Spondyloarthritis in familial Mediterranean fever: a cohort study

Kasman, Sevtap Acer; Duruöz, Mehmet Tuncay

doi:10.1007/s00296-022-05158-5

Cited by 8 publications

(4 citation statements)

References 44 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…M694V carriers often have severe disease, especially in the homozygous individual, with multisite involvement during attacks and a high risk of amyloidosis [ 23 ]. Standing myalgia and enthesitis are related to severe presentation of the disease [ 8 , 9 ]; however, in our study, none of the patient and disease-related parameters was associated with pulmonary findings on thorax CT.…”

Section: Discussioncontrasting

confidence: 75%

“…[ 2 , 5 ]. Features such as myalgia, persistent arthritis, spondyloarthropathy, and enthesitis are associated with persistent inflammation, high disease scores, and severe serositis attack features, as seen in M694V mutation carriers [ 6 – 9 ]. Thus, as expected, systemic involvement and associated persistent chronic inflammation may be associated with AA amyloidosis in FMF [ 10 ].…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Apical fibrosis was the most common incidental pulmonary finding in a familial Mediterranean fever cohort

et al. 2023

Self Cite

View full text Add to dashboard Cite

Introduction Familial Mediterranean fever (FMF) is one of the common autoinflammatory diseases with multisystemic manifestation. Pleuritis is the only known pulmonary involvement of FMF; however, as far as we know, thoracic involvements in pleural, parenchymal, bronchial, and vascular structures have not been evaluated yet. Method We included 243 consecutive FMF patients who applied to our clinic within the last 5 years and were requested to have a thorax CT for any reason and 122 trauma patients without any comorbidity. An experienced radiologist evaluated the thorax CT images blindly according to the relevant guidelines. We then presented the common incidental pulmonary and mediastinal findings on the thorax CT. Additionally, we compared patients with and without lung involvement according to demographic and disease-related parameters. Results In our study, 167 of 243 patients (68.7%) had at least one of the pulmonary findings on their thorax CT. The most common pulmonary findings were apical fibrosis in 96 (39.5%) patients, parenchymal fibrotic changes in 48 (19.8%) patients, and a solitary parenchymal nodule smaller than 4 mm in 33 (13.6%) patients. All demographic, genetic, and disease-related characteristics, including the frequency of spondyloarthropathy, were similar in patients with and without pulmonary findings. Conclusions We showed that the most common incidental pulmonary finding in our FMF cohort was apical fibrosis on thoracic CT. Our data did not show causality between FMF and apical fibrosis; therefore, more studies are needed to evaluate the frequency and clinical significance of apical fibrosis in FMF. Key Points • More than two-thirds of familial Mediterranean fever (FMF) patients in our study group who underwent a thoracic scan for any reason had pulmonary and mediastinal findings on thorax computed tomography (CT) . • In our FMF cohort, the most common incidental pulmonary finding on their thorax CT was apical fibrosis . • All demographic and disease-related characteristics, including the frequency of spondyloarthritis, were similar between patients with and without pulmonary and mediastinal findings .

show abstract

Section: Discussioncontrasting

confidence: 75%

Section: Introductionmentioning

confidence: 99%

Apical fibrosis was the most common incidental pulmonary finding in a familial Mediterranean fever cohort

et al. 2023

Self Cite

View full text Add to dashboard Cite

show abstract

“…Besides vasculitis, patients with FMF could have an increased prevalence of various immune-mediated conditions, including spondyloarthritis ( 27 , 28 ), psoriasis ( 29 ), hidradenitis suppurativa ( 30 , 31 ), juvenile idiopathic arthritis ( 32 , 33 ), multiple sclerosis ( 34 ), and inflammatory bowel disease ( 35 , 36 ). The pathogenic mechanism of these associations remains unknown, particularly the role of MEFV mutations ( 37 – 42 ).…”

Section: Discussionmentioning

confidence: 99%

Vasculitis and familial Mediterranean fever: Description of 22 French adults from the juvenile inflammatory rheumatism cohort

Abbara

Monfort²,

Savey³

et al. 2022

Front. Med.

View full text Add to dashboard Cite

ObjectiveThe frequency of vasculitis may be increased in patients with Familial Mediterranean Fever (FMF), according to several studies. Our aim was to assess the characteristics of French adult patients with both diseases.MethodsPatients with vasculitis were selected from patients followed for FMF in the French JIR-cohort.ResultsTwenty-two patients were included [polyarteritis nodosa (PAN) n = 10, IgA vasculitis n = 8, unclassified vasculitis n = 2, granulomatosis with polyangiitis n = 1, and microscopic polyangiitis n = 1]. Pathogenic mutations in exon 10 were found in all 21 patients (96%) for which MEFV testing results were available, and 18 (82%) had two pathogenic mutations. Histology showed vasculitis in 59% of patients. Most patients with FMF-associated PAN were HBV-negative and had an inactive FMF before PAN onset, and 40% had a peri-renal or central nervous system bleeding. Most patients with FMF-associated IgA vasculitis had an active FMF before vasculitis onset, and 25% had digestive bleeding. Both patients with unclassified vasculitis had ischemic and/or hemorrhagic complications.ConclusionThis study confirms the predominance of PAN and IgA vasculitis in patients with FMF and the high frequency of bleeding in FMF-associated PAN. FMF should be considered in case of persistent symptoms and/or inflammatory syndrome despite vasculitis treatment in Mediterranean patients.

show abstract

“…When we discovered apical fibrosis as the most common thorax CT sign, we needed to differentiate patients with SpA because apical fibrosis is commonly seen in SpA. Furthermore, SpA and FMF exhibits several common findings [2]. That is why we did not ignore the SpA characteristics of the patients.…”

Section: Dear Editormentioning

confidence: 99%

Authors’ response: Main article "Apical fibrosis was the most common incidental pulmonary finding in a familial Mediterranean fever cohort"

2023

Self Cite

View full text Add to dashboard Cite

Spondyloarthritis in familial Mediterranean fever: a cohort study

Cited by 8 publications

References 44 publications

Apical fibrosis was the most common incidental pulmonary finding in a familial Mediterranean fever cohort

Apical fibrosis was the most common incidental pulmonary finding in a familial Mediterranean fever cohort

Vasculitis and familial Mediterranean fever: Description of 22 French adults from the juvenile inflammatory rheumatism cohort

Authors’ response: Main article "Apical fibrosis was the most common incidental pulmonary finding in a familial Mediterranean fever cohort"

Contact Info

Product

Resources

About