Relapsing Polychondritis Complicated by Giant Cell Myocarditis and Myositis

Watanabe, Mari; Suzuki, Haruo; Ara, Tomoka; Nishizuka, Midori; Morita, Mami; Sato, Chisa; Tsuchida, Fumihiro; Takeda, Hiroaki; Kato, Tomoya

doi:10.2169/internalmedicine.52.9080

Cited by 8 publications

(7 citation statements)

References 11 publications

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“…RP is a rare inflammatory chondropathy in multiple cartilages that is not readily diagnosed in the absence of typical clinical findings [2, 20, 27–31]. The diagnosis of RP is still based on clinical grounds [4, 5], and histologic confirmation is not required.…”

Section: Discussionmentioning

confidence: 99%

Is 18F-FDG PET/CT useful for diagnosing relapsing polychondritis with airway involvement and monitoring response to steroid-based therapy?

Zeng

Chen

et al. 2019

Arthritis Res Ther

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Background18F-fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) is a promising tool for diagnosing relapsing polychondritis (RP). However, its usefulness in assessing RP with airway involvement is unknown.ObjectiveThis study aimed to further evaluate and confirm the potency of 18F-FDG PET/CT in diagnosing RP with airway involvement and monitoring response to steroid-based therapy.MethodsA total of 30 patients from a dedicated respiratory centre, diagnosed with RP in accordance with McAdam, Damiani or Levine criteria, were included in this study. All patients underwent baseline 18F-FDG PET/CT, and 10 patients underwent second scans after 2.5–15 months of steroid-based therapy. Visual scores (VS) and maximal standard uptake values (SUVmax) were analysed.ResultsIn the initial scan, 83.3% (25/30) of patients were found to have FDG uptake in more than one cartilage. The median VS and SUVmax in the cartilages were 3 (range, 1–3) and 3.8 (range, 1.9–17.9), respectively. Positive rates for PET/CT-guided biopsy in nasal, auricular, and tracheal/bronchial cartilages were 100% (5/5), 88.9% (8/9), and 10.5% (2/19), respectively, but the positive biopsy rate in the auricular cartilage was 92.3% (12/13) even without PET/CT assessment. Based on biopsy-proven sites, the sensitivity of PET/CT was 55.6%, and the specificity was 5.3%. Compared with the baseline scan, the second scan showed much lower median VS (2 vs 3, respectively; p < 0.0001) and SUVmax (2.9 vs 3.8, respectively; p < 0.001). Of 10 patients who underwent second PET/CT, 8 had complete therapeutic response, while 2 had partial response.Conclusion18F-FDG PET/CT assists in identifying multiple cartilage involvement in RP, but it seems neither a sensitive nor specific modality in diagnosing RP with airway involvement. Moreover, PET/CT has limited utility in locating biopsy sites and monitoring therapeutic response to corticosteroids.

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Section: Discussionmentioning

confidence: 99%

Is 18F-FDG PET/CT useful for diagnosing relapsing polychondritis with airway involvement and monitoring response to steroid-based therapy?

Zeng

Chen

et al. 2019

Arthritis Res Ther

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“…RP is a rare inflammatory chondropathy in multiple cartilages that is not readily diagnosed in the absence of typical clinical findings [2,17,[25][26][27][28][29]. The diagnosis of RP diagnosis is based on clinical criteria, such as those described by McAdam et al [4] and on a biopsy of affected cartilage.…”

Section: Discussionmentioning

confidence: 99%

WITHDRAWN: A re-evaluation of 18F-FDG PET/CT for diagnosing relapsing polychondritis and monitoring the therapeutic response to treatment

Wang¹,

Li²,

Chen³

et al. 2018

Oncotarget

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Background: Relapsing polychondritis (RP) is a rare degenerative disease and its diagnosis is often delayed and difficult. Although the use of 18 F-fluorodeoxyglucose positron emission tomography/computed tomography (18 F-FDG PET/CT) for diagnosing RP has been reported previously, its true diagnostic value is uncertain due to the limited number of published case reports. Moreover, its value for biopsy guidance and therapeutic response monitoring remain controversial. Materials and Methods: Data on 30 patients with a clinical diagnosis of RP who underwent PET/CT scans were retrospectively reviewed. Follow-up scans were performed in 10 patients, and visual scores (VS) and maximal standard uptake (SUV max) values were analysed. Results: With the initial scan, 83.3% of patients showed lesions in more than one cartilage. Mean VS and SUV max values in the cartilages were 2.92 ± 0.38 and 4.06 ± 0.18, respectively. In 18 patients with no history of corticosteroid treatment, more cartilages were affected than in patients who previously received corticosteroids (3.89 ± 0.29 vs 2.75 ± 0.63, respectively; p = 0.07). Positive rates for PET/CT-guided biopsy in nasal, auricular and tracheal/bronchial cartilages were 100%, 88.9% and 10.5%, respectively. In comparison, the positive rate for non-PET/CT-guided biopsy of auricular cartilage was 92.3%. Compared with the initial scan, the second scan had significantly lower mean VS (1.41 ± 0.20 vs 2.92 ± 0.38, respectively; p < 0.0001) and SUVmax values (2.76 ± 0.14 vs 4.06 ± 0.18, respectively; p < 0.001). Conclusions: FDG uptake in multiple cartilages suggests a diagnosis of RP, but repeated administration of corticosteroids may undermine the diagnostic value of 18 F-FDG PET/CT. Further investigation of its use in locating biopsy sites and in monitoring the response to treatment is required.

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“…Little is known about the pathogenesis of relapsing polychondritis, although there is some evidence of cell-mediated autoimmunity playing a role (20). Besides the strong association with the genetic allele HLA-DR4, T cells have been found in affected cartilage, and T cell responses specific to collagen type II peptides were reported (21)(22)(23)(24). Also, decreased regulatory T cell (Treg) counts and a less diverse TCR repertoire were observed in patients with relapsing polychondritis compared to healthy FIGURE 3 | Absolute CD8 + and CD4 + T cell numbers and CRP levels after autologous HSCT.…”

Section: Discussionmentioning

confidence: 99%

Case Report: Lessons Learned From Subsequent Autologous and Allogeneic Hematopoietic Stem Cell Transplantations in a Pediatric Patient With Relapsing Polychondritis

et al. 2022

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BackgroundAutologous hematopoietic stem cell transplantation (autoHSCT) is increasingly being recognized as a treatment option for severe refractory autoimmune diseases (AD). However, efficacy is hampered by high relapse rates. In contrast, allogeneic HSCT (alloHSCT) has high potential to cure AD, but is associated with significant morbidity and mortality, and data in AD are limited. Experience with autoHSCT in relapsing polychondritis, a rare episodic inflammatory disorder characterized by destruction of cartilage, is scarce and alloHSCT has not been described before.Case PresentationHere, we present a case of a 9-year-old girl who was diagnosed with relapsing polychondritis, with severe airway involvement requiring a tracheostomy. The disease proved to be steroid-dependent and refractory to a wide array of disease-modifying anti-rheumatic drugs and biologicals. After an autoHSCT procedure, the disease became inactive for a short period of time, until the patient experienced a relapse after 31 days, accompanied by repopulation of effector/memory CD8+ T cells. Because of persistent inflammation and serious steroid toxicity, including severe osteoporosis, growth restriction, and excessive weight gain, the patient was offered an alloHSCT. She experienced transient antibody-mediated immune events post-alloHSCT, which subsided after rituximab. She ultimately developed a balanced immune reconstitution and is currently still in long-term disease remission, 8 years after alloHSCT.ConclusionThis case adds to the few existing reports on autoHSCT in relapsing polychondritis and gives new insights in its pathogenesis, with a possible role for CD8+ T cells. Moreover, it is the first report of successful alloHSCT as a treatment for children with this severe autoimmune disease.

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Relapsing Polychondritis Complicated by Giant Cell Myocarditis and Myositis

Cited by 8 publications

References 11 publications

Is 18F-FDG PET/CT useful for diagnosing relapsing polychondritis with airway involvement and monitoring response to steroid-based therapy?

Is 18F-FDG PET/CT useful for diagnosing relapsing polychondritis with airway involvement and monitoring response to steroid-based therapy?

WITHDRAWN: A re-evaluation of 18F-FDG PET/CT for diagnosing relapsing polychondritis and monitoring the therapeutic response to treatment

Case Report: Lessons Learned From Subsequent Autologous and Allogeneic Hematopoietic Stem Cell Transplantations in a Pediatric Patient With Relapsing Polychondritis

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