Allergy Asthma Respir Dis.  2015 Sep;3(5):370-374. 10.4168/aard.2015.3.5.370.

Pulmonary hemorrhage as an unusual initial manifestation of systemic lupus erythematosus

• Yang YS
• Cheon BR
• Shim JW
• Kim DS
• Jung HL
• Park MS
• Shim JY

• KMID: 2218649
• DOI: http://doi.org/10.4168/aard.2015.3.5.370

Abstract

Pulmonary hemorrhage as the initial manifestation of systemic lupus erythematosus (SLE) has been rarely reported in children. We present the case of a 10-year-old girl who was admitted to Kangbuk Samsung Hospital with hemoptysis. She had a 5-day history of cough with dyspnea. On physical exam, breath sound was significantly decreased combined with rales on both lung fields. Blood tests revealed pancytopenia, decreased complement levels (C3, 21.28 mg/dL; C4, 3.10 mg/dL), positive antinuclear antibody (>1:640) and anti-double-stranded DNA antibody (262.5 IU/mL). Chest computed tomography revealed patchy ground glass opacity on both lung fields. She had proteinuria and diffuse lupus nephritis (International Society of Nephrology/Renal Pathology Society class IV-G(A)) confirmed by renal biopsy. High-dose methylprednisolone pulse therapy (30 mg/kg/day) was given for 3 days and then switched to a maintenance dose (1 mg/kg/day). Initially hemoptysis resolved after administration of methylprednisolone, but recurred on the 14th day of treatment. She was then treated with cyclophosphamide pulse therapy and hemoptysis subsided without recurrence. She was discharged on the 31st day of admission. She continued to receive monthly cyclophosphamide pulse therapy until the occurrence of leukopenia and then her regimen was switched to mycophenolate and hydroxychloroquine. SLE continues to be well controlled after 18 months of treatment. Recognition of pulmonary hemorrhage as a possible initial manifestation of SLE is crucial for early diagnosis. SLE was successfully treated with good outcome.

Keyword

Systemic lupus erythematosus; Pulmonary hemorrhage; Pediatrics

MeSH Terms

Antibodies, Antinuclear
Biopsy
Child
Complement System Proteins
Cough
Cyclophosphamide
DNA
Dyspnea
Early Diagnosis
Female
Glass
Hematologic Tests
Hemoptysis
Hemorrhage*
Humans
Hydroxychloroquine
Leukopenia
Lung
Lupus Erythematosus, Systemic*
Lupus Nephritis
Methylprednisolone
Pancytopenia
Pathology
Pediatrics
Proteinuria
Recurrence
Respiratory Sounds
Thorax
Antibodies, Antinuclear
Complement System Proteins
Cyclophosphamide
DNA
Hydroxychloroquine
Methylprednisolone

Figure

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  Fig. 1 Chest radiograph at admission (A), at hospital day 20th (B), at discharge (C). (A) Initial chest radiograph shows diffuse ground glass opacities and consolidations in both lung fields. (B) Twenty days later, bilateral ground glass opacities are extended. (C) Previous ground glass opacities are nearly disappeared in both lung fields.

• 

  Fig. 2 Computed tomography image at 2nd day (A), at 21st day (B), and at discharge (C). (A) Axial computed tomography image demonstrates diffuse bilateral ground glass opacities with interlobular and intralobular septal line thickening. (B) Previous bilateral ground glass opacities are decreased in the both lung fields, but there are newly developed multifocal ground glass opacities and consolidations at superior segment of both lower lobes. (C) Previous ground glass opacities are disappeared at both lung fields.

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  Fig. 3 An overview of clinical course associated with treatment modality. HD, hospital day; O2, oxygen; C3, complement component 3.

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