Imaging features and clinical value of 18F-FDG PET/CT for predicting airway involvement in patients with relapsing polychondritis.

BACKGROUND: The clinical value of 18F-fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) in assessing relapsing polychondritis (RP) with airway involvement remains controversial. This study aimed to investigate PET/CT features of RP with airway involvement and explore its clinical value in predicting disease pattern, severity and prognosis. METHODS: RP patients with airway involvement who underwent PET/CT from January 2010 to July 2022 were retrospectively reviewed. PET/CT features were analyzed both visually and semiquantitatively with the maximum standardized uptake value (SUVmax) and total lesion glycolysis (TLG). Patterns of airway involvement on PET were summarized. Correlations of SUVmax and TLG of the airway were made with spirometric indicators and serological inflammatory markers (CRP and ESR). In addition, long-term follow-up was conducted through questionnaires in regard to symptom control, subjective feeling, pulmonary function, and quality of life. RESULTS: Fifty-two cases were finally included. 18F-FDG PET showed FDG-avid lesions with increased FDG uptake in the airway among 94.2% of the patients. Three patterns (focal, multifocal and diffuse patterns) were identified. TLG of the whole airway was lower in patients with previous therapy (p = 0.046). Bronchoscopy was more sensitive in detecting tracheal abnormalities (90.7% vs.53.5%, p = 0.039) but less sensitive for peripheral airway lesions (65.1% vs. 79.1%, p = 0.046) compared with PET. SUVmax and TLG of the airway positively correlated with spirometry indicators (FEV1%pred, FEV1/FVC, MEF 50%pred, etc.) and serological inflammatory markers. Five patients died during the follow-up, with two deaths related to airway problems. Higher FDG uptake predicted worse subjective feeling, but not with symptom control or pulmonary function. CONCLUSION: PET/CT is a valuable tool for RP with airway involvement, particularly in assessing peripheral airway lesions, and PET/CT related parameters are significantly associated with disease patterns, severity, and long-term outcomes.

Relapsing Polychondritis: The Oto-Rhino-Tracheobronchial Triad on 18 F-FDG PET/CT.

ABSTRACT: We present a 54-year-old woman with 3-month history of skin rash and bilateral hearing loss. Multiple investigations including biopsy failed to reveal the underlying diagnosis of relapsing polychondritis, until 18 F-FDG PET/CT demonstrated a characteristic FDG activity pattern-the oto-rhino-tracheobronchial triad. The patient was subsequently diagnosed to have relapsing polychondritis and received treatment. The second FDG PET/CT revealed a differential response in different disease sites, which prompted an escalation of treatment.

Differentiating tracheobronchial involvement in granulomatosis with polyangiitis and relapsing polychondritis on chest CT: a cohort study.

BACKGROUND: In patients with tracheobronchial involvement, the differential diagnosis between granulomatosis with polyangiitis (GPA) and relapsing polychondritis (RP) can be challenging. The aim of this study was to describe the characteristics of airway abnormalities on chest computed tomography (CT) in patients with GPA or RP and to determine whether specific imaging criteria could be used to differentiate them. METHODS: GPA and RP patients with tracheobronchial involvement referred to a national referral center from 2008 to 2020 were evaluated. Their chest CT images were reviewed by two radiologists who were blinded to the final diagnosis in order to analyze the characteristics of airway involvement. The association between imaging features and a diagnosis of GPA rather than RP was analyzed using a generalized linear regression model. RESULTS: Chest CTs from 26 GPA and 19 RP patients were analyzed. Involvement of the subglottic trachea (odds ratio for GPA=28.56 [95% CI: 3.17; 847.63]; P=0.001) and extensive airway involvement (odds ratio for GPA=0.02 [95% CI: 0.00; 0.43]; P=0.008) were the two independent CT features that differentiated GPA from RP in multivariate analysis. Tracheal thickening sparing the posterior membrane was significantly associated to RP (odds ratio for GPA=0.09 [95% CI: 0.02; 0.39]; P=0.003) but only in the univariate analysis and suffered from only moderate interobserver agreement (kappa=0.55). Tracheal calcifications were also associated with RP only in the univariate analysis (odds ratio for GPA=0.21 [95% CI: 0.05; 0.78]; P=0.045). CONCLUSION: The presence of subglottic involvement and diffuse airway involvement are the two most relevant criteria in differentiating between GPA and RP on chest CT. Although generally considered to be a highly suggestive sign of RP, posterior tracheal membrane sparing is a nonspecific and an overly subjective sign.

Comparison of relapsing polychondritis patients with and without respiratory involvement based on chest computed tomography: a retrospective cohort study.

BACKGROUND: Relapsing polychondritis (RP) patients with tracheal cartilage involvement are different from other patients. The objective of this study was to compare the clinical features and disease patterns between a respiratory involvement subgroup and a non-respiratory involvement subgroup according to chest computed tomography. METHOD: We performed a retrospective cohort study collecting RP patients hospitalized at the Beijing Chao-Yang Hospital between January 2012 and August 2021. RESULTS: Respiratory involvement affected 59.7% of patients in our cohort. The incidence of costochondritis was more common in RP patients with respiratory involvement (p = 0.03); the incidence of inflammatory eye disease (p = 0.001) and auricular chondritis (p = 0.001) was less frequent in RP respiratory involvement patients.. Compared with the non-respiratory involvement subgroup the incidence of pulmonary infection marginally increased in the respiratory involvement subgroup (p = 0.06). Inflammatory indexes except for C-reactive protein to albumin ratio (CAR) were significantly higher in the respiratory involvement subgroup; analysis revealed no significant relationship between inflammatory indexes and pulmonary infection. CONCLUSION: RP patients with respiratory involvement had a greater incidence of costochondritis and pulmonary infectionand lesser incidence of inflammatory eye diseases and auricular chondritis compared to non-respiratory involvement. Increasing inflammatory indexes suggests that patients with respiratory involvement had a higher disease activity index of RP. The difference in probability of survival was insignificant between subgroups.

Correlation between Computed Tomographic Analysis and Pulmonary Function Measurements in Patients with Relapsing Polychondritis.

BACKGROUND: Relapsing polychondritis (RP) is a rare systemic disease of unknown origin, with cartilaginous involvement in multiple organs. Airway involvement is the most important prognostic factor in RP. OBJECTIVES: Spirometric measurements and minimum tracheal cross-sectional area (mtCSA) have been reported as useful to assess the degree of airway stenosis. Because the length and severity of tracheal involvement in RP can vary, mtCSA might not provide enough information to assess tracheal abnormalities. We introduced tracheal volume (TrV) as a new method to evaluate correlations between chest computed tomography (CT) measurements and pulmonary function tests, including impulse oscillometry (IOS). METHOD: We analyzed chest CT images, spirometry, and IOS collected at our institution from April 2004 to March 2019. We calculated correlations between chest CT measurements using software (TrV, TrV/tracheal length [TrV/TL], and mtCSA) and pulmonary function parameters. RESULTS: Twenty-five of 73 clinically diagnosed patients with RP were included. Spirometric findings showed moderate airway obstruction. Peak flow (PEF) was strongly correlated with mtCSA, TrV, and TrV/TL (ρ = 0.74, p < 0.001). FEV1 was significantly correlated with mtCSA (ρ = 0.56, p = 0.004), TrV (ρ = 0.52, p = 0.007), and TrV/TL (ρ = 0.53, p = 0.006). Whereas respiratory resistance at 5 Hz (R5) and 20 Hz (R20) and resonant frequencies (RFs) were significantly correlated with TrV (ρ = -0.46, p = 0.021; ρ = -0.46, p = 0.046; and ρ = -0.42, p = 0.037, respectively), IOS parameters and mtCSA were not. CONCLUSIONS: In patients with RP, TrV and mtCSA were strongly correlated with spirometric measurements. Respiratory resistances assessed by IOS correlated only with TrV. This suggests TrV assessment reflects pulmonary function in patients with RP more appropriately than mtCSA.

Fluorodeoxyglucose positron emission tomography/computed tomography in the diagnosis, assessment of disease activity and therapeutic response in relapsing polychondritis.

OBJECTIVE: To evaluate 18F-fluorodeoxyglucose (FDG) PET/CT in the assessment of disease activity, extent of the disease and response to therapy in relapsing polychondritis. METHODS: Twenty-five patients (9 men, 16 women) with a mean age of 38.2 years (s.d. 13.7; range 18-62), diagnosed to have relapsing polychondritis according to Damiani and Levine's modification of McAdam's criteria, who underwent PET/CT examination were included. Ten patients underwent a second PET/CT examination after therapy or during follow-up. Clinical symptoms and auxiliary examination findings were recorded. PET/CT findings were reviewed and correlated with the clinical symptoms. RESULTS: The major symptoms were aural pain (n = 21), nasal pain (n = 10), stridor (n = 5), cough (n = 9), fever (n = 8) and laryngeal tenderness (n = 8). The initial PET/CT was positive in 23/25 patients. PET/CT revealed involvement of auricular (n = 14), nasal (n = 8), laryngeal (n = 7), tracheobronchial (n = 6) and Eustachian (n = 3) cartilages with a mean maximum standardized uptake value (SUVmax) of 4.1 (s.d. 2.5; range 1.7-12.7). Fair correlation of aural/nasal pain/stridor with FDG avidity of cartilage involvement on PET/CT was noted. The key finding was detection of asymptomatic large airway involvement in seven patients (28%). Re-examination PET in 10 patients revealed complete therapeutic response (n = 5), partial response (n = 1), stable disease (n = 1), progressive disease (n = 1) and disease recurrence (n = 2). CONCLUSION: FDG PET/CT is a useful tool for the assessment of the disease activity and extent. It identified activity in clinically inaccessible sites that are of clinical significance. It is also useful in assessing treatment response and finding relapse.

Concomitant aortic valve replacement, mitral valve replacement, and coronary artery bypass grafting for aortic stenosis and mitral regurgitation in a patient with relapsing polychondritis.

Relapsing polychondritis is a rare multi-system disease characterized by inflammation in cartilaginous structures and other connective tissues. Cardiovascular complications occur in 10-51% of the patients. We report a case of concomitant aortic valve replacement, mitral valve replacement, and coronary artery bypass grafting in a patient with relapsing polychondritis. A 71-year-old female with relapsing polychondritis on prednisolone (5 mg/day) for 15 years presented at our hospital for further evaluation of valvular disease. Severe aortic stenosis and severe mitral regurgitation were diagnosed. We performed aortic and mitral valve replacement. During surgery, we found connective tissue surrounding the intima of the sinus of Valsalva and stenosis of the right coronary artery ostium, which was not noted on preoperative coronary angiography. We removed the tissue and performed bypass grafting to the right coronary artery. Postoperative recovery was uneventful, and she was discharged 27 days after surgery.

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

BACKGROUND: 18F-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. OBJECTIVE: This 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. METHODS: A 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. RESULTS: In 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. CONCLUSION: 18F-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.

Imaging of Thoracic Wall Abnormalities.

Identification of certain abnormalities of the chest wall can be extremely helpful in correctly diagnosing a number of syndromic conditions and systemic diseases. Additionally, chest wall abnormalities may sometimes constitute diagnoses by themselves. In the present pictorial essay, we review a number of such conditions and provide illustrative cases that were retrospectively identified from our clinical imaging database. These include pentalogy of Cantrell, Klippel-Feil syndrome, cleidocranial dysplasia, Poland syndrome, osteopetrosis, neurofibromatosis type 1, Marfan syndrome, Gardner syndrome, systemic sclerosis, relapsing polychondritis, polymyositis/dermatomyositis, ankylosing spondylitis, hyperparathyroidism, rickets, sickle cell anemia, thalassemia, tuberculosis, septic arthritis of the sternoclavicular joint, elastofibroma dorsi, and sternal dehiscence.

Multimodal Imaging Report of Crystalline Keratopathy in Relapsing Polychondritis.

PURPOSE: To describe a case of bilateral multifocal stromal crystalline keratopathy in the setting of relapsing polychondritis (RP). METHODS: Case report. RESULTS: We describe a 31-year-old woman who presented with ocular inflammation, bilateral auricular chondritis, and nasal chondritis, meeting the clinical criteria of RP. We illustrate her auricular cartilaginous abnormalities, saddle nose deformity, scleritis, and discrete mid-stromal opacities in both corneas that extend through the central cornea. Uniquely, her opacities feature a marked crystalline component as demonstrated on photography, anterior segment optical coherence tomography, and confocal microscopy. CONCLUSION: A central keratopathy is not typically reported in patients with RP. In this case report, we describe a unique diffuse bilateral nummular mid-stromal crystalline keratitis that extends into the central cornea and further define it using multimodal imaging.

Long-Term Outcome of Metallic Stenting for Central Airway Involvement in Relapsing Polychondritis.

BACKGROUND: Placement of uncovered self-expandable metallic stents was found to successfully alleviate critical airflow limitation in patients with relapsing polychondritis (RP) with central airway involvement by several reports. However, the long-term outcome of airway metallic stenting in patients with RP remain unclear. METHODS: We retrospectively analyzed patients with RP who underwent airway metallic stenting with the use of fiberoptic bronchoscopy between September 1, 2009, and October 1, 2017, in Shanghai. Outcome measurements, including modified Medical Research Council (mMRC) dyspnea score, 6-minute walk distance (6MWD), spirometry, and bronchoscopic findings, as well as adverse events after stent placement, were collected. RESULTS: A total of 27 patients were included; the median patient age was 58 years (range: 41 to 74 years), and 19 were men (70.4%). Nineteen uncovered self-expandable metallic stents were placed in the trachea and 39 in the main bronchi. The median follow-up time was 50.5 months (range: 6 to 100 months). The baseline forced expiratory volume in 1 second (FEV1) percentage predicted (%pred), FEV1/forced vital capacity (FVC), and peak expiratory flow (PEF) was 24.2 ± 3.7, 27.2 ± 5.6, and 0.99 ± 0.21 L/min, respectively. One day after the procedure, improvement from baseline in FEV1 %pred, FEV1/FVC, and PEF was 17.9 ± 8.9 (p = 0.001), 19.8 ± 10.9 (p = 0.002), and 0.69 ± 0.44 L/min (p = 0.001), respectively. Changes in the following variables were also statistically and clinically significant: 6MWD of 193.7 ± 83.4 m; mMRC dyspnea score of -1.2 ± 0.4 points (both p < 0.05). The improvements were maintained at 5 years: a mean change in FEV1 %pred, FEV1/FVC, PEF, 6MWD, and mMRC score was 19.5 ± 6.7, 13.9 ± 5.0, 0.82 ± 0.40 L/min, 134.7 ± 66.2 m, and -0.83 ± 0.29 points, respectively. Cough, foreign body sensation, mucus production, and granulomas were common adverse events, occurring in 48.1% (13 of 27), 40.7% (11 of 27), 29.6% (8 of 27), and 25.9% (7 of 27) of the subjects. However, none of those complications were severe enough to require urgent bronchoscopic interventions. CONCLUSIONS: Airway metallic stenting in patients with RP with central airway involvement resulted in long-term clinical benefits in lung function, exercise tolerance, and dyspnea with an acceptable safety profile.

[The Characteristics of Imaging, Fiber-bronchoscope and Pulmonary Function Testing of Relapsing Polychondritis Patients with Respiratory Symptoms].

OBJECTIVE: To analyze the characteristics of clinical manifestation, imaging, fiber-bronchoscope finding and pulmonary function testing in the patients of relapsing polychodriti with respiratory symptoms. METHODS: The clinical data of 33 patients with RP in West China Hospital of Sichuan University from May 2007 to May 2017 were analyzed retrospectively. The patients were divided into respiratory group (21 cases) and non-respiratory group (12 cases) based on whether the respiratory symptoms were main involvement, and the data of the two groups were statistically analyzed. RESULTS: Among the 33 patients, the most vulnerable system was respiratory system, accounting for 63.6%. The other involvement areas were as follows: ears 48.5%, joints 30.3%, skin 15.2%, noses and eyes 12.1% respectively. The chief complaints in the respiratory group were cough, polypnea, and fever. The major manifestations of the non-respiratory group were pain and swelling of ears and nose, joints. CT showed that the wall of trachea and main bronchus were thickened, calcified, and narrowed in 13 cases (61.9%) of the respiratory group. while 3 patients (25.0%) found thickened bronchus wall without bronchostenosis in non-respiratory group . Bronchoscopy showed that 86.7% (13/15) of the patients were found airway abnormal in the respiratory group, and none of the patients in the non-respiratory group had. In the test of pulmonary function, the respiratory group had lower one second forced expiratory volume (FEV1), [FEV1/ forced vital capacity (FVC)] and peak expiratory flow (PEF), and all the differences were significant (P<0.05). CONCLUSION: CT, broncho-scopy, and pulmonary function tests could provide early evidence to diagnosis of relapsing polychondritis, which is lack of specificity of diagnosis, especially respiratory system symptom onset.

Aseptic meningitis in relapsing polychondritis: a case report and literature review.

Aseptic meningitis is an extremely rare neurologic complication of relapsing polychondritis (RP). We reported a case of a 58-year-old Chinese female with intractable headache, puffy ears, pleocytosis, and cranial magnetic resonance imaging (MRI) showing thickened and enhanced meninges. She was finally diagnosed of aseptic meningitis due to RP after full exclusion of infectious causes. She gradually developed neurosensory hearing loss, vertigo, and saddle nose while glucocorticosteroid therapy and combined cyclophosphamide could not control her headache. Ultimately, cyclosporin A was tried showing a good response. Only 18 previous cases were found in the literature and the clinical manifestation, cerebrospinal fluid (CSF) characteristics, imaging features, and therapy considerations of RP-related aseptic meningitis were summarized by reviewing the literature. Aseptic meningitis due to RP is a rare condition of undetermined pathoetiology. Its diagnosis is primarily based on clinical manifestations combined with CSF and MRI examinations plus adequate exclusion of possible infections. Corticosteroid is the basic therapy but choice of protocol should be individualized.