Relapsing polychondritis

Diagnosis

Relapsing polychondritis

History

Study requested to rule out occult infection and assess disease activity in a thirteen year old boy before bone marrow transplantation.

Findings

Multiple foci of FDG uptake including ears, larynx, trachea and bronchial tree, costal cartilage, knees as well as right ankle and foot. Calcifications of laryngeal structures and costal cartilage. Tracheostomy. Mild spleen activity probably related to the inflammatory process.

DDx

Relapsing polychondritis

Sarcoïdosis

Wegener’s granulomatosis

Discussion

Relapsing polychondritis (RP) is a rare systemic disease of unclear etiology characterized by recurrent inflammation of cartilaginous tissues including in particular external ear, nose, peripheral joints, larynx and tracheobronchial tree. Development of autoimmunity against cartilage components is tought to be the pathogenic mechanism.

RP is more common in caucasian adults during the fourth or fifth decades. One study estimates that pediatric-onset RP represents <5% of the reported cases. The clinical manifestations are variable but commonly imply auricular chondritis, arthritis, nasal chondritis and costal chondritis. The features are similar between adults and children One report identifies a higher incidence and severity of laryngotracheobronchial involvement in children, with greater need of tracheostomy.

Roles of PET/CT FDG

Several authors have demonstrated utility of PET/CT FDG for diagnosis and monitoring of the therapeutic response. It is a powerful tool for early diagnosis and to assess distribution of lesions. Correlation between findings on PET/CT FDG and disease activity on a clinical basis has been reported. Metabolic activity is thought to be related to inflammatory cells such as neutrophils, activated macrophages, and lymphocytes, who take up large amount of glucose as a result of an increased metabolic rate.

References

Belot et al : Pediatric-onset relapsing polychondritis: case series and systematic review. J Pediatr. 2010 Mar;156(3):484-9

Sharma et al : Relapsing polychondritis: clinical presentations, disease activity and outcomes. Orphanet J Rare Dis. 2014 Dec 20;9:198

Puéchal et al : Relapsing polychondritis. Joint Bone Spine. 2014 Mar;81(2):118-24

Knipp et al : Relapsing polychondritis in childhood--case report and short review. Rheumatol Int. 2000;19(6):231-4

Fonseca et al : Relapsing polychondritis in childhood: three case reports, comparison with adulthood disease and literature review. Rheumatol Int. 2013 Jul;33(7):1873-8

Sato et al : F-18 FDG PET/CT in relapsing polychondritis. Ann Nucl Med. 2010 Nov;24(9):687-90

Wang et al : ¹⁸F-FDG PET/CT is a valuable tool for relapsing polychondritis diagnose and therapeutic response monitoring. Ann Nucl Med. 2014 Apr;28(3):276-84

Deng et al : Relapsing polychondritis on PET/CT. Clin Nucl Med. 2012 Jul;37(7):712-5

De Geeter et al : Fluorodeoxyglucose PET in relapsing polychondritis. N Engl J Med. 2008 Jan 31;358(5):536-7

Yamashita et al : Utility of fluorodeoxyglucose positron emission tomography/computed tomography for early diagnosis and evaluation of disease activity of relapsing polychondritis: a case series and literature review. Rheumatology (Oxford). 2014 Aug;53(8):1482-90

Bayer et al : Utility of 18F-FDG PET/CT in relapsing polychondritis. QJM. 2015 Apr;108(4):339-40

Honne et al : Fluorodeoxyglucose positron emission tomography/computed tomography for diagnostic imaging in relapsing polychondritis with atypical manifestations. Clin Rheumatol. 2013 Mar;19(2):104-5