Different disease subtypes with distinct clinical expression in familial Mediterranean fever: results of a cluster analysis

Abstract

Patients with FMF can be clustered into distinct patterns of clinical and genetic manifestations and these patterns may have different prognostic significance.

Nationwide Experience With Off‐Label Use of Interleukin‐1 Targeting Treatment in Familial Mediterranean Fever Patients

“…The demographic and some clinical characteristics of the patients are summarized in Table . The frequencies of peritonitis, pleuritis, and protracted febrile myalgia were lower in our study group than in ordinary Turkish FMF patients whom we have described previously . However, age at symptom onset was somewhat earlier and the presence of the M694V allele, a family history of FMF, and amyloidosis were more frequent in patients who used anti–IL‐1 therapy (Table ).…”

“…Familial Mediterranean fever (FMF) is an autoinflammatory disease characterized by self‐limited episodes of febrile serosal inflammation . The disease is most frequently seen in Turks, non‐Ashkenazi Jews, Armenians, and Arabs.…”

“…Since 1972, colchicine has become the mainstay of treatment for patients with FMF because continuous colchicine treatment is not only effective in prevention and amelioration of FMF attacks , but also in decreasing the frequency of amyloidosis . However, approximately 30–45% of FMF patients have been reported to have had attacks despite colchicine treatment . Currently, data on the treatment of colchicine‐unresponsive or colchicine‐intolerant FMF patients are limited.…”

Exon 2: Is it the good police in familial mediterranean fever?

“…Some studies suggested that patients with ELE had a more severe clinical course and were also related to M694V homozygosity and amyloidosis (8). In a previous study, researchers showed that amyloidosis, arthritis, family history of FMF, and presence of the M694V allele had been clustered in a severe outcome group (9). In this study, we showed that patients with isolated exon 2 mutations had a higher percentages of females, late onset of disease, lower rates of self, and a family history of amyloidosis, ELE, and arthritis compared to patients with exon 10 mutations.…”

Second Metatarsal Transfer Lesions Due to First Metatarsal Shortening After Distal Chevron Metatarsal Osteotomy for Hallux Valgus

“…We categorized the 185 feet according to the extent of postoperative shortening of the first metatarsal, relative to the second metatarsal, as measured by Hardy-Clapham's method: group A, postoperative shortening of first metatarsal <2.5 mm; group B, shortening of 2.5-5 mm; and group C, shortening of >5 mm. Cluster analysis 2,7,9,15 was used to determine whether the amount of postoperative shortening of the first metatarsal by Hardy and Clapham's method could be used to assign patients to different groups. To do this, the cutoff of postoperative shortening of the first metatarsal by Hardy and Clapham was set at 2.5 and 5 mm.…”