The effect of colchicine and disease severity on physical growth in children with familial Mediterranean fever

Abstract

This study aimed to investigate the effects of colchicine on growth parameters in familial Mediterranean fever (FMF) patients. Fifty-one (29 girls, 22 boys) FMF patients were enrolled in the study. All of the patients were in the prepubertal stage and had not received colchicine treatment before the study. Anthropometric measurements, demographic features, clinical findings at diagnosis and during periods of attacks of FMF, disease activity, frequency of exacerbations, colchicine dosage, and weight and height measurements were recorded at an interval of 6 months. Height, weight, and body mass index standard deviation scores and Z-scores were calculated. The mean height standard deviation score (HSDS) was significantly increased from -0.64 ± 1.20 to -0.26 ± 1.07 (p < 0.001), the mean weight standard deviation score (WSDS) was significantly increased from -0.60 ± 1.03 to -0.45 ± 0.98 (p = 0.008), and the mean body mass index standard deviation score was decreased from -0.33 ± 1.06 to -0.47 ± 0.98 (p = 0.128) at 1 year after colchicine treatment compared with before initiation of treatment. In patients who had no FMF attacks during colchicine treatment, height and weight were significantly increased at 1 year (HSDS: p < 0.001 WSDS: p = 0.002), but in patients who had recurrent attacks, height and weight did not change (HSDS: p = 0.051, WSDS: p = 0.816). Even when subclinical inflammation is present, preventing attacks of FMF with colchicine allows growth to continue. However, suppression of subclinical inflammation and control of attacks of FMF are required for weight gain.

Disease Severity and Genotype Affect Physical Growth in Children With Familial Mediterranean Fever

“…8 Two recent studies also showed that disease severity was not correlated with growth parameters; however, these data were obtained from small numbers of patients. 9,10 Disease severity assessment may be helpful in daily practice in terms of predicting the prognosis and guidance of patients. There are mainly two other severity scoring systems, conducted by Mor et al 20 and Pras et al, 21 in addition to Tel Hashomer severity scoring system.…”

“…Moreover, several studies including limited numbers of patients addressed the question whether physical growth is affected by genotype and disease severity or not. [5][6][7][8][9][10] However, all these studies utilized an ancient Tel Hashomer severity scoring system, which was not validated in the pediatric population. 11 Thus, in this study, we aimed to analyze the growth parameters in children with FMF according to disease characteristics including genotype and disease severity by a recently validated tool in relatively more patients.…”

Conventional and novel therapeutic options in children with familial Mediterranean fever: A rare autoinflammatory disease

“…recently assessed 51 prepubertal FMF children: the mean height Z‐score significantly increased from −0.64 to −0.26 at 1 year after starting colchicine, compared to the pretreatment period. Importantly, this improvement was substantially absent in those children who had recurrent attacks 50 …”

“…Importantly, this improvement was substantially absent in those children who had recurrent attacks. 50 Even though one may expect puberty to be delayed in untreated patients with poor controlled disease, there are no specific studies investigating timing of pubertal onset in patients with FMF; it is therefore not currently possible to evaluate the role of colchicine in potentially improving this issue. 51…”

The clinical and mechanistic intersection of primary atopic disorders and inborn errors of growth and metabolism

“…It is likely that chronic allergic inflammation impairs growth secondarily. Certainly, chronic and recurrent autoinflammation has been shown to negatively impact linear growth, and this impairment can be mitigated with corticosteroid‐sparing immunosuppressive therapy . However, a number of Mendelian disorders have provided additional mechanistic insight into ways in which specific pathway defects that result in atopy may also negatively impact growth.…”