title: Childhood sarcoidosis: Louisiana experience.
abstract: A retrospective chart review was conducted to detect patients with sarcoidosis seen by pediatric rheumatology service from the period of 1992 to 2013 at Children's hospital of New Orleans. Twenty-seven patients were identified. The average duration of symptoms before diagnosis was 5 (range 1-120) months. Five patients had onset before the age of 5 years and were diagnosed with early-onset sarcoidosis. The most common manifestations at presentation were constitutional symptoms (62 %) followed by ocular (38 %). During the course of illness, 19/27 (70 %) had multiorgan involvement. Common manifestations included uveitis/iritis (77 %), fever (50 %), hilar adenopathy (42 %), arthritis (31 %), peripheral lympadenopathy (31 %), hepatosplenomegaly (31 %), parenchymal lung disease (27 %), and skin rash (19 %). Unusual manifestations included granulomatous bone marrow disease (3 cases), hypertension (2), abdominal aortic aneurysm (large vessel vasculitis; 1), granulomatous hepatitis (1), nephrocalcinosis (1), membranous nephropathy (1), refractory granulomatous interstitial nephritis with recurrence in transplanted kidney (1), CNS involvement (2), parotid gland enlargement (1), and sensorineural hearing loss (1). Biopsy specimen was obtained in 21/27 (77 %) patients, and demonstration of noncaseating granuloma associated with negative stains for mycobacteria and fungi was seen in 18 patients. Elevated angiotensin-converting enzyme level was seen in 74 % of patients. Treatment with oral prednisone was initiated in symptomatic patients with significant clinical improvement. Low-dose methotrexate (MTX) 10-15 mg/m(2)/week orally, as steroid-sparing agent, was administered in 14 patients. Other immunomodulators included cyclophosphamide (2 patients), etanercept (2), infliximab (2), mycophenolate mofetil (1), and tacrolimus (1). Childhood sarcoidosis is prevalent in Louisiana. Most of the affected children present with a multisystem disease associated with manifestations similar to those of adult patients. Low-dose MTX seems to be effective, steroid sparing, and safe adjunct to treat sarcoidosis with multiorgan involvement. Early-onset disease is less common and associated with increased morbidity, flares, and poor prognosis.
authors.affiliation: Division of Pediatric Rheumatology, Children's Hospital and Louisiana State University Health Sciences Center, 200 Henry Clay Ave, New Orleans, LA, 70118, USA. agedal@lsuhsc.edu.
authors.forename: Abraham
authors.initials: A
authors.lastname: Gedalia
authors.affiliation: Division of Pediatric Rheumatology, Children's Hospital and Louisiana State University Health Sciences Center, 200 Henry Clay Ave, New Orleans, LA, 70118, USA.|Section of Rheumatology, LSU Health Sciences Center, New Orleans, LA, USA.
authors.forename: Tahir A
authors.initials: TA
authors.lastname: Khan
authors.affiliation: Division of Pediatric Rheumatology, Children's Hospital and Louisiana State University Health Sciences Center, 200 Henry Clay Ave, New Orleans, LA, 70118, USA.|Pediatric Infectious disease, Wake Forest University Medical Center, Winston Salem, NC, USA.
authors.forename: Avinash K
authors.initials: AK
authors.lastname: Shetty
authors.affiliation: Division of Pediatric Rheumatology, Children's Hospital and Louisiana State University Health Sciences Center, 200 Henry Clay Ave, New Orleans, LA, 70118, USA.
authors.forename: Victoria R
authors.initials: VR
authors.lastname: Dimitriades
authors.affiliation: Division of Pediatric Rheumatology, Children's Hospital and Louisiana State University Health Sciences Center, 200 Henry Clay Ave, New Orleans, LA, 70118, USA.|Section of Rheumatology, LSU Health Sciences Center, New Orleans, LA, USA.
authors.forename: Luis R
authors.initials: LR
authors.lastname: Espinoza
mesh_terms: D000293:Adolescent; D001168:Arthritis / Q000188:drug therapy* / Q000453:epidemiology* / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007223:Infant; D000069285:Infliximab / Q000627:therapeutic use; D008145:Louisiana; D008297:Male; D008727:Methotrexate / Q000627:therapeutic use*; D011241:Prednisone / Q000627:therapeutic use*; D012189:Retrospective Studies; D012507:Sarcoidosis; D013585:Synovitis / Q000188:drug therapy* / Q000453:epidemiology* / Q000473:pathology; D014605:Uveitis / Q000188:drug therapy* / Q000453:epidemiology* / Q000473:pathology; D055815:Young Adult; D056660:Hereditary Autoinflammatory Diseases
chemical_list: D003520:Cyclophosphamide; D000069285:Infliximab; D011241:Prednisone; D008727:Methotrexate
keywords: Early-onset childhood sarcoidosis; Methotrexate; Prednisone; Steroid sparing