Volume 10 Supplement 1

2011 Pediatric Rheumatology Symposium: Abstracts

Open Access

Pulmonary thromboembolism in children with rheumatic diseases

  • Larry B Vogler4,
  • Sheila Angeles-Han3,
  • Sampath Prahalad2 and
  • Egla C Rabinovich1
Pediatric Rheumatology201210(Suppl 1):A73

DOI: 10.1186/1546-0096-10-S1-A73

Published: 13 July 2012

Purpose

To demonstrate the clinical features and predisposing factors of pulmonary thrombotic events in children with rheumatic diseases.

Methods

Chart review, observational.

Results

Thrombotic events have been associated with antiphospholipid antibodies in autoimmune diseases, including systemic lupus erythematosus (SLE). However, pulmonary thromboembolism (PTE) from deep vein thromboses (DVT) or in situ pulmonary arterial thrombosis is uncommon in rheumatic diseases, especially in children. The diagnosis and treatment of PTE may be delayed due to a paucity of symptoms or to symptoms attributed to more common manifestations such as pleuritis or pneumonia. We report findings in 6 children with PTE secondary to SLE (4), Systemic Sclerosis (SSc) (1) and Polyarteritis Nodosa (PAN) (1).

Table 1

Pt/Gender

1/F

2/F

3/M

4/F

5/M

6/M

Dx

SLE

SLE

SLE

SLE

SSc

PAN

 

thrombocytopenia

nephritis (IV)

nephritis (V)

nephritis (IV)

PAH

CVA

Age at Dx (yr)

12.6

14.1

9.0

12.0

12.8

0.3

Age at PTE

15.2

14.8

16.8

12.6

16

6/4

Symptoms

leg pain

chest pain

chest pain

chest pain

chest pain

leg pain

  

dyspnea

dyspnea

dyspnea

dyspnea

 

DVT

+

-

-

-

-

+

Lupus AC

+

-

-

-

-

+/-

Anticardio AB

-

-

-

-

-

+

D-dimer (ng/ml)

647 (nl <220)

8770

1600

>10,000

n/a

>10,000

Albumin (g/dl)

4.7 (nl 3.7-5.5)

2.0

0.7

1.7

4.1

3.6

AT III (%)

105 (nl 77-132)

278

7/2

154

n/a

114

Fibrinogen (ng/dl)

718 (nl 180-394)

234

n/a

298

n/a

421

[PAH pulmonary arterial hypertension, CVA: cerebral vascular accident, AT III: anti-thrombin III, N/A: not available] All patients were treated with heparin and improved. No patient had any other genetic risk factors predisposing to thrombophilia.

Conclusion

Although antiphospholipid antibodies are common in SLE, pulmonary arterial thrombosis is rare. These 4 cases of SLE represent only 1.7% of 234 pediatric lupus patients seen at Emory University over 18 years. Pulmonary thromboemboli may mimic pleuritis with effusion or pneumonia. Besides antiphospholipid antibodies, which were present in only 2 of these patients, other associated findings include nephrotic syndrome, elevated D-dimers and elevated fibrinogen levels. Recognition of PTE in pediatric patients with rheumatic diseases and prompt anti-coagulation therapy is important and potentially life-saving.

Disclosure

Larry B. Vogler: None; Sheila Angeles-Han: None; Sampath Prahalad: None; Egla C. Rabinovich: None.

Authors’ Affiliations

(1)
Duke University Medical Center
(2)
Emory Children's Center
(3)
Emory University
(4)
Emory University School of Medicine

Copyright

© Vogler et al; licensee BioMed Central Ltd. 2012

This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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