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Clinical and Instrumental Characteristic of Systemic Lupus Erythematosus in Children

V.V. Berezhnyi, T.V. Marushko, T.V. Taranenko, Ye.Yu. Marushko P.L.

Shupik National Medical Academy of Postgraduate Education, Kiev, Ukraine



The onset of SLE in children poses a diagnostic challenge for different health professionals, particularly because the onset of the disease shows no clear clinical symptoms and characteristically disguised under a variety of onset masks and highly aggressive course. Analysis of the disease onset allows identifying groups of the most frequent SLE onset masks in children. The SLE onset in children characterises in 21% cases with atypical or incomplete progress, in which case the disease progresses under the mask of other ailments giving grounds for erroneous diagnoses.
Keywords: systemic lupus erythematosus, children, onset mask, clinical and instrumental characteristic.

Introduction

Systemic lupus erythematosus (SLE) is a systemic inflammatory connective tissue disease (SICTD) characterised by immune tolerance failure and synthesis of a wide spectrum of antibodies, predominantly those to nuclear antigens of body's own cells, with subsequent development of immune complex vasculitis that ultimately affects multiple body systems. The disease throws down a challenge to many health professionals because the polysystemic nature of damage to health and variety of manifestations generally make long the way the patients have to go through from the onset to diagnosis that is often preceded by numerous consultations with a host of professionals in various fields of medical science. Correctly diagnosing the onset of the disease in children is a particularly complex task as it has no clear clinical symptoms, hides behind various onset masks, characteristically demon strates highly aggressive progress and leads to a reduction of average lifespan compared with that of patients who have contracted the disease in adult age.

The complexity of diagnostics and severity of SLE manifestations in children dictate the need for paediatricians' extra awareness in respect of the diagnosis. SLE prevalence among adult population world_ wide is between four to 250 cases (16.5 in Ukraine) per 100 thousand [4]. The morbidity among the paediatric population in the U. S. makes in average 0.53–0.6 per 100 thousand children a year [5] and the disease prevalence stands at 4.4 per 100 thousand for girls and 1–1.6 per 100 thousand for boys [14]. As of 2010, the SLE prevalence in Ukraine among children aged between 0 and 17 made two per 100 thousand children; noteworthy, it was in that year when some 32 children patients were first registered in the country [1].

According to Pelkonen et al. (1994), SLE ranks first among other SICTDs most prevalent in children population [13].

The aetiology of the disease remains unknown and studies into its pathogenesis continue. A theory considered the most advanced to date is the one of «predisposition threshold»; it postulates the existence of a certain threshold of action of environmental initiating factors that is conditioned by genetic predisposition and launches an autoimmune process as soon as is trespassed [16]. The process is characterised by immunisation mostly to nuclear antigens (e.g., gestones, native DNA, nucleosomes etc.) of one's own cells.

There exists conclusive evidence of the involvement of disturbed apoptosis in this phenomenon when the mentioned nucleus components, normally hidden from the immune system, become accessible for immune cells [12]. Insufficiency in the number and suppressor function of T cells together with polyclonal activation of B cells compel production of a great number of autoantibodies to the above autoantigens followed by formation of circulating immune complexes (CIC). Disruption of CIC elimination from the bloodstream is one of the key chains in SLE pathogenesis; it is caused by both reduced phagocytosis in such patients and by genetically conditioned defects of Fc immunoglobulin fragment receptors in CIC and the deficit of various complement components, most frequently of С3 and С4 [16]. Blood saturation with CIC leads to their deposition on vascular walls and subsequent development of vasculitis the localisation of which conditions the disease symptoms.


The SLE diagnosis is carried out based on the criteria set out by the American College of Rheumatology (ACR) developed in 1982 (ACR1982) and reviewed in 1997 (ACR1997) when four or more criteria are available (Table 1) [10, 17]. These criteria, while designed as classification ones, are widely used worldwide as diagnostic ones.

The presented criteria are rather sensitive and specific: e.g., the sensitivity and specificity of SLE ACR1997 diagnostic detection in adults reaches 96%. Using the ACR1982 criteria (that differed from the above only in the ascertaining of LE cells availability and not included detection of lupus anticoagulant and anticardiolipin antibodies) for diagnosing children produced 96% and 100%, respectively (though the validity of ACR1997 for children has not been confirmed to date) [8].

Nevertheless, the presented characteristics are suitable for applying the mentioned criteria during a
Full-blown SLE. According to L. Hiraki et al (2002), the onset of the disease in children can characterize with lesser number of symptoms the list of which increases with disease progress [7]. The frequency of changes in various systems during SLE onset in children established in various researches in different years is shown in Table 2. Interestingly, the most expressive dynamics as regards reduction of frequency of manifestations at the disease onset can be observed in the heart. The absence of information about Libman—Sacks disease detection in children with SLE onset in the researches of 2002 and 2005 draws particular attention [2,7,11].



As the international rheumatologic experience shows, at the onset of the disease two groups, one with incomplete SLE and another, with SLE with atypical beginning, can be singled out from among the patients compliant with ACR1997 criteria.

The incomplete SLE is understood to have less than four diagnostic criteria that generally represent a combination of clinical signs with increased titres of antinuclear antibodies (ANA) and/or antibodies to double-stranded DNA (AB to DS DNA) [11]. French researchers B. Bader—Meunier, J.B. Armengaud, E. Haddad et al. (2005) during their multi-centre study of SLE onset characteristics in 155 children noted that the disease began in incomplete form in 18 (11.6%) children. Herewith, the symptoms included cytopenia, articular syndrome and nephropathy and were followed by increased levels of ANA and AB to DS DNA [11]. A study carried out in Sweden by C.S. Hallengren, О. Nived, G. Surfeit (2004) showed that the progress of the incomplete SLE into a «complete» form in a half of the patients with «incomplete» onset took 5.3 years in average. At that, the predictors for such catamnesis included «butterfly» eruptions and presence of anticardiolipin antibodies [9].

 The atypical SLE form is diagnosed when the onset of the disease shows, first and foremost, clinical signs that do not belong to ACR1997 diagnostic criteria but have nevertheless been described in literature as associated with the disease (fever, generalised lymphadenopathy, hepatosplenomegaly, haemorrhagic or punctuate eruptions) [15]. According to research data, the prevalence of atypical SLE onset in children makes 24–43.4% of all cases [2, 11, 15]. The SLE with atypical onset has drawn particular attention of late because of the findings of A. Taddio, E. Rossetto and C.D. Rose (2010) who showed that atypical disease manifestations at onset in children are related with poorer prognosis in their catamnesis [15]. We would like to mention it here that, while fever and raised inflammatory markers in blood do not belong to ACR1997 criteria, we do not deem them atypical signs as we do know from our personal experience that they are practically commonplace at SLE onset in children and yet also characteristic for a great number of other diseases of not only autoimmune but also of infectious and haematological nature and hence are utterly non-specific which is why they are not included in either ACR1982 or ACR1997.

The assessment of SLE onsets in the forms of incomplete or atypical variant has led to the singling out of a group of most frequent masks. A mask is the progress of a disease in which its clinical findings resemble those of another ailment and thus often lead to a misdiagnosis. SLE masks in children were studied in a rather detailed manner by Russian researches L. A. Isayeva and N. S. Podcherniayeva. According to their data, an atypical SLE progress in children during the onset phase under the mask of other diseases was registered in 43.4% cases, which roughly corresponds to data from other researches [11, 15]. The most frequent (16.1%) mask was the SLE onset under the disguise of glomerulonephritis (GN) with nephritic, nephritic or isolated urinary syndrome. GN as SLE onset mask is characterised by malignant and rapidly progressing course. The haemolytic anaemia mask (5.9%) and the one of haemorrhagic vasculitis (5.1%) took the second and the third place, respectively.

Patients with haemolytic anaemia generally had ochrodermia, hepatosplenomegaly and increased serum level of fre bilirubin. The haemorrhagic vasculitis mask showed haemorrhagic eruptions, mostly on the frontal surface of the lower leg, that was frequently connected to arthritis/arthralgias, abdominal syndrome and nephropathy in the form of haematuria, leukocyturia and/or proteinuria. Herewith the above haematological signs were characterised mostly with medium severity of progress, absence of complications and high-level response to glucocorticoid therapy. The isolated articular syndrome at the onset that forged the mask of juvenile arthritis was observed in 4.7% patients; herewith, the arthritis was of nonerosive type. Facial dermatitis without any other SLE manifestations at the onset of the disease was observed in 4.6% patients, mostly boys. Herewith, a considerable aggravation of dermatitis after sun exposure and subsiding of its intensity in autumn and winter became the key clue to diagnosis. Other SLE onset masks in children were: idiopathic thrombocytopenic purpura (3.8%); isolated Raynaud disease (1.3%), isolated chorea (1.1%); epilepsy syndrome (0.8%) [2]. Besides that, the authors in their earlier publications noted a «lymphoblast» mask of the disease in the form of generalised lymphadenopathy, hepatosplenmegaly, a considerable increase of ESR and leukogram changes in 1.7–5% SLE onsets in children. For differentiated diagnosis the sternal puncture was used; herewith, no changes specific for haematological diseases except for responsive ones were found in biopsy samples. Acute rheumatic fever was another yet possible SLE onset mask found in children: this type of disease progress showed carditis at different stages combined with the articular syndrome, sometimes with chorea and/or erythema annulare. At that, the level of Antisteptolysin O was generally within normal ranged provided the absence of associate infection with A-type â-haemolytic streptococcus; in presence of the latter, the task of making differentiated diagnosis became much more complicated [3]. A stable SLE's prevalence under masks of different other diseases has been noted by the authors across 25 years-long (from 1961 till 1985) period when 506 SLE cases in children were analysed (Table 3).



Quite another picture was observed in the survey by B. Bader—Meunier et al (2005): according to its data, atypical SLE onset progression in children was found in 32% cases; notably, in half the instances the disease masked under «surgical abdomen» thus leading to admittance to surgery ward and, in some cases, to laparotomy [11].

Besides the abovementioned most frequent SLE mask variants, the literature offers one-of-a-kind notices of their more rare forms, like Guillain—Barre syndrome, coagulopathy, renal artery thrombosis, polyomyositis, rheumatic nodules on vocal cords, pericarditis with heart tamponade, cardiac failure, complete heart block, ascites, excudative pleurisy, polymyalgia rheumatica, cryoglobulinaemia, cyclic reverse granulocytopenia, derated immunoglobulin E syndrome, optic neuritis and Bell's palsy, and palsies of VIth and VIIIth pairs of cranial nerves. Tuberculosis, yersiniosis and malignant tumour masks that simulate SLE are also known [3].

Meningoencephalitis is a mask with which the speed with which a correct diagnosis has been made determines the survival prognosis for a patient. In these cases, lumbar puncture to detect lymphocytic pleocytosis, anti-nuclear factor and AB to DS DNA in cerebrospinal fluid is conducive to correct diagnosis. During the culture test, the liquor is sterile [3].

In 2009, Charuvanij and Houghton were the first in the history of medical science to publish their report on a five-year-old girl who had SLE onset under the mask of acute epiglottitis, X-ray and laryngoscopy test-confirmed [6].

Both data in literature and our own personal experience show that SLE can manifest with symptoms of acute pancreatitis [5]. O. Richer, T. Ulinski (2007) present data on prevalence of the mentioned onset type giving it 6% of all atypical SLE onset cases. In that case, a girdle-type pain, multiple vomiting with out any alleviation, constipation or diarrhoea without pathological admixtures in faeces and intoxication symptoms developed on the background of hyperthermia. In presence of the above clinical manifestations, pancytopenia with pronounced leucopenia and raised ESR were found in complete blood count results and chemistry blood results showed the normal level of serum alpha-amylase and higher seromucoid, CIC, CRP, ANA and AB to DS DNA levels.

Small vessel vasculitis of the pancreas is deemed the reason for the diagnosis [5].

According to the data in literature and our own personal observations, the onset of the disease may include alopecia that, in absence of general clinical signs of a systemic inflammatory process, compels patients to seek dermatological assistance [5]. On patient examination diffuse alopecia or alopecia areata are found; head skin changes remind seborrheic dermatitis but differ from it with hyperkeratosis of the follicles and ulerythema that eventually lead to a steady loss of hear (cicatrical alopecia). Lab tests reveal symptoms of a systemic autoimmune inflammation (increased total blood protein seromucoid, SRP, ANA, AB to nucleosomes and DS DNA levels). Besides, there are mentions in literature of SLE onset in the form of thrombophlebitis of superficial veins of lower extremities. We have noted one case of such similar manifestation when thrombophlebitis was not the only sign and when general clinical, haematological and immunological findings fit well in ACR1997 diagnostic criteria.

Neonatal SLE merits particular attention: in such cases the newborn may inherit from his SLE-affected mother a «butterfly» form of erythema, heart beat disorder (down to complete atrioventricular heart block) and, in rarer cases, renal injury, hepatosplenomegaly etc. The mechanism of such signs development comprises transplacental transmission of autoantibodies from mother's blood stream into that of the foetus [5].

The presented data confirm that SLE in its atypical progress form may demonstrate high levels of mimicry to other pathologies in a wide range of medical spheres and hence, poses a diagnostic challenge to any health professional. With a view of that, studies of clinical variants of SLE onset in children surely remains a highly relevant issue for paediatrics and paediatric cardiorheumatology.

Having regard to the above data, the objective of our work has concerned the assessment of prevalence of clinical manifestations in various systems of children with SLE onset.


Research Material and Methods

We retrospectively analysed patient histories andabstracts of hospital records of some 19 children with SLE onset who had been admitted to KMCH 1 Cardiorheumatological Care Unit in 1999 through 2012. Studies of further disease progress in the above children found that all of them complied with four or more ACR1997 criteria already on the onset or later on. The average age of the patients was 13.2 years. There were 16 girls and three boys with girls to boys ratio at 5.3:1. The degree of disease activity was between 2nd and 3rd level. The average disease duration from the onset until diagnosis was 23 days (from 12 to 42 days). At admission, all the patients were checked for complaints and anamnesis. Furthermore, they underwent physical examination and had CBC and urine tests, coagulation profile, blood chemistry and rheumatological (antistreptolysin О and С reactive protein (CRP) titres; presence of rheumatoid factor; circulating immune complex (CIC) levels) tests; ANA and AB to DS DNA tests; checks for presence of antiphospholipid antibodies, electrocardiogram (ECG) and Doppler echocardiogram (DECG); ultrasonography of abdominal cavity organs and kidneys carried out. Additional examinations and specialty consultations: Zemnitsky's urinalysis, Nechiporenko's test, 24-hour urinary protein, articular ultrasonography, Holter ECG monitoring, and consultation of nephrologists were administered on an as-necessary basis.




Research Results and Their Discussion

The clinical, lab and instrumental characteristic of children with SLE onset is presented in Table 4. For the sake of comparison, that same Table also shows the prevalence of signs in various organs and systems for the whole period until the diagnosis was made.


References
  1. Berejniy V.V., Marushko Т.V., Romankevich I.V. Stan nadannya kardiorevmatologichnoi dopomogy dityam Ukrainy za 2009 r. Sovr. pediatriya. 2010; No. 5 (33): 14—18.
  2. Detskaya revmatologiya: [ruk_vo dlya vrachey] / pod red. A.A. Baranova, L.K. Bajhenovoy. M.: Meditcina. 2002: 336.
  3. Svinitc'kiy A.S. Suchasni pidhodi do diagnostiky ta likuvannya systemnogo chervonogo vovchaka. Zdorov'e Ukraini. 2008; No. 21/1: 66—69.
  4. Cassidy J.T., Petty R.E. Textbook of Pediatric Rheumatology. 5th ed. Philadelphia: W.B. Saunders Company. 2005: 792.
  5. Charuvanij S., Houghton K.M. Acute epiglottitis as the initial presentation of pediatric Systemic Lupus Erythematosus. Pediatric Rheumatology. 2009; 7: 19—23.
  6. Hiraki L., Schneider R., Hebert D. [et al.] Clinical and laboratory manifestations of systemic lupus erythematosus in pediatric patients. Arhritis and Rheumatism. 2002; 46: 315.
  7. Ferraz M.B., Goldenherg J., Hilario M.O. [et al.] Evaluation of the 1982 ARA lupus criteria data set in pediatric patients. Clinical and Experimental Rheumatology. 1994; 12: 83—87.
  8. Hallengren C.S., Nived O., Surfeit G. Outcome of incomplete systemic lupus erythematosus after 10 years. Lupus. 2004; 13: 85—88.
  9. Hochberg M. C. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus / M. C. Hochberg // Arthritis and Rheumatism. — 1997. — Vol. 40. — P. 1725.
  10. Bader—Meunier B., Armengaud J.B., Haddad E. [et al.] Initial presentation of childhood-onset systemic lupus erythematosus: A French multicenter study. The Journal of Pediatrics. 2005; 146, Issue 5: 648—653.
  11. Pediatrics in Systemic Autoimmune Diseases. Edited by Cimaz R. and Lehman T. — Elsevier B.V., 2008: 273.
  12. Pelkonen P.M., Jalanko H.J., Lantto R.K. Incidence of systemic connective tissue diseases in children: nationwide prospective study in Finland. Journal of Rheumatology. 1994; 21 (11): 2143—2146.
  13. Lehman T.J., McCurdy D.K., Bernstein B.H. [et al.] Systemic lupus erythematosus in the first decade of life. Pediatrics. 1989; 83 (2): 235—239.
  14. Taddio A., Rossetto E., Rose C.D. Prognostic impact of atypical presentation in pediatric systemic lupus erythematosus: results from a multicenter study. Journal of Pediatrics. 2010; 156 (6): 972—977.
  15. Textbook of pediatric rheumatology. Edited by James T. Cassidy, Ross E. Petty. 5th ed. Elsevier Inc., 2005: 792.
  16. Tan E.M., Cohen A.S., Fries J.F. [et al.] The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis and Rheumatism. 1982; 25: 1271—1277.

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