Abstract
The concept of Childhood Interstitial Lung Disease (ChILD) is relatively young. There has been tremendous progress in this field in the last decade. The key advance has been the recognition of interstitial lung diseases that are often distinct and occur mainly in infants. Diagnosis is challenging because the incidence is low and no single center in the world has enough cases to promote experience and clinical skills. This has led to formation of international groups of people interested in the field and the “Children’s interstitial and diffuse lung disease research network” (ChILDRN) is one such group which contributed to the progress of this field. Clinically, these disorders overlap with those of other common respiratory disorders. Hence, clinical practice guidelines emphasize the additional role of chest imaging, genetic testing and lung biopsy in the diagnostic evaluation. Genetic testing, in particular, has shown tremendous progress in this field. Being noninvasive, it has the potential to help early recognition in a vast majority. Despite progress, definitive therapeutic modalities are still lacking and supportive care is still the backbone of management in the majority. Early recognition of the definitive diagnosis helps in the management, even if, in a significant number, it helps in avoiding unnecessary therapy. Also discussed in this article, is the pulmonary manifestation of rheumatic diseases in children. The incidence and spectrum of pulmonary involvement in rheumatic conditions vary and can be result of the primary disease or its management or due to an concurrent infection.
Similar content being viewed by others
References
Dinwiddie R. Idiopathic interstitial pneumonitis in children: a national survey in the United Kingdom and Ireland. Pediatr Pulmonol. 2002;34:23.
Kurland G, Deterding RR, Hagood JS, et al. An official american thoracic society clinical practice guideline: classification, evaluation, and management of childhood interstitial lung disease in infancy. Am J Respir Crit Care Med. 2013;188:376–94.
Armes JE, Mifsud W, Ashworth M. Diffuse lung disease of infancy: a pattern-based, algorithmic approach to histological diagnosis. J Clin Pathol. 2014;68:100–10.
Brody, Alan S, Paul G, Young LR. Thoracic imaging: direct diagnosis in radiology. Am J Roentgenol. 2011;197:W203.
Nogee LM. Genetic basis of children’s interstitial lung disease. Pediatr Allergy, Immunol Pulmonol. 2010;23:15–24.
Kuo CS, Young LR. Interstitial lung disease in children. Curr Opin Pediatr. 2014;26:320–7.
Fan LL, Langston C. Pediatric interstitial lung disease: children are not small adults. Am J Respir Crit Care Med. 2002;165:1466–7.
Desmarquest P, Tamalet A, Fauroux B, et al. Chronic interstitial lung disease in children: response to high-dose intravenous methylprednisolone pulses. Pediatr Pulmonol. 1998;26:332–8.
Dinwiddie R Treatment of interstitial lung disease in children. Paediatr Respir Rev. 2004;5:108–15.
Hamvas A, Nogee LM, Mallory GB, et al. Lung transplantation for treatment of infants with surfactant protein B deficiency. J Pediatr. 1997;130:231–9.
Palomar LM, Nogee LM, Sweet SC, Huddleston CB, Cole FS, Hamvas A. Long-term outcomes after infant lung transplantation for surfactant protein B deficiency related to other causes of respiratory failure. J Pediatr. 2006;149:548–53.
Pluchinotta FR, Schiavo B, Vittadello F, Martini G, Perilongo G, Zulian F. Distinctive clinical features of pediatric systemic lupus erythematosus in three different age classes. Lupus. 2007;16:550–5.
Caeiro F, Michielson FM, Bernstein R, Hughes GR, Ansell BM. Systemic lupus erythematosus in childhood. Ann Rheum Dis. 1981;40:325–31.
Kimura Y, Weiss JE, Haroldson KL, et al. Pulmonary hypertension and other potentially fatal pulmonary complications in systemic juvenile idiopathic arthritis. Arthritis Care Res. 2013;65:745–52.
Na SJ, Kim SM, Sunwoo IN, Choi YC. Clinical characteristics and outcomes of juvenile and adult dermatomyositis. J Korean Med Sci. 2009;24:715.
Trapani S, Camiciottoli G, Vierucci A, Pistolesi M, Falcini F. Pulmonary involvement in juvenile dermatomyositis: a two-year longitudinal study. Rheumatology. 2001;40:216–20.
Sanner H, Aaløkken TM, Gran JT, Sjaastad I, Johansen B, Flatø B. Pulmonary outcome in juvenile dermatomyositis: a case–control study. Ann Rheum Dis. 2011;70:86–91.
Misra R, Singh G, Aggarwal P, Aggarwal A. Juvenile onset systemic sclerosis: a single center experience of 23 cases from Asia. Clin Rheumatol. 2006;26:1259–62.
Tsai YY, Yang YH, Yu HH, Wang LC, Lee JH, Chiang BL. Fifteen-year experience of pediatric-onset mixed connective tissue disease. Clin Rheumatol. 2010;29:53–8.
Cabral DA, Uribe AG, Benseler S, et al. Classification, presentation, and initial treatment of Wegener’s granulomatosis in childhood. Arthritis Rheum. 2009;60:3413–24.
Milman N, Hoffmann AL. Childhood sarcoidosis: long-term follow-up. Eur Respir J. 2008;31:592–8.
Lynch J, Ma Y, Koss M, White E. Pulmonary sarcoidosis. Semin Respir Crit Care Med. 2007;28:53–74.
Conflict of Interest
None.
Source of Funding
None.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Ramamurthy, M.b., Goh, D.Y. & Lim, M.T.C. Rare Lung Diseases: Interstitial Lung Diseases and Lung Manifestations of Rheumatological Diseases. Indian J Pediatr 82, 956–961 (2015). https://doi.org/10.1007/s12098-015-1867-3
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s12098-015-1867-3