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Journal of Muscle Health

Cite this article: Mamunur Rashid AKM, Islam T, Biswas P (2017) Pediatric Rheumatologic Diseases (PRDS) and its Clinical Scenario. J Muscle Health 1(1): 1003.

*Corresponding authorAKM Mamunur Rashid, Associate Professor, Head of the Department Pediatrics, Khulna Medical College, Khulna, Bangladesh, Email:

Submitted: 22 May 2017

Accepted: 14 June 2017

Published: 16 June 2017

Copyright© 2017 Rashid et al.

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Keywords•PRDs•Clinical view

Research Article

Pediatric Rheumatologic Diseases (PRDS) and its Clinical ScenarioAKM Mamunur Rashid*, Tasnuva Islam, and Palash BiswasDepartment of Pediatrics, Khulna Medical College, Bangladesh

Abstract

Pediatric Rheumatic Diseases (PRDs) are chronic inflammatory illness causing childhood morbidity and mortality. These diseases now a day’s creates burden to the childhood illness. Better understanding of these diseases help in the early diagnosis of these chronic diseases. It is important for the early diagnosis and timely intervention can improve the outcome of PRDs. To detect the different types of rheumatic diseases in our children and to compare the clinical parameters of different PRDs.

A prospective study is done in a medical college hospital of Bangladesh 2010 to 2013. All the patients fulfilling the standard criteria of rheumatic diseases are included in these diseases. A total 159 patients are enrolled in this period. Male/Female ratio is 1.65:1. JIA is common among the PRDs. It is 55 (34.8%) in number. Other diseases are SA 8 (5.2%), RF 15(9.7%), SLE 8 (5.2%), KD 1 (8.4%), HSP 7 (4.5%), LN 18 (11.6%), PAN 3 (1.9%), Others 26 (17.4%). Fever is observed in 113 (71.06%) and joint symptom/signs in 102 (68.4%) PRDs. Among the total 135 PRDs 55 has leucocytosis, total 49 has 28 CRP positive, total140 has 95 increased ESR. Out of total 58 PBF in PRDs 35 has MH anemia. JIA is the most common rheumatic diseases in children. Among the pediatric vasculitis KD is the common type. Fever and the joint complaints are the most common presenting features in PRDs. CRP, ESR and leucocytosis is found to be significant in most of the PRDs. MH anemia is also the common association of PRDs.

ABBREVIATIONSPRDs: Pediatric Rheumatic Diseases; MH: Microcytic

Hypochromic Anemia; JIA: Juvenile Idiopathic Arthritis; ERA: Enthesitis Related Arthritis; KD: Kawasaki Disease; SoJIA: Systemic onset JIA; HSP: Henoch Schonlein Purpura; UK: United Kingdom; SLE: Systemic Lupus Erythematosus; ILAR: International League of Association for Rheumatology; SA: Septic Arthritis; RF: Rheumatic Fever; ACR: American College of Rheumatology; LN: Lupus Nephritis; PAN: Polyarteritis Nodosa; EULAR: European League Against Rheumatism; CRP: C reactive Protein; ESR: Erythrocyte Sedimentation Rate

INTRODUCTIONPediatric Rheumatic Diseases (PRDs) are the commonest

chronic causes of childhood morbidity and disability [1]. These diseases are chronic intractable inflammatory diseases with systemic life threatening complications [2]. One of the most important advances in pediatric rheumatology is that verities of rheumatic diseases affect children frequently and its normal growth and development. There is advancement in understanding the nature of inflammation, the cells and the molecules that mediate it and the therapeutics for regulating the abnormal

immune response [3]. Number of rheumatic diseases affecting the children, most common is Juvenile Idiopathic Arthritis (JIA). Early recognition and diagnosis as well as timely intervention can improve the outcome of pediatric rheumatologic diseases [4]. Treatment modalities vary in different rheumatic diseases.

Clinical profile of the rheumatic diseases in children is not yet known in our setting. This study focuses overall clinical view of rheumatic diseases in children.

METHODSIt was a prospective study carried out in the inpatient

department of pediatrics in a tertiary care hospital of Bangladesh from 2010 to 2013. All the patients are included randomly who fulfilled the ILAR, ACR, EULAR criteria for the diagnosis of different rheumatic diseases. Primary/Autoimmune vasculitis are only included in this study. Clinical history and features are recorded according to the preformed questionnaire. Finally data are analyzed and results are compiled.

RESULTSA total 159 pediatric rheumatic diseases are diagnosed in

that study period. Mean age of the children suffering from the

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diseases are 8 years and 5 months. Minimum age of the child is 1 year and maximum 12.5 years. Among them 99(62.3%) are male and 60(37.7%) female. Male/ Female ratio is 1.65:1. JIA is common among the pediatric rheumatic diseases. It is 55(34.8%) in number. Oligoarticular JIA is diagnosed in 21(38.2%) patients. Types of JIA are shown in pie diagram. Kawasaki disease (KD) is the commonest vasculitis observed in our study. It is 13(8.4%) in number among the total rheumatic diseases. The subtypes of JIA are shown in Pie diagram. Majority of the pediatric rheumatic diseases has fever and joint symptoms and signs in our study. Joint symptom/sign is found in 102 (68.4%) and fever in 113 (71.06%) children with PRDs. History and clinical features as a whole of pediatric rheumatic diseases are described in Table (1). In Table (2), History and clinical view are shown according to the individual pediatric rheumatic diseases. Leucocytosis is observed in 55 (40.7%) of valid 135 PRDs. Raised ESR is found in 95 among 140 valid cases. Among 49 PRDs 28 (57.1%) has CRP positive. MH anemia is the common association in PRDs which is marked in 35 (60.3%) of total 58 valid cases. Total investigation report of PRDs is depicted in Table (3).

DISCUSSIONPediatric male children suffer frequently from rheumatic

diseases than female in our study. Mohammad II and et al also

has the similar view like us in this point [1]. In other studies PRDs are female predominating diseases [5,6]. In our perspective male children are given more importance for medical advice and care. Mean age of the children suffering from the diseases in our study is 8.5 years. In another study, highest number is observed between the ages of 11-16 years [1]. JIA is the commonest rheumatic disease in children found in our study like others [1,2,7]. Oligoarticular (38.2%) is the most common type of JIA observed which differ from the study in Indian where ERA (35.3%) is the highest. Oligoarticular type is common like ours 33.3% in Canadian study [8,9]. Among the vasculitis KD is the common type observed in our study. These variations might be due to the difference in genetic and environmental influence. In another study Henoch Schonlein Purpura (HSP) is the common vasculitis observed [1]. These two vacuitides are found to be common in children with rheumatic diseases. Reports of KD have recently increased in some developing countries such as India because of recognition or the appearance of the disease at the time of industrialization. KD has a higher in incidence than previously indicated in the UK [10]. Fever and joint symptoms/signs are frequently observed in most of the rheumatic diseases. These correlate with the chronic inflammatory connective tissue diseases.

JIA is the most common rheumatic diseases in children. KD

Table 1: Number of histories and Signs/Symptoms present in Pediatric Rheumatic diseases.Age in monthsAverage

Sex H/O consanguinity Family history H/O sore throat H/O infection

100.5 M F VA MI Y N VA MI Y N VA MI Y N VA MI Y N99

(62.3)60

(37.7) 151 8 5(3.1)

146 (96.7) 153 6 8

(5)145(95) 155 4 39

(25)116(75) 145 14 47

(29.6)98

(61.6)

Fever Joint swelling Lymph node enlarged Rash hepatomegaly

VA MI Y N VA MI Y N VA MI Y N VA MI Y N

159 0 113(71.06)

46(28.93) 149 10 102

(68.4)47

(31.5) 156 03 49(31.4)

107(68.6) 154 05 47

(30.5)107

(69.5)4 or less

5 or more AXL CERL INGL GENL

61(40.9)

41(27.5) 7(4.5) 23

(14.5) 4(2.5) 15(9.4)

Splenomegaly Conjunctivitis Oral ulcer Gottron

VA MI Y N VA MI Y N VA MI Y N VA MI Y N

158 01 16(10.1)

142(89.3) 157 02 07

(4.5)150

(91.8) 158 01 13(8.2)

145(91.8) 156 03 19

(12.2)137

(87.8)Photosensitivity Hair fall Malar rash Skin pilling

VA MI Y N VA MI Y N VA MI Y N VA MI Y N

150 09 01(.6)149

(93.7) 155 04 10(6.5)

145(93.5) 157 02 13

(8.2) 144(90.6) 157 02 08(5) 149(93.7)

Haliotrop rash Bleeding Eye change Convulsion

VA MI Y N VA MI Y N VA MI Y N VA MI Y N

158 01 25(15.7)

133(83.6) 148 11 01

(.6)147

(91.2) 154 05 9(5.7) 145(91.2) 149 10 2(1.3) 147

(92.5)Abbreviations: VA: Valid Value; MI: Missing Value; Y: Yes; N: No; M: Male; F: Female; AXL: Axillary Lymph Node; CERL: Cervical Lymph Node; INGL: Inguinal Lymph Node; GENL: Generalized Lymph Node; Values within the parenthesis indicate percentage

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Table 2: Number of Signs/Symptoms present in individual pediatric Rheumatologic disease Patients.

DIS FeverJoint

symptomLymph node Rash

Hepato-megaly

Spleno megaly

Conjunctivitis oral

ulcerPhoto

SensitivityHairfall

malar rash

skin pilling

Halio-trop

GottronPapule

bleedingeye

change

Convu-lsion

C A I G

SLE08

(5.2)06 07 03 01 00 00 02 05 02 00 00 04 06 03 04 03 01 0 0 0 02

JIA55

(34.8)41 46 18 08 02 01 07 01 08 02 02 00 03 01 03 0 01 00 00 01 0

KD13

(8.4)13 02 16 10 02 03 01 12 01 01 01 08 06 00 00 03 08 00 00 01 0

HSP7

(4.5)05 06 01 00 01 00 00 05 0 00 0 0 0 0 0 0 03 0 0 4 0

LN18

(11.6)03 03 00 0 0 0 0 01 00 00 00 01 05 02 03 01 02 0 0 0 01

SA8

(5.2)08 08 00 0 0 0 0 01 0 01 0 0 0 0 0 0 01 0 0 0 0

PAN3

(1.9)01 01 00 0 0 0 0 01 0 - 0 0 0 0 0 0 0 0 0 0 0

Pso A3

(1.9)03 03 00 0 0 0 0 03 0 - 0 0 0 0 01 0 0 0 0 0 0

MCTD1

(.66)00 01 02 01 01 0 0 01 0 - 0 0 0 0 01 0 0 0 0 0 0

Others26

(17.4)17 14 03 01 00 02 00 09 03 02 02 01 01 01 01 0 0 0 0 0 0

Others include:Leukemia, Renal osteodystrophy, JAS, Angioneurotic oedema, IBD, Osteomyelitis, Wagners Granulomatosis, Reactive Arthritis, Rickets, Legg Calve Perthes disease, Morphea, Erythema Nodosum, TB Arthritis, ITP, Ricketshial Disease, Musculoskletal pain syndrome.DIS: Diseases; JIA: Juvenile Idiopathic Arthritis; KD: Kawasaki Disease; HSP: Henoch Schonlein Purpura; LN: Lupus Nephritis; S: Septic Arthritis; PAN: Polyartiritis Nodosa; Pso A: Psoriatic Arthritis; MCTD: Mixed Connective Tissue Disease. Figure within the parenthesis indicate percentage

Table 3: Investigation Findings of Pediatric Rheumatologic Disease Patients.Param-

eters VI MI MIN MAX MEA NOR INCR DECR TOTAL NOR ABN NN MH LEU LR THRO NS

TLC 135 24 4300 62000 11008 61(45.2)

55(40.7)

19(14.1)

PLAT 94 65 20000 620000 233893 89(94.7)

2(2.1)

3(3.2)

ESR 140 19 09 160 64.6 45(32.1)

95(67.9) 00(00)

CRP 49 21(42.9)

28(57.1)

ANA 61 39(63.9)

22(36)

RA 58 54(93) 4(6.9)

USG ABD 37 20

(54.1)17

(45.9)URIN R/E 123 85

(69.1)38

(30.4)

PBF 58 1(1.7)

35(60.3)

1(1.7)

3(5.2)

1(1.7)

17(29.3)

ASO 58 38(62.3)

23(37.2)

ECG 38 22(57.9)

16(42.1)

ECHO 35 22(62.9)

13(37.1)

VI: Valid Value; MI: Missing Value; MIN: Minimum; MAX: Maximum; MEA: Mean value; NOR: Normal; INCRE: Increased; DECR: Decreased; ABN: Abnormal; NN: Nor-mocytic Normochromic; MH: Microcytic Hypochromic; LEUK: Leukemic; LR: Leukemoid Reaction; THRO: Thrombocytopenic; NS: Non Specific; TLC: Total Leucocyte Count; PLAT: Platelet Count; ESR: Erythrocyte Sedimentation Rate; CRP: C- reactive protein; ANA: Anti Neuclear Antibody; RA: Rheumatoid Antibody; USG ABD: Ul-trasonography of Abdomen; URIN R/E: Urine Routine Examination; PBF: Peripheral Blood Film; ASO: Anti Streptolysin O Antibody; ECG: Electro Cardiograph; ECHO: Echocardiogram. Figure within the parenthesis indicate percentage

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Mamunur Rashid AKM, Islam T, Biswas P (2017) Pediatric Rheumatologic Diseases (PRDS) and its Clinical Scenario. J Muscle Health 1(1): 1003.

Cite this article

Figure 1 Showing type of JIA TypeTotal 55(100%)So JIA 13(23.6%), Polyarticular (Seropositive) 2(3.6%), Polyarticular (Seronegative) 14(25.5%)Oligoarticular 21(38.2%), ERA 1(1.8%), Psoriatic 3(5.6%), Unclassified 1(1.8%)

is the vasculitis in children which is frequent than HSP. Fever and joint symptoms/signs are the most frequent presentation in pediatric rheumatic diseases. Leucocytosis, raised ESR, positive CRP and MH anemia is commonly associated with PRDs.

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