Sarcoidosis : Child vs adult
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《美国医学杂志》
1 Department of Lung Diseases, Cerrahpasa Medical Faculty, University of Istanbul, Istanbul, Turkey
2 Department of Rheumatology, Trakya Medical Faculty, University of Trakya, Edirne-Turkey, Turkey
Abstract
Objective : To compare the features of sarcoidosis in children to those of adults. In spite of the fact that sarcoidosis is a disease frequently seen in adults of 30-40 years, pediatric cases have rarely been reported. Methods : The authors evaluated 17 (3.3%, 12 females, 5 males) of the 516 patients of sarcoidosis, aged 16 yr and diagnosed within a 36-year period. Results : When the features of sarcoidosis subjects diagnosed during the childhood period were compared to those of subjects >16 years of age, it was seen that patients with advanced stage of the disease were more in the first group (41.2% vs 18%, p=0.02). In addition, children had more frequent extrapulmonary (64.7% vs 40.3%) and lacrimal gland involvement (p values, respectively, 0.044 and 0.003). Conclusion : When clinical data are relevant, sarcoidosis should be borne in mind. A child with sarcoidosis has more frequent pulmonary parenchymal and extrapulmonary involvement than in adults.
Keywords: Sarcoidosis; Child sarcoidosis; Extrapulmoner sarcoidosis; Adult sarcoidosis
Sarcoidosis is a granulomatous disease of unknown etiology which might affect many systems.[1] Although the disease is generally encountered between 20-40 yr of age, it might be seen at any age.[2] Sarcoidosis in childhood is relatively rare; however, some cases have been reported.[3],[4] The authors reviewed the general clinical features of patients diagnosed with sarcoidosis within a 36-year period and who were 16 yr old or younger. The reviews were evaluated. In addition, the clinical features of child patients suffering from sarcoidosis are compared to those of adult patients.
Materials and methods
Five hundred and sixteen patients diagnosed with sarcoidosis at the Cerrahpasa Medical Faculty, Department of Internal Medicine, Division of Pulmonary Diseases were included in the present study. The demographic and clinical features, radiologic stage of the disease at initial diagnosis, presence of extrapulmonary involvement, results of Kveim and tuberculin (Mantoux) tests of 17 patients who were 16 yr or younger at the time of initial diagnosis were obtained retrospectively from the hospital records. The features of these patients were compared to the features of other 499 subjects who were older than 16 yr at the time of diagnosis.
Clinical, radiological, and histopathological data were used to diagnose sarcoidosis. The criteria for diagnosis were: the presence of noncaseating granulomas in the histological examination of at least one tissue specimen; the absence of mycobacteria and fungi in tissues and cultures; and the exclusion of other granulomatous diseases. Tissues for histopathological examination were obtained by various means: in 8 cases by transbronchial biopsy, in 3 cases by peripheral lymph node biopsy, in 3 cases by mediastinoscopic biopsy, in 2 cases by lacrimal gland biopsy, and in one case by skin biopsy. Classification of chest X-rays was based on the criteria of DeRemee[5] as follows : stage I, bilateral hilar lymphadenopathy (BHL); stage II, BHL and parenchymal infiltration; stage III, parenchymal infiltration without BHL.
Results
Of the 516 sarcoidosis patients (341 females, 175 males), 17 (3.3%) were of 16 yr or younger. Twelve of these were females and 5 were males. Radiologically, 3 patients had stage 0 disease, 7 had stage I disease, 5 had stage II disease, and 2 had stage III disease. Findings of extrapulmonary involvement were present in 11 (64.7%) of the patients. These were as follows: skin and lacrimal gland involvement (3 cases each); anterior uveitis, erythema nodosum, peripheral lymph node and spleen involvement (2 cases each); and arthritis (one case). Immune thrombocytopenia was present in one patient.
Kveim test was performed in 16 patients and it was positive in 10 (62.5%). Mantoux test was performed in all cases and a positive result was obtained in 4 patients (23.5%). The results of these two tests were not different from those of the adult patients (p>0.05). The comparison of the features of child and adult sarcoidosis patients is seen in table1. The frequency of advanced stage (II+III) disease in children was significantly higher than that in adults (p=0.02). In addition, children with sarcoidosis had significantly higher rates of extrapulmonary organ and lacrimal gland involvement when compared to adults (p values respectively, 0.044 and 0.003) table1.
Discussion
Although some childhood sarcoidosis series have been reported,[2],[3],[4],[6],[7] most of these series have not been compared to adult sarcoidosis patients in detail. In addition, some recent studies reported that sarcoidosis had different clinical features in patients younger than and older than 5 years of age.[6],[7] These studies concluded that while skin, eye and joint involvement was more prominent in children younger than 5 years, lung, lymph node and eye involvement was prominent in older children.[7] In the present study, the age of onset of the disease was 16 yr in 3.3% of the sarcoidosis patients. However, all of those patients were >9 yr of age. Therefore, the authors especially compared the clinical features of the child sarcoidosis patients to those of the adult patients. According to literature data about childhood sarcoidosis, nearly 3% of the sarcoidosis cases reported until 1953 were children.[8] Although it is known that the onset of sarcoidosis in children is generally between 9-15 years of age, it was also reported that the disease might start at a very early age.[9],[10] According to the results of a large series,[3] 1.7% of the patients were 9-12 yr of age and 1.2% were 13-15 yr of age.
In the present study, female sex was predominant in children with sarcoidosis, similar to adults. Some series about this subject reported that female/male ratio in child sarcoidosis patients was similar.[2],[4] However, in the series of Siltzbach et al.[3] there were more male patients. The presence of a few number of male patients in child sarcoidosis series probably might have led to confusion about sex distribution.
It was observed that the frequency of patients with advanced stage disease and pulmonary parenchymal involvement was significantly more among the child sarcoidosis patients in this study. This condition might be explained by sarcoidosis not being considered in the differential diagnosis at early stages in children and diagnosing the disease at advanced stages only when it causes prominent symptoms and clinical signs. In addition, the usage of routine chest X-ray less frequently in pediatric patients might be another cause. However, the results of different series claimed that the frequencies of pulmonary parenchymal involvement in children and adults were similar.[11] In the series of Merten et al.[2], pulmonary parenchymal involvement was present in 64% of the patients; and in the present series, 41.2% of the cases had pulmonary parenchymal involvement.
Extrapulmonary organ involvement was more frequent in the child sarcoidosis patients of this study than in the adult patients. The present study about child sarcoidosis patients reported that multisystem involvement was present in 23 of the 25 patients.[4] In another series, 14 of the 18 subjects had extrathoracic involvement.[3] We might conclude that extrapulmonary involvement is of great concern in pediatric sarcoidosis patients. This is probably because patients have more advanced stage disease at initial diagnosis, and also pediatric patients with a diagnosis are ones with extrapulmonary involvement.
References
1. Loddenkemper R, Kloppenborg A, Schoenfeld N, Grosser H, Costabel U (WATL Study Group). Clinical findings in 715 patients with newly detected pulmonary sarcoidosis -results of a cooperative study in former West Germany and Switzerland. Sarcoidosis Vasc Diffuse Lung Dis 1998; 15 : 178-182.
2. Merten DF, Kirks DR, Grossman H. Pulmonary sarcoidosis in childhood. AJR 1980; 135 : 673-679.
3. Siltzbach LE, Greenberg M. Childhood sarcoidosis -a study of 18 patients. N Engl J Med 1968; 279; 1239-1245.
4. Jasper PL, Denny FW. Sarcoidosis in children. J Pediatr 1968;73 : 505-512.
5. De Remee RA. The roentgenographic staging of sarcoidosis. Historic and contemporary perspectives. Chest 1983; 1 : 128-133.
6. Shetty AK, Gedalia A. Sarcoidosis: a pediatric perspective. Clin Pediatr (Phila) 1998; 37 : 707-717.
7. Milman N, Hoffmann AL, Byg KE. Sarcoidosis in children. Epidemiology in Danes, clinical features, diagnosis, treatment and prognosis. Acta Paediatr 1998; 87 : 871-878.
8. McGovern JP, Merritt DM. Sarcoidosis in childhood. Adv Pediatr 1956; 8: 97-135.
9. Hetherington S. Sarcoidosis in young children. Am J Dis Child 1982; 136 : 13-15.
10. Pattishall EN, Strope GL, Spinola SM, Denny FW. Childhood sarcoidosis. J Pediatr 1986; 108: 169-177.
11. Kirks DR, McCormick VD, Greenspan RH. Pulmonary sarcoidosis: roentgenologic analysis of 150 patients. AJR 1973; 177 : 777-786.(Yanardag Halil, Pamuk Ome)
2 Department of Rheumatology, Trakya Medical Faculty, University of Trakya, Edirne-Turkey, Turkey
Abstract
Objective : To compare the features of sarcoidosis in children to those of adults. In spite of the fact that sarcoidosis is a disease frequently seen in adults of 30-40 years, pediatric cases have rarely been reported. Methods : The authors evaluated 17 (3.3%, 12 females, 5 males) of the 516 patients of sarcoidosis, aged 16 yr and diagnosed within a 36-year period. Results : When the features of sarcoidosis subjects diagnosed during the childhood period were compared to those of subjects >16 years of age, it was seen that patients with advanced stage of the disease were more in the first group (41.2% vs 18%, p=0.02). In addition, children had more frequent extrapulmonary (64.7% vs 40.3%) and lacrimal gland involvement (p values, respectively, 0.044 and 0.003). Conclusion : When clinical data are relevant, sarcoidosis should be borne in mind. A child with sarcoidosis has more frequent pulmonary parenchymal and extrapulmonary involvement than in adults.
Keywords: Sarcoidosis; Child sarcoidosis; Extrapulmoner sarcoidosis; Adult sarcoidosis
Sarcoidosis is a granulomatous disease of unknown etiology which might affect many systems.[1] Although the disease is generally encountered between 20-40 yr of age, it might be seen at any age.[2] Sarcoidosis in childhood is relatively rare; however, some cases have been reported.[3],[4] The authors reviewed the general clinical features of patients diagnosed with sarcoidosis within a 36-year period and who were 16 yr old or younger. The reviews were evaluated. In addition, the clinical features of child patients suffering from sarcoidosis are compared to those of adult patients.
Materials and methods
Five hundred and sixteen patients diagnosed with sarcoidosis at the Cerrahpasa Medical Faculty, Department of Internal Medicine, Division of Pulmonary Diseases were included in the present study. The demographic and clinical features, radiologic stage of the disease at initial diagnosis, presence of extrapulmonary involvement, results of Kveim and tuberculin (Mantoux) tests of 17 patients who were 16 yr or younger at the time of initial diagnosis were obtained retrospectively from the hospital records. The features of these patients were compared to the features of other 499 subjects who were older than 16 yr at the time of diagnosis.
Clinical, radiological, and histopathological data were used to diagnose sarcoidosis. The criteria for diagnosis were: the presence of noncaseating granulomas in the histological examination of at least one tissue specimen; the absence of mycobacteria and fungi in tissues and cultures; and the exclusion of other granulomatous diseases. Tissues for histopathological examination were obtained by various means: in 8 cases by transbronchial biopsy, in 3 cases by peripheral lymph node biopsy, in 3 cases by mediastinoscopic biopsy, in 2 cases by lacrimal gland biopsy, and in one case by skin biopsy. Classification of chest X-rays was based on the criteria of DeRemee[5] as follows : stage I, bilateral hilar lymphadenopathy (BHL); stage II, BHL and parenchymal infiltration; stage III, parenchymal infiltration without BHL.
Results
Of the 516 sarcoidosis patients (341 females, 175 males), 17 (3.3%) were of 16 yr or younger. Twelve of these were females and 5 were males. Radiologically, 3 patients had stage 0 disease, 7 had stage I disease, 5 had stage II disease, and 2 had stage III disease. Findings of extrapulmonary involvement were present in 11 (64.7%) of the patients. These were as follows: skin and lacrimal gland involvement (3 cases each); anterior uveitis, erythema nodosum, peripheral lymph node and spleen involvement (2 cases each); and arthritis (one case). Immune thrombocytopenia was present in one patient.
Kveim test was performed in 16 patients and it was positive in 10 (62.5%). Mantoux test was performed in all cases and a positive result was obtained in 4 patients (23.5%). The results of these two tests were not different from those of the adult patients (p>0.05). The comparison of the features of child and adult sarcoidosis patients is seen in table1. The frequency of advanced stage (II+III) disease in children was significantly higher than that in adults (p=0.02). In addition, children with sarcoidosis had significantly higher rates of extrapulmonary organ and lacrimal gland involvement when compared to adults (p values respectively, 0.044 and 0.003) table1.
Discussion
Although some childhood sarcoidosis series have been reported,[2],[3],[4],[6],[7] most of these series have not been compared to adult sarcoidosis patients in detail. In addition, some recent studies reported that sarcoidosis had different clinical features in patients younger than and older than 5 years of age.[6],[7] These studies concluded that while skin, eye and joint involvement was more prominent in children younger than 5 years, lung, lymph node and eye involvement was prominent in older children.[7] In the present study, the age of onset of the disease was 16 yr in 3.3% of the sarcoidosis patients. However, all of those patients were >9 yr of age. Therefore, the authors especially compared the clinical features of the child sarcoidosis patients to those of the adult patients. According to literature data about childhood sarcoidosis, nearly 3% of the sarcoidosis cases reported until 1953 were children.[8] Although it is known that the onset of sarcoidosis in children is generally between 9-15 years of age, it was also reported that the disease might start at a very early age.[9],[10] According to the results of a large series,[3] 1.7% of the patients were 9-12 yr of age and 1.2% were 13-15 yr of age.
In the present study, female sex was predominant in children with sarcoidosis, similar to adults. Some series about this subject reported that female/male ratio in child sarcoidosis patients was similar.[2],[4] However, in the series of Siltzbach et al.[3] there were more male patients. The presence of a few number of male patients in child sarcoidosis series probably might have led to confusion about sex distribution.
It was observed that the frequency of patients with advanced stage disease and pulmonary parenchymal involvement was significantly more among the child sarcoidosis patients in this study. This condition might be explained by sarcoidosis not being considered in the differential diagnosis at early stages in children and diagnosing the disease at advanced stages only when it causes prominent symptoms and clinical signs. In addition, the usage of routine chest X-ray less frequently in pediatric patients might be another cause. However, the results of different series claimed that the frequencies of pulmonary parenchymal involvement in children and adults were similar.[11] In the series of Merten et al.[2], pulmonary parenchymal involvement was present in 64% of the patients; and in the present series, 41.2% of the cases had pulmonary parenchymal involvement.
Extrapulmonary organ involvement was more frequent in the child sarcoidosis patients of this study than in the adult patients. The present study about child sarcoidosis patients reported that multisystem involvement was present in 23 of the 25 patients.[4] In another series, 14 of the 18 subjects had extrathoracic involvement.[3] We might conclude that extrapulmonary involvement is of great concern in pediatric sarcoidosis patients. This is probably because patients have more advanced stage disease at initial diagnosis, and also pediatric patients with a diagnosis are ones with extrapulmonary involvement.
References
1. Loddenkemper R, Kloppenborg A, Schoenfeld N, Grosser H, Costabel U (WATL Study Group). Clinical findings in 715 patients with newly detected pulmonary sarcoidosis -results of a cooperative study in former West Germany and Switzerland. Sarcoidosis Vasc Diffuse Lung Dis 1998; 15 : 178-182.
2. Merten DF, Kirks DR, Grossman H. Pulmonary sarcoidosis in childhood. AJR 1980; 135 : 673-679.
3. Siltzbach LE, Greenberg M. Childhood sarcoidosis -a study of 18 patients. N Engl J Med 1968; 279; 1239-1245.
4. Jasper PL, Denny FW. Sarcoidosis in children. J Pediatr 1968;73 : 505-512.
5. De Remee RA. The roentgenographic staging of sarcoidosis. Historic and contemporary perspectives. Chest 1983; 1 : 128-133.
6. Shetty AK, Gedalia A. Sarcoidosis: a pediatric perspective. Clin Pediatr (Phila) 1998; 37 : 707-717.
7. Milman N, Hoffmann AL, Byg KE. Sarcoidosis in children. Epidemiology in Danes, clinical features, diagnosis, treatment and prognosis. Acta Paediatr 1998; 87 : 871-878.
8. McGovern JP, Merritt DM. Sarcoidosis in childhood. Adv Pediatr 1956; 8: 97-135.
9. Hetherington S. Sarcoidosis in young children. Am J Dis Child 1982; 136 : 13-15.
10. Pattishall EN, Strope GL, Spinola SM, Denny FW. Childhood sarcoidosis. J Pediatr 1986; 108: 169-177.
11. Kirks DR, McCormick VD, Greenspan RH. Pulmonary sarcoidosis: roentgenologic analysis of 150 patients. AJR 1973; 177 : 777-786.(Yanardag Halil, Pamuk Ome)