Childhood sarcoidosis

Louisiana experience

Abraham Gedalia, Tahir A. Khan, Avinash K. Shetty, Victoria R. Dimitriades, Luis R. Espinoza

Research output: Contribution to journalReview article

14 Citations (Scopus)

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/m2/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.

Original languageEnglish
Pages (from-to)1879-1884
Number of pages6
JournalClinical Rheumatology
Volume35
Issue number7
DOIs
Publication statusPublished - 01-07-2016
Externally publishedYes

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Sarcoidosis
Methotrexate
Bone Marrow Diseases
Steroids
Iritis
Nephrocalcinosis
Mycophenolic Acid
Interstitial Nephritis
Membranous Glomerulonephritis
Sensorineural Hearing Loss
Parotid Gland
Uveitis
Abdominal Aortic Aneurysm
Immunologic Factors
Rheumatology
Tacrolimus
Peptidyl-Dipeptidase A
Mycobacterium
Vasculitis
Prednisone

All Science Journal Classification (ASJC) codes

  • Rheumatology

Cite this

Gedalia, A., Khan, T. A., Shetty, A. K., Dimitriades, V. R., & Espinoza, L. R. (2016). Childhood sarcoidosis: Louisiana experience. Clinical Rheumatology, 35(7), 1879-1884. https://doi.org/10.1007/s10067-015-2870-9
Gedalia, Abraham ; Khan, Tahir A. ; Shetty, Avinash K. ; Dimitriades, Victoria R. ; Espinoza, Luis R. / Childhood sarcoidosis : Louisiana experience. In: Clinical Rheumatology. 2016 ; Vol. 35, No. 7. pp. 1879-1884.
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Gedalia, A, Khan, TA, Shetty, AK, Dimitriades, VR & Espinoza, LR 2016, 'Childhood sarcoidosis: Louisiana experience', Clinical Rheumatology, vol. 35, no. 7, pp. 1879-1884. https://doi.org/10.1007/s10067-015-2870-9

Childhood sarcoidosis : Louisiana experience. / Gedalia, Abraham; Khan, Tahir A.; Shetty, Avinash K.; Dimitriades, Victoria R.; Espinoza, Luis R.

In: Clinical Rheumatology, Vol. 35, No. 7, 01.07.2016, p. 1879-1884.

Research output: Contribution to journalReview article

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AU - Gedalia, Abraham

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AU - Dimitriades, Victoria R.

AU - Espinoza, Luis R.

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N2 - 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/m2/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.

AB - 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/m2/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.

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Gedalia A, Khan TA, Shetty AK, Dimitriades VR, Espinoza LR. Childhood sarcoidosis: Louisiana experience. Clinical Rheumatology. 2016 Jul 1;35(7):1879-1884. https://doi.org/10.1007/s10067-015-2870-9