does not clearly lead to improved survival.

Section snippets

Patient selection

Tan Tock Seng Hospital is a tertiary referral center for rheumatology in Singapore where 200 to 300 patients with SLE are admitted each year. The lupus cohort here has 1500 patients on follow-up. From our lupus database and from a search of in-patient medical records, we obtained the names of SLE patients admitted with PH from 1994 to 2002. A total of 23 names were obtained, but 1 patient was excluded because she did not have PH. All patients fulfilled the American College of Rheumatology (ACR)

Patient characteristics

PH in our group of SLE patients occurred in young patients (mean age, 31.6 ± 12.3 years) early in the course of disease (median duration of SLE was 0.96 years; IQR, 0 to 7 years) Table 1, Table 2, Table 3. Seven (32%) patients presented with PH as 1 of their disease manifestations at the onset of SLE and 55% had PH within the first year of SLE diagnosis. The patients were mostly female (91%) and Chinese (68%) other races were Malay (14%), Indian (9%), and Eurasian (9%).

Disease activity

At the time of PH,

Discussion

To our knowledge, this is the largest collection of SLE patients with PH reported. Our patients were young, with active SLE and multi-organ involvement.

Humeira Badsha, MD: Consultant, Department of Rheumatology, Allergy and Immunology, Tan Tock Seng Hospital, Singapore

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Pulmonary haemorrhage (PH) in systemic lupus erythematosus (SLE) is a rare but potentially fatal complication due to its high mortality. Early treatment benefits the outcome.
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To describe a case series of PH in SLE patients that were attended in the Rheumatology Section of the J. M. Cullen Hospital and to compare this data with published results.
Patients with SLE (1982-1997 ACR criteria) and PH diagnosed by clinical criteria (cough, dyspnoea, haemoptysis), haemoglobin below 12 g/dL or drop greater than 2 points, new radiological infiltrate and bronchioalveolar lavage, monitored between June 1987 and December 2019 were studied. Demographic, clinical, laboratory, treatment and prognosis data related to PH were analysed.
From a database of 306 SLE patients, 25 (8.2%) developed 29 episodes of PH. PH was the first manifestation of SLE in 8 patients. Renal involvement was the most frequent manifestation prior to the development of PH. SLE activity (measured by SLEDAI) was high during the episodes (mean: 16.8). Renal failure (p = 0.027) and mechanical respiratory support (p = 0.006) were related to mortality (40.7%) with statistical significance. Patients with SLEDAI higher to 10 at SLE onset showed more likelihood of developing PH. The OR was 2.68 (p = 0.046).
Although treatment in SLE has progressed in recent years, PH continues to be a rare and severe complication of this disease. When a PH is suspected, studies to confirm it must be done rapidly, since early diagnosis and aggressive treatment have been shown to improve survival. We observed that patients with renal involvement and mechanical respiratory support had higher mortality than SLE patients without them.
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Citation Excerpt :
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A multicenter retrospective study was conducted in 29 Italian Centers. Clinicians were asked to recruit all patients diagnosed with AAV-associated AH during the last 10 years, from 2007 to 2016. Univariate and multivariable analysis were performed.
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View all citing articles on Scopus

Humeira Badsha, MD: Consultant, Department of Rheumatology, Allergy and Immunology, Tan Tock Seng Hospital, Singapore

Cheng Lay Teh, MD, MRCP: Registrar, Department of Rheumatology, Allergy and Immunology, Tan Tock Seng Hospital, Singapore

Kok Ooi Kong, MBBS, MRCP: Registrar, Department of Rheumatology, Allergy and Immunology, Tan Tock Seng Hospital, Singapore

Tsui Yee Lian, MBBS, MRCP: Associate Consultant, Department of Rheumatology, Allergy and Immunology, Tan Tock Seng Hospital, Singapore

Hiok Hee Chng, MMed (Int Med), FRCP (Glas), FAMS: Senior Consultant and Head, Department of Rheumatology, Allergy and Immunology, Tan Tock Seng Hospital, Singapore.

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Pulmonary hemorrhage in systemic lupus erythematosus

Abstract

Objective

Methods

Results

Conclusion

Section snippets

Patient selection

Patient characteristics

Disease activity

Discussion

Semin Arthritis Rheum

Chest

Chest

Fatal pulmonary haemorrhage in systemic lupus erythematosus. Occurrence without haemoptysis

J Rheumatol

Pulmonary haemorrhage in systemic lupus erythematosus

Lupus

Derivation of the SLEDAI. A disease activity index for lupus patients

Arthritis Rheum

Reliability and validity of six systems for the clinical assessment of disease activity in systemic lupus erythematosus

Arthritis Rheum

The 1982 revised criteria for the classification of systemic lupus erythematosus

Arthritis Rheum

Diffuse alveolar hemorrhage and systemic lupus erythematosus. Clinical presentation, histology, survival, and outcome

Medicine (Baltimore)

CT appearance of diffuse alveolar hemorrhage in a patient with systemic lupus erythematosus

Acta Radiol

Pulmonary haemorrhage complicating systemic lupus erythematosusrole of MR imaging in diagnosis

AJR Am J Roentgenol