Pediatric Critical Care Medicine:

May 2014 - Volume 15 - Issue 4_suppl - p 98

doi: 10.1097/01.pcc.0000449152.75037.6a

MICROSCOPIC POLYANGITIS DISEASE IN A CHILD SUCCESSFULY TREATED BY PLASMA EXCHANGE AND IMMUNOSUPRESIVE THERAPY

Haytoglu, Z.; Misirlioglu, P.

ABSTRACT

Background and aims: Microscopic polyangitis (MAP) is a rare systemic vasculitis that affects small vessels causes life threating diffuse alveolar hemorrhage (DAH) and renal involvement.

Aims: Here we report a rare diseaes seen in children.

Methods: 13 year-old girl with DAH and glomerulonephritis diagnosed MAP and improved by immunosupresive therapy and plasma exchange.

Results: 13 year-old girl with progressive cough, dyspnea and blood-tinged sputum for previous two weeks admitted to our hospital. A chest x-ray revealed an increased intertisiel opacities, chest CT revealed ground-glass opacities in both whole lung fields. Bronchoscopy revealed bloody bronchoalveolar lavage. On the third day urinanalysis showed microscopic hematuria moderate proteinuria with normal renal function test. Presence of pulmonary and renal involvement suggested serological testing and p-ANCA was positive while ANA, antiDNA, C3, C4, antiGBM antibodies were negative.On the fifth day ARDS occured she was entubated and put on mechanical ventilation. We diagnosed this condition as microscopic polyangitis presenting with initial pulmonary involvement, We started pulse steroid therapy for five days, 500mg/m2 pulse cyclophosphamide every 4 weeks Because of life threating DAH plasma exchange started.Seven daily afterwards seven times every other day, four times weekly continued. After the six weeks treatment started p-ANCA was negative and chest x-ray was normal.Three months later urinanalysis revealed no hematuria but still moderte proteinuria.

Conclusions: MAP is a rare systemic vasculitis involves many vital organs. The managment includes induction therapy which is typically done with cyclophosphamide and methylprednisone, takes usually 2 or 6 months.These are the recomendations extrapolated from adults.Randomized controlled studies are needed for the managment of MPA in children.