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.

Idiopathic Sclerosing Orbital Inflammation in a Relapsing Polychondritis Patient With Impaired Vision.

A 55-year-old woman with relapsing polychondritis had progressively enlarged right retro-orbital tumor invading the optic nerve, followed by left retrobulbar infiltrating lesions despite prescription of high-dose corticosteroids and pulse methylprednisolone. Repeated histopathologic analyses showed dense collagen fibers with scanty inflammatory cells, consistent with the diagnosis of idiopathic sclerosing orbital inflammation. This disorder has been recognized as a distinct entity with unique clinical features and coexisting rheumatologic disorders, requiring more focused diagnostic strategies and therapeutic regimens. In summary, we demonstrate a rare ocular manifestation in relapsing polychondritis and emphasize the importance of serial radiological and pathological evaluations in such patients presenting with exophthalmos.

Quantitative Computed Tomography Measurement of Tracheal Cross-Sectional Areas in Relapsing Polychondritis: Correlations with Spirometric Values.

BACKGROUND: Although tracheal stenosis occurs in relapsing polychondritis (RP), no studies exist that have clarified correlations between quantitative airway measurement and spirometry in RP patients. OBJECTIVES: The aim of this study was to investigate correlations between the cross-sectional area (CSA) of the trachea and spirometric values in patients with RP. METHODS: The institutional review board approved this retrospective study, and written informed consent was waived. Twenty-six patients with RP underwent spirometry and chest computed tomography (CT) at full inspiration and end-expiration. On inspiratory and expiratory chest CT images, CSA at the intrathoracic trachea was measured for all CT slices, and the mean and minimum tracheal CSA were obtained. Correlations between the tracheal CSA and spirometric values were assessed by Spearman's rank correlation analysis. Results: Tracheal CSA measurements for inspiratory and expiratory scans were significantly correlated with FEV 1 , FEV 25-75% , and peak flow values (ρ = 0.51-0.86, p <0.01). During each inspiratory or expiratory phase, the minimum tracheal CSA achieved a higher correlation coefficient with spirometric values than the mean CSA. CONCLUSION: Tracheal dimensions for both inspiratory and expiratory CT are significant predictors of pulmonary function in patients with RP. The narrowest tracheal dimension likely determines the severity of airflow limitation in RP.

Tracheobronchial stenosis evaluated by inspiratory and expiratory three-dimensional computed tomography and impulse oscillation with three-dimensional color imaging in a patient with relapsing polychondritis.

Patients with relapsing polychondritis (RP) and airway stenosis have difficulty performing conventional spirometry that requires maximum forced expiration. We report a patient with RP who showed progressive severe bronchial stenosis on three-dimensional computed tomography (3D-CT) and impulse oscillation (IOS) with 3D color imaging using a Mostgraph®. The forced oscillation technique using IOS allows within-breath evaluation without forced expiration. A 68-year-old man who had RP presented with dyspnea due to stenosis of the trachea and left main bronchus (lt. mb). Stenting was performed twice in two years. Chest 3D-CT revealed a marked difference in the extent of bronchial collapse during expiration compared with inspiration. The forced expiratory volume in 1 second (FEV1.0), reactance at 5Hz (X5), resonant frequency (Fres), and integrated low frequency reactance area (ALX) measured by IOS showed temporary improvement after placement of the first stent, but respiratory resistance at 5Hz (R5) and 20Hz (R20) remained poor. 3D color images of respiratory resistance obtained with a Mostgraph® already showed high values at the time of diagnosis, resembling the features of chronic obstructive disease (COPD). 3D color images were helpful for interpreting the changes of IOS parameters during the clinical course. In conclusion, 3D-CT in inspiration/expiration and noninvasive IOS with 3D color imaging are useful for assessing airway stenosis in RP while reducing the burden of repeated spirometry.

Quantitative computed tomography assessment of air trapping in relapsing polychondritis: correlations with spirometric values.

OBJECTIVE: The purpose of this study was to evaluate the relationship between quantitative computed tomography (CT) parameters of air trapping obtained with inspiratory and expiratory CT and pulmonary function tests (PFTs) in patients with relapsing polychondritis (RP). MATERIALS AND METHODS: This study included 23 patients with RP who underwent both CT and PFTs. In each patient, the mean lung density (MLD) was obtained by averaging CT attenuation of the lung parenchyma on both inspiratory and expiratory CT. The ratio of expiratory MLD to inspiratory MLD (E/I ratio) was also calculated. Correlations between those quantitative CT measurements and the results of PFTs were evaluated using Spearman rank correlation. RESULTS: The expiratory MLD and E/I ratio were significantly correlated with forced expiratory volume in 1 second (FEV1) %predicted, ratio of FEV1 to FVC (FEV1/FVC), and the mid expiratory phase of forced expiratory flow (FEF25%-75%) %predicted (expiratory MLD: FEV1 %predicted, r = 0.764, P < 0.0001; FEV1/FVC, r = 0.764, P < 0.0001; FEF25%-75% %predicted, r = 0.674, P < 0.001, respectively; the E/I ratio: FEV1 %predicted, r = -0.689, P < 0.001; FEV1/FVC, r = -0.689, P < 0.001; FEF25%-75% %predicted, r = -0.586, P < 0.01, respectively). The correlation between inspiratory MLD and PFTs did not reach statistical significance. CONCLUSIONS: In RP patients, air trapping demonstrated on expiratory CT correlated with airway obstruction. This study may assist further refinement of the use of CT as quantitative evaluation for small and large airway obstruction in RP.

Clinical analysis of 15 patients with relapsing auricular polychondritis.

Relapsing polychondritis (RP) is a rare autoimmune disorder of unknown etiology. The disease is characterized by episodic inflammation and destruction of cartilaginous and connective tissue structures, including the ear, eye, nose, larynx, trachea, bronchi, joints, skin, heart valves, and aorta. As the symptoms of RP are diverse and complex, it is easily misdiagnosed. The aim of this paper was to improve the understanding of the clinical features of RP, thereby facilitating its early diagnosis. Fifteen patients with RP were analyzed retrospectively and the relevant literature reviewed. The number of patients presenting with auricular chondritis was 13, while two presented with polyarthritis. Among them, the treatment of 2 RP patients with respiratory tract involvement failed and 1 patient died. Eleven patients with RP (73 %) were initially misdiagnosed. RP involves cartilage and connective tissue. The prognosis for patients with respiratory tract involvement is poor. RP causes episodic and progressive inflammation of cartilage throughout the body and is associated with a variety of clinical manifestations. Early diagnosis of RP depends on a thorough understanding of its clinical features.

[Relapsing polychondritis].

Relapsing polychondritis (RP) is a systemic inflammatory condition leading to irreversible structural changes in cartilaginous tissue as a result of generalized proteoglycane decomposition. Auricular and nasal cartilage is usually the first to be affected at the onset of the disease which leads to episcleritis. At the later stages, the larynx and trachea become involved, vasculitis develops affecting the organs of hearing, coronary arteries, kidneys, meninges, and other organs. The disease has an intermittent, recurrent character. The clinical course of RP is irregular. The disease is a rare occurrence and difficult to diagnose. The present review is based on the publications by domestic and foreign authors. It is intended to enhance awareness of the clinical picture and peculiarities of RP among clinicians with a view to improving its diagnostics.

Evaluation of Semicircular Canal Function in Relapsing Polychondritis Patients With Dizziness and Sensorineural Hearing Loss Using Video Head Impulse Test.

OBJECTIVE: To evaluate semicircular canal function using video head impulse test (vHIT) in relapsing polychondritis (RP) patients presenting with dizziness and sensorineural hearing loss. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: Three patients with RP underwent vHIT and hearing tests. INTERVENTION: Diagnostic. MAIN OUTCOME MEASURES: The gain in vestibulo-ocular reflex (VOR) and the presence of catch-up saccade were examined, and the correlation between semicircular canal dysfunction and hearing loss was investigated. RESULTS: Of the six ears, five exhibited semicircular canal dysfunction. Among these, one ear showed dysfunction in two semicircular canals, while the remaining four ears demonstrated dysfunction in all three semicircular canals. Sensorineural hearing loss, ranging from moderate to profound, was detected by pure-tone audiometry in all six ears. Furthermore, a significant correlation was observed between VOR gain in the horizontal semicircular canal (HSC) and hearing level. CONCLUSIONS: This study demonstrated semicircular canal dysfunction in RP patients presenting with dizziness and hearing loss using vHIT. Moreover, a significant correlation was found between HSC dysfunction and the severity of hearing loss. While inner ear involvement is a key clinical symptom included in the diagnostic criteria for RP, there have been few reports evaluating vestibular dysfunction, and this is the first report on the evaluation of several cases using vHIT. Accurate assessment of vestibular function by vHIT may facilitate early diagnosis and intervention in RP, potentially improving patient outcomes.

Ocular manifestations of systemic inflammatory diseases.

Inflammation of the eye is often times seen in association with systemic inflammatory diseases. Understanding the various forms of ocular involvement in these conditions is important as untreated ophthalmic involvement can lead to severe vision loss. In addition to providing a basic framework for diagnosis and treatment, this review will highlight the ocular manifestations of the following systemic inflammatory conditions: rheumatoid arthritis, systemic lupus erythematosus, Wegener's granulomatosis, Sjögren's syndrome, polyarteritisnodosa, primary antiphospholipid syndrome, Behçet's syndrome, Kawasaki disease, Cogan's syndrome and relapsing polychondritis.

Relapsing polychondritis: state-of-the-art review with three case presentations.

BACKGROUND: Relapsing polychondritis (RPC) is a complex immune-mediated systemic disease affecting cartilaginous tissue and proteoglycan-rich organs. The most common and earliest clinical features are intermittent inflammation involving the auricular and nasal regions, although all cartilage types can be potentially affected. The life-threatening effects of rpc involve the tracheobronchial tree and cardiac connective components. Rpc is difficult to identify among other autoimmune comorbidities; diagnosis is usually delayed and based on nonspecific clinical symptoms with limited laboratory aid and investigations. Medications can vary, from steroids, immunosuppressants, and biologics, including anti-tnf alpha antagonist drugs. METHOD: Information on updated etiology, clinical symptoms, diagnosis, and treatment of rpc has been obtained via extensive research of electronic literature published between 1976 and 2019 using PubMed and medline databases. English was the language of use. Search inputs included 'relapsing polychondritis,' 'polychondritis,' 'relapsing polychondritis symptoms,' and 'treatment of relapsing polychondritis.' Published articles in English that outlined and reported rpc's clinical manifestations and treatment ultimately met the inclusion criteria. Articles that failed to report the above and reported on other cartilaginous diseases met the exclusion criteria. RESULT: Utilizing an extensive overview of work undertaken in critical areas of RPC research, this review intends to further explore and educate the approach to this disease in all dimensions from pathophysiology, diagnosis, and management. CONCLUSION: RPC is a rare multi-systemic autoimmune disease and possibly fatal. The management remains empiric and is identified based on the severity of the disease per case. The optimal way to advance is to continue sharing data on RPC from reference centers; furthermore, clinical trials in randomized control groups must provide evidence-based treatment and management. Acquiring such information will refine the current knowledge of RPC, which will improve not only treatment but also diagnostic methods, including imaging and biological markers.

Treatment of relapsing polychondritis in the era of biological agents.

Relapsing polychondritis (RP) is a rare disorder, often requiring high doses of immunosuppressive therapy to control its potentially life-threatening consequences. The advent of biological agents has added to the armamentarium available to treat RP, but the lack of controlled trials, along with the small numbers of patients and disease heterogeneity means that new therapies are prescribed without the benefits of rigorous clinical research. Thus, information on individual cases is of value in expanding our knowledge of the use of biologic agents in rare conditions. We report on the use of rituximab in a patient who subsequently developed catastrophic aortic incompetence, and we review the literature in relation to the use of this drug in RP.

The relapsing polychondritis damage index (RPDAM): Development of a disease-specific damage score for relapsing polychondritis.

OBJECTIVES: Relapsing polychondritis is a rare, multi-systemic and inflammatory condition of unknown origin. We currently lack a core set of measures to assess and follow damage in patients suffering from this condition. Our primary aim was to derive a disease-specific damage measuring tool for relapsing polychondritis, the Relapsing Polychondritis Damage Index (RPDAM). METHODS: We performed an international 4-round multicenter Delphi study during which experts were asked to rate the relevance of potential damage items for relapsing polychondritis (141 items were obtained from a literature review and 12 from expert suggestion), using a Likert Scale. The selection of items for each subsequent round was based on the median rating of each item. RESULTS: Twenty-four experts from 11 nationalities participated in round 1 and 22 in rounds 2, 3 and 4. From the initial 153 potential damage items, 44 items were selected during round 1, 30 items during round 2 and 16 during round 3. During round 4, we refined the index to a total of 17 items referring to ear nose and throat, eye, respiratory, cardiovascular and hematological systems as well as to treatment-related specific damage items. CONCLUSION: We have developed by international consensus a scoring system to assess damage in patients with relapsing polychondritis. Following its validation, the RPDAM may contribute to improve the care of patients suffering from this rare condition as well as to standardize data collection for future clinical trials.