Sarcoidosis
Sarcoidosis: Excerpt from The 5-Minute Pediatric Consult
Frank Pessler, MD, PhDRandy Q. Cron, MD, PhD
Sarcoidosis - BASICS
Sarcoidosis - description
A multisystem chronic granulomatous disease that has 2 distinct variations often differentiated by age of onset
Sarcoidosis - epidemiology
Disease occurs before age 4 years as arthritis, uveitis, and rash, and in adolescence as Lofgren syndrome of erythema nodosum, polyarthritis, and hilar adenopathy. Adult-type disease with marked pulmonary involvement may also occur in older adolescents. CNS involvement (rare): Seizures, cranial neuropathy, hypothalamic dysfunction
Sarcoidosis - risk factors
Sarcoidosis - genetics
- Blacks are more commonly affected than whites; specific genetic tendencies not identified.
- Early childhood cases may result from spontaneous mutations in CARD15/NOD2 gene.
Sarcoidosis - pathophysiology
T-cell–mediated disease resulting in noncaseating granulomas in affected organs
Sarcoidosis - etiology
Unknown (possibly infectious)
Sarcoidosis - DIAGNOSIS
Sarcoidosis - signs & symptoms
Sarcoidosis - history
Malaise, fever, rash, evanescent painful arthritis, swollen lymph nodes, chronic cough, and hematuria may be initial complaints.
Sarcoidosis - physical exam
- Peripheral lymphadenopathy is most common manifestation.
- Eyes may be injected.
- Bilateral parotid gland enlargement and hepatosplenomegaly may be present.
- The arthritis, usually in the ankles, is extremely tender and boggy.
- Rash is diffuse, erythematous, and macular or plaquelike.
- History of papule:
- Forming after needlestick (pathergy)
Sarcoidosis - tests
Sarcoidosis - lab
- CBC
- ESR
- Synovial effusions are typically noninflammatory.
- Biopsy of affected organ, such as peripheral lymph nodegland, skin, conjunctivae, or minor salivary gland or synovium (demonstrating noncaseating granuloma) is helpful.
- ACE level:
- Produced in most granulomatous diseases, but is useful in cases in which index of suspicion is high
- Lysozyme level:
- May be more sensitive than ACE level for detecting sarcoidosis
- Serum calcium and creatinine levels:
- Important in baseline evaluation
- Urine test for blood:
- Seen in patients with hypercalciuria
Sarcoidosis - imaging
- Chest radiography:
- May demonstrate hilar adenopathy
- Gallium scan:
- Demonstrates uptake diffusely in lungs (extremely sensitive test)
- False-positives:
- ACE level: May be elevated in patients with miliary tuberculosis and biliary cirrhosis. Not a perfect screening test; however, can follow levels in response to treatment
- Lysozyme level: Also elevated in lymphoma. May be useful to follow disease activity in proven cases, if ACE levels can’t be used.
Uveitis may be occult; ophthalmology evaluation is important.
Sarcoidosis - differencial diagnosis
- Infection:
- Tuberculosis, bacterial sepsis, mumps, HIV, gonorrhea, Lyme disease, pulmonary mycoses
- Tumors:
- Leukemia, neuroblastoma, lymphoma
- Immunologic:
- Oligoarticular juvenile idiopathic arthritis (for early-onset type), systemic juvenile rheumatoid arthritis, systemic lupus erythematosus, dermatomyositis, Behçet disease
- Genetic:
- Blau syndrome (autosomal dominant hereditary granulomatous syndrome of synovitis, uveitis, and cranial neuropathy, owing to defective CARD15/NOD2 gene)
Sarcoidosis - TREATMENT
Sarcoidosis - initial stabilization
Medical therapy during times of disease activity causing clinical symptoms
Sarcoidosis - general measures
Sarcoidosis - diet
Hydration is important in patients with hypercalcemia.
Sarcoidosis - medication
- NSAIDs, analgesics, and steroids all have a role. In cases of chronic disease
- Immunosuppressive drugs, such as corticosteroids (oral and/or inhaled) and methotrexate, can be used.
- The tumor necrosis factor-แinhibitor infliximab shows promising preliminary results. In cases of hypercalciuria/hypercalcemia, consider hydration and furosemide.
Sarcoidosis - FOLLOW UP
Sarcoidosis - prognosis
Generally good for most children. Lofgren syndrome resolves. >40% of older children with adult-type disease have persistent pulmonary changes, but only a few will have pulmonary symptoms.
Sarcoidosis - complications
- In children, usually related to uveitis or from hypercalciuria resulting in renal injury
- In older adolescents, pulmonary problems such as restrictive lung disease, as well as severe growth delay, may occur.
Sarcoidosis - patient monitoring
- When to expect improvement:
- Early childhood sarcoid and Lofgren syndrome may resolve over several months. Rarely, older children with pulmonary manifestations may develop chronic interstitial lung disease or bony infiltrates over years of disease.
- Signs to watch for:
- Climbing creatinine, shortness of breath, or persistent uveal tract inflammation
- Pitfalls:
- Overtreating asymptomatic lymphadenopathy and not detecting hypercalciuria
Sarcoidosis - bibliography
- Baughman RP, Iannuzzi M. Tumour necrosis factor in sarcoidosis and its potential for targeted therapy. BioDrugs. 2003;17:425–431.
- Baumann RJ, Robertson WC Jr. Neurosarcoid presents differently in children than in adults. Pediatrics. 2003;112:e480–e486.
- Cron RQ, Wallace CA, Sherry DD. Childhood sarcoidosis—does age of onset predict clinical manifestations? J Rheumatol. 1997;24:1654–1656.
- Fink CW, Cimaz R. Early onset sarcoidosis: Not a benign disease. J Rheumatol. 1997;24:174–177.
- Gedalia A, Molina JF, Ellis GS Jr, et al. Low-dose methotrexate therapy for childhood sarcoidosis. J Pediatr. 1997;130:3–4.
- Hafner R, Vogel P. Sarcoidosis of early onset. A challenge for the pediatric rheumatologist. Clin Exp Rheumatol. 1993;11:685–691.
- Lindsley CB, Petty RE. Overview and report on international registry of sarcoid arthritis in childhood. Curr Rheumatol Rep. 2000;2:343–348.
- Pattishall EN, Kendig EL Jr. Sarcoidosis in children. Pediatr Pulmonol. 1996;22:195–203.
- Rose CD, Wouters CH, Meiorin S, et al. Pediatric granulomatous arthritis: An international registry. Arthritis Rheum. 2006;54:3337–3344.
- Shetty AK, Gedalia A. Sarcoidosis: A pediatric perspective. Clin Pediatr. 1998;37:707–717.
- Tomita H, Sato S, Matsuda R, et al. Serum lysozyme levels and clinical features of sarcoidosis. Lung. 1999;177:161–167.
Sarcoidosis - CODES
Sarcoidosis - icd9
135.0 Sarcoidosis
Sarcoidosis - FAQ
- Q: Why is the outcome better in childhood sarcoid compared with adults with sarcoid?
- A: These may be 2 distinct granulomatous diseases. The 2 clearly have different patterns of organ involvement.
Book Source Details
- Book Title: The 5-Minute Pediatric Consult
- Author(s): M. William Schwartz MD; et al.
- Year of Publication: 2008
- Copyright Details: The 5-Minute Pediatric Consult, Copyright © 2008 Lippincott Williams & Wilkins.
More About Sarcoidosis
More Medical Textbooks Online about Sarcoidosis
Review other book chapters online related to Sarcoidosis:
Medical Books Excerpts
- Sarcoidosis
- "Professional Guide to Diseases (Eighth Edition)" (2005)
- [ read ]
Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.
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More About This Book:
Title: The 5-Minute Pediatric Consult
Authors: M. William Schwartz MD; et al.
Publisher: Lippincott Williams & Wilkins
Copyright: 2008
ISBN: 0-7817-7577-9
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