A group of autoinflammatory disorders causing recurring episodes of fever and inflammation without infection.
✦ AI summary
Here's what these sources cover on periodic fever syndromes:
**Familial Mediterranean Fever (FMF)** appears most often. Research shows FMF carries a long-term excess risk of other rheumatologic and autoimmune conditions—including Behçet disease, rheumatoid arthritis, ankylosing spondylitis, lupus, vasculitis, and Crohn's disease—along with a much higher rate of amyloidosis, even in people treated with colchicine [5]. Kidney biopsy studies confirm amyloidosis is the most common kidney finding in FMF patients who need biopsies [1]. Children who develop FMF symptoms very early (age 2 or younger) tend to have more frequent fevers, longer delays to diagnosis, more severe disease, and more colchicine-resistant cases, often linked to a specific gene variant (M694V) [6]. One imaging study found that liver, spleen, and thyroid stiffness measurements didn't differ between children with FMF in remission and healthy children, even though blood markers showed ongoing low-level inflammation—suggesting this particular scanning method may not catch subtle organ effects [4].
**PFAPA syndrome** (periodic fever, mou
This is an AI-generated summary of the sources below — not medical advice. Always talk to a doctor or pharmacist about your own situation.
Evaluation of clinicopathological features of renal biopsies in non-lupus rheumatic diseases: a university hospital experience.
Renal involvement is a common condition in rheumatologic diseases. The aim of this study was to evaluate the clinical presentations and histopathological results in patients diagnosed with non-lupus rheumatic diseases who underwent renal biopsy. The records of 900 renal biopsies performed at our hos…
Assessing the proinflammatory potential of sterile fecal microbiome filtrate from ulcerative colitis patients using an intestine-on-chip platform and automated image analysis.
Ulcerative colitis (UC) is characterized by disruptions of the gut microbiome and an exaggerated mucosal immune response in genetically susceptible individuals. Alterations in the composition of the intestinal metabolome associated with dysbiosis can trigger chronic inflammation. However, it remains…
Long-term rheumatologic comorbidities in familial Mediterranean fever.
Familial Mediterranean fever (FMF) is characterized by pyrin inflammasome dysregulation and chronic subclinical inflammation. Whether FMF carries an increased long-term burden of rheumatologic and autoimmune inflammatory disease is unclear. We evaluated baseline and long-term occurrence of rheumatol…
Shear-wave elastography-based assessment of liver, spleen, and thyroid stiffness in children with Familial Mediterranean Fever in remission: a prospective case-control study.
To determine whether shear-wave elastography (SWE) can detect subclinical parenchymal changes related to chronic inflammation and AA amyloid risk by measuring liver, spleen, and thyroid stiffness in children with Familial Mediterranean Fever (FMF) in remission compared with healthy controls. In this…
Comparison of Clinical Features Between Early-onset (≤2 Years) and Later-onset Familial Mediterranean Fever.
The objective of this study was to investigate differences between familial Mediterranean fever (FMF) patients with symptom onset at aged 2 years or younger and those with later onset, with a focus on clinical presentation, disease course, and treatment outcomes. The medical records of FMF patients…
Seasonal and geographic variations in vitamin D levels among patients with familial Mediterranean fever: a systematic review and meta-analysis.
Familial Mediterranean fever (FMF) is an autoinflammatory condition caused by a single-gene mutation, inherited in an autosomal recessive pattern, and characterized by recurrent episodes of self-limited inflammation. Studies suggest that vitamin D levels may be lower in FMF patients, but evidence re…
The clinical significance of heterozygous E148Q variant in patients with familial Mediterranean fever.
OBJECTıVE: Our objective was to compare the clinical characteristics of heterozygous E148Q-positive familial Mediterranean fever (FMF) patients with those of E148Q/M694, M694V-homozygous, and M694V-heterozygous-positive patients. METHODS: Tel-Hashomer classification criteria were used to diagnose FM…