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SAT0271 Hepatosplenomegaly: A Complication of Persistent Subclinical Inflammation in Children with Familial Mediterranean Fever?
  1. E. Comak1,
  2. G. Duygulu2,
  3. E. Isıyel3,
  4. F. Kılıçbay4,
  5. M. Koyun1,
  6. S. Akman1
  1. 1Pediatric Nephrology - Rheumatology, Akdeniz university, Antalya
  2. 2Radiology
  3. 3Pediatric Nephrology - Rheumatology
  4. 4Pediatrics, Kocaeli Derince Training and Research Hospital, Kocaeli, Turkey

Abstract

Background Familial Mediterranean Fever (FMF) is a hereditary autoinflammatory disorder characterized by attacks of painful inflammation. Some patients with FMF have subclinical inflammation persisting between the attacks. This subclinical inflammation may leads to many important clinical complications such as anemia, splenomegaly, decreased bone density, and amyloidosis.

Objectives The aims of this study were to evaluate sonographic findings in patients with familial Mediterranean fever (FMF) during attack-free periods and examine whether there is an association between hepatosplenomegaly and inflammatory markers in FMF.

Methods Medical records of children with FMF were reviewed during attack-free periods. Demographic features, clinical and laboratory findings, hematological data (leukocyte, hemoglobin and platelet counts),erythrocyte sedimentation rate (ESR), SAA and C-reactive protein (CRP) levels, type of MEFV mutation, family history of FMF were collected from the hospital's computerized database following approval by the local ethics committee.

Results A total of 136 patients, 74 (54.4%) girls were included in this cross sectional study during non-attack period. Median age of 11.25 years (2–18 years) and median follow-up period were 18 months (3–129 months) at time of sonographic evaluation. Thirty one patients (22.8%) had parental consanguinity and 472 (52.9%) patients had family history of FMF. Thirty three patients (24.3%) patients were homozygous, 61 (44.9%) patients were compound heterozygous and 42 (30.9%) patients were heterozygous for MEFV gene. Hepatomegaly and splenomegaly were found in 22 (16.2%) and 32 patients (23.5%), respectively. Ten patients had both hepatomegaly and splenomegaly. Spleen size had a negative correlation with platelet count and a positive correlation with ESR, CRP and SAA (all p<0.05). Also, there was significant positive correlation between liver size and CRP levels (p<0.05). White blood cell count, hemoglobin, urinary protein-creatinine ratio were not associated with spleen and liver size. And also, patients with splenomegaly had significantly higher SAA, CRP and ESR levels than the patients with normal spleen size (Table 1).

Table 1.

Laboratory findings of patients

Conclusions Hepatomegaly and splenomegaly are not uncommon in FMF, which might be due to the chronic inflammatory characteristics of the disease. We speculate that FMF is a chronic inflammatory disease rather than episodic illness.

Disclosure of Interest None declared

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