A rare presentation of systemic lupus erythematosus: diffuse alveolar hemorrhage in an infant

Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease characterized by a variable clinical picture and serological abnormalities. Children and adolescents generally have a more severe disease presentation; develop disease damage more quickly than adults with SLE. Diffuse alveolar hemorrhage (DAH) is a rare life-threatening complication in SLE. In this report a case of infantile onset SLE with DAH and negative ANA is presented.


Introduction
Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease characterized by a variable clinical picture and serological abnormalities. Children and adolescents generally have a more severe disease presentation; develop disease damage more quickly than adults with SLE. Diffuse alveolar hemorrhage (DAH) is a rare life-threatening complication in SLE. In this report a case of infantile onset SLE with DAH and negative ANA is presented.

Objectives
In this report a case of infantile onset SLE with DAH and negative ANA is presented.

Results
The patient was diagnosed as SLE with Systemic Lupus International Collaborating Clinics (SLICC) criteria which were reported to be more sensitive than ACR (American College of Rheumatology) criteria. The initial SLE Disease Activity Index (SLEDAI) was 15. Intravenous methylprednisolone was administered at a dose of 30 mg/kg/day. In the second day of treatment he suddenly developed dyspnea. Chest radiograph demonstrated diffuse alveolar shadows in both lungs without cardiyomegaly. He was transferred to the intensive care unit with the diagnosis of acute respiratory distress syndrome (ARDS) and supported with mechanical ventilation. During this support severe lung bleeding was inspected from the endotracheal tube. Intravenous cyclophosphamide 500 mg/m 2 was added to therapy and response to treatment was observed in a few days. He was successfully weaned off ventilator support after 7 days. In his 4th follow-up month, he is in remission with monthly cyclophosphamide and prednisolone.

Conclusion
Children and adolescents generally have a more severe disease presentation; develop disease damage more quickly than adults with SLE. Diffuse alveolar hemorrhage (DAH) is a rare life-threatening complication of SLE. It is an uncommon complication with estimates ranging from < 2 to 5.4 % in cohorts of lupus patients. DAH usually occurs in patients with established diagnosis of 1 Department of Pediatrics Rheumatology, Kocaeli Univertsity Faculty of Medicine, Kocaeli, Turkey Full list of author information is available at the end of the article Ozturk et al. Pediatric Rheumatology 2014, 12(Suppl 1):P315 http://www.ped-rheum.com/content/12/S1/P315 SLE; however, cases have been reported where DAH was initial presentation of SLE. DAH can often mimic severe pneumonia or ARDS. Optimal management of DAH has not been established. Use of cyclophosphamide has been linked to better survival as in our case.This case is having a combination of the rarely known presenting features of SLE as infantile onset, DAH and negative ANA.

Disclosure of interest
None declared.