Recommendations of diagnosis and treatment of relapsing polychondritis in China / 中华内科杂志

Relapsing polychondritis is an immune mediated systemic inflammatory disease, involving the cartilaginous and proteoglycan rich structures. The characteristic manifestations were inflammation and deformity of ear and nasal cartilage. Here, Chinese Rheumatology Association summarized manifestations, diagnosis and disease activity index evaluation of relapsing polychondritis, standardized treatment regimens, to improve disease prognosis.

Encefalopatía subaguda en una paciente con policondritis recidivante: caso clínico / Subacute encephalopathy associated with relapsing polychondritis: report of one case

Relapsing polychondritis (RP) is a rare multisystemic autoimmune disorder characterized by the inflammation and destruction of cartilages, with preference for auricular, nasal and laryngotracheal cartilages. RP may also affect proteoglycan-rich structures, such as, blood vessels, eyes, kidneys, and heart. The central nervous system (CNS) is involved in less than 3% of patients. We report a 32-year-old female with RP associated with a progressive subacute encephalopathy characterized by behavioral disturbances, auditory and visual hallucinations. The EEG showed generalized slow activity and a mononuclear pleocytosis with increased protein was found in the cerebrospinal fluid. The brain magnetic resonance imaging showed multiple supra and infratentorial nodular inflammatory lesions. After initiating treatment with corticosteroids and cyclophosphamide, a significant improvement in chondritis and neurological status was observed.

Reconstructive rhinoplasty with costal cartilage grafting: A case report of relapsing polychondritis

Relapsing polychondritis (RP) is a rare autoimmune disorder of unknown etiology characterized by recurrent episodes of inflammation and the destruction of cartilaginous tissues, primarily involving the ear, nose, and the respiratory tract. Nasal chondritis is present in 24% of patients at the time of diagnosis and develops subsequently in 53% throughout the diseases progress. Progressive destruction of nasal cartilage leads to the characteristic flattening of the nasal bridge, resulting in the saddle nose deformity. In patients with RP, surgical management for saddle nose is carefully decided due to the disease relapsing characteristics. We present a RP patient with a saddle nose deformity who underwent reconstruction rhinoplasty with autologous costal cartilage grafting. At 6-month follow-up, the patient retained good esthetic results and showed neither complication nor relapse of RP.

18F-FDG PET/CT in diagnosis of relapsing polychondritis / 中国医学影像技术

Objective To explore the value of 18F-FDG PET/CT in diagnosis of relapsing polychondritis (RP). Methods 18F-FDG PET/CT imaging features of 28 patients of RP (RP group) and 28 healthy volunteers (control group) were retrospectively analyzed. The lesions' location, shape, margins, density and the maximum standardized uptake (SUVmax) were analyzed. ROC curve was used to evaluate the efficacy of SUVmax of lesions in diagnosis of RP. Results In all 28 RP patients, increased density, blurred boundary and thickened wall of trachea and bronchus were found in 24 cases. Four cases showed increased density and fuzzy boundary, as well as edema and thickening in larynx cartilages. Totally 91 lesions were found with PET/CT, located in the nasal cartilages, bilateral auricular cartilages, costal cartilages, laryngeal cartilages, trachea and bronchial cartilages, respectively. SUVmax of lesions on each site in RP group was higher than those in control group (all P0.90), while SUVmax of nasal cartilages had a moderate diagnostic value (AUC=0.71). Conclusion 18F-FDG PET/CT manifestations of RP have some characteristics, and can display multiple lesions simultaneously, therefore being helpful to diagnosis of RP.

Whole ear involvement in non-elder patients with relapsing polychondritis / 中华风湿病学杂志

Objective To investigate the clinical and audiological characteristics of non-elder patients with relapsing polychondritis (RP).Methods Clinical and audiological data of patients with RP under 60 years old were collected consecutively and analyzed.The t-test and Mann-Whitney U test were used for statistical analysis between the two groups in quantitative data in normal distribution and non-normal distribution respectively,while Chi-square test was use for qualitative data analysis.Results One hundred and seventy four patients with complete data who fulfilled the Michet criteria were enrolled with a M∶F=1∶1.1.The mean age of disease onset was (39±13) (8-60) years;the median time of disease duration was 12 (1-480) months;the median relapsing polychondritis disease activity index (RPDAI) was 38(10-77) and the median RPODI was 2.4(0.1-56).Auricular chondritis (32.8％,57/174),ocular involvement (24.7％,43/174) and airway chonchritis (21.3％,37/174) were the top three onset-pattern.All parts of external,middle and inner ear were involved in RP.Inner-ear damage was the most common (95.4％,166/174) with insidious cochlea and vestibule equally distributed.Auricular chondritis was predominant in external ear involvement (55.2％,96/174);ET dysfunction was included in eardrum abnormalities of neglected middle-ear involvement (29.9％,52/174).Positive HL by active detection was 71.8％(125/174) with 14.3(25/174) HL fulfilled world health organization (WHO)-2006 criteria,including 52.0％(13/25) disabling HL.Sensorineural hearing loss (SNHL) was predominant in RP after 21 years old while 60.0％(6/10) was seen in childhood RP with non-SNHL.At least two parts of ear involvement were seen in almost all patients with heavy overlap.Conclusion All parts of ear are involved in non-elder RP with age related clinical characteristics.Active detection is a key to find insidious middle-and inner-ear involvement for early RP recognition.RPODI is a potential marker for RP evaluation.

Insidious airway involvement in patients with relapsing polychondritis / 中华风湿病学杂志

Objective To investigate clinical and imaging characteristics of patients with relapsing polychondritis (RP) with insidious airway involvement.Methods Data collected prospectively and consecutively for patients with the diagnosis of RP and their disease activity evaluation was analyzed.The t-test and Mann-Whitney U test were used for statistical analysis between the two groups forquantitative datain normal distri-bution and non-normal distribution respectively,while Chi-square test was use for qualitative data analysis.Results Two hundred and sixteen patients with complete data from Dec 1,2007 to Jul 31,2016 were enrolled with a M:F ratio of 1:1.Mean age of disease onset was (44±16)(8～86) years.The median disease duration was 12 (0.3～480) month.The median relapsing polychondritis disease activity index (RPDAI) was 35 (8～67),the median RPODI was 2.4 (0.1～84).The top three initial presentation were auricular chondritis (28.7％),airway chondritis (24.1％) and ocular involvement (22.2％) respectively.RPODI was significantly higher in auricular chondritis (4.4) (Z=-2.084,P＜0.05) and lower in nose chondritis (0.6) (Z=-2.425,P＜0.05).Up to 81.5％ of the patients were found with airway damage and 52.3％ of them were asymptomatic.Airway damage was mostly located in trachea (79.0％ 139/176) and common features on CT scan werecharacterized by airway wail thickening (72.7％,128/176).Airway narrowing was mostly seen in symptomatic patients while calcification was seen more in asymptomatic patients.Insidious hearing-loss and nose chondritis were found not related to airway damage while obvious auricular chondritis (x2=15.580,P＜0.01),ocular involvement (x2=8.105,P＜0.01) were found to be more in patients with asymptomatic airway damage.All diagnosis before RP was organ-driven.Conclusion Airway involvement in RP is one of the three most common disease initial presentation-sand half of them are asymptomatic.RPODI is a reasonable marker for disease evaluation.Routine follow-up of airway damage (wall thickness,calcification and lumen narrowing) is essential for early RP recognition.

The value of 18F-fluorodeoxyglucose posotron emission tomography/computed tomography for detecting the relapse of polychondritis in patients with fever of unknown origin / 中华风湿病学杂志

Objective To analyze the value of 18F-fluorodeoxyglucose (FDG) posotron emission tomography/computed tomography (PET/CT) in the detection of relapsing polychondritis (RP) in fever of unknown origin (FUO) patients.Methods Retrospective analysis of the clinical data of 3 patients with FUO,who were final clinical diagnosed as RP,were conducted.Clinical symptoms,laboratory tests and 18F-FDG PET/CT image data were analyzed,and literature were reviewed.Results Three patients with FUO,2 had increased FDG uptake in PET/CT in multiple cartilages,including ear,nose,trachea/bronchial and rib cartilage (SUVmax 2.5-7.5),which were consistent with RP.One case with a history of RP,PET/CT detected pulmonary infla rrmatory lesions which might be the cause of fever.At the same time,PET/CT showed RP presentation in CT with nasal and airway structure changes,but there was no abnormal FDG uptake.Conclusion 18F-FDG PET/CT has an important clinical value in diagnosis and differential diagnosis of RP,and also in follow-up and clinical response observation.

Relapsing polychondritis presenting with inflammatory pseudotumor

No abstract available.

Clinical analysis of 38 patients with respiratory involvement in relapsing polychondritis / 中国医师进修杂志

Objective To analyze the clinical characteristics of respiratory involvement in relapsing polychondritis(RPC). Methods The clinical data of 38 patients with respiratory (larynx, trachea and bronchus) involvement in RPC were retrospectively analyzed. Results The incidence of respiratory involvement in patients with RPC was 51.35%(38/74), and the most common symptoms were cough, wheezing, chest tightness and dyspnea. The incidences of erythrocyte sedimentation rate (ESR) increasing, C- reactive protein (CRP) increasing, fibrinogen increasing, D- dimer increased and rheumatoid factor (RF) positive in patients with respiratory involvement were significantly higher than those in patients without respiratory involvement: 47.37% (18/38) vs. 30.56% (11/36), 52.63% (20/38) vs. 33.33% (12/36), 31.58% (12/38) vs. 25.00% (9/36), 21.05% (8/38) vs. 13.89% (5/36) and 36.84%(14/38) vs. 5.56% (2/36), and there were statistical differences (P<0.05). CT was the main method to discover the respiratory involvement, and MRI could detect early cartilage inflammation lesions. Laryngoscope and bronchoscope could early detect mucosa and cartilage damage. Pathology was given priority to lymphocytes and neutrophils infiltration. Some patients had epithelium metaplasia and even canceration. Primary treatment methods were glucocorticoids combined with immunosuppressant. Airway stenosis and infection was the main factors influencing the prognosis of patients. Conclusions The respiratory involvement is not uncommon in RPC, and early CT, MRI, laryngoscope and bronchoscope examination is an important means of early diagnosis.Early glucocorticoid combined immunosuppressive therapy is the key to achieve good prognosis.

A Rapidly Expanding Ascending Aortic Aneurysm in a Patient with Relapsing Polychondritis / 대한내과학회지

Here we describe a case of rapidly expanding ascending aortic aneurysm in a patient with relapsing polychondritis. To prevent aneurysm rupture, the patient underwent emergent surgical repair. Silent inflammation can progress in the aorta wall, even in asymptomatic patients with mild disease activity under immunosuppressive treatment, leading to the rapid growth of aortic aneurysms. Close monitoring with routine imaging is needed once a patient with relapsing polychondritis is diagnosed with an aortic aneurysm.

Policondritis recidivante, un desafío diagnóstico / Relapsing Polychondritis, a Constant Challenge

La policondritis recidivante es una enfermedad sistémica, infrecuente, que afecta las estructuras cartilaginosas, predominantemente del pabellón auricular, nariz, articulaciones y árbol traqueobronquial. Se presenta una paciente de sexo femenino de 63 años con historia de estenosis traqueobronquial, condritis clínica, nariz en silla de montar e hipoacusia neurosensorial con diagnóstico de policondritis recidivante. Debido a su baja frecuencia, diagnóstico clínico y similitud con otras enfermedades esta entidad siempre plantea un desafío diagnóstico.

Relapsing Polychronditis is an uncommon, systemic disease that affects the cartilage structures, primarily the elastic cartilage of the ears, nose, joints and the tracheobronchial tree. We present the case of a 63 year-old female with a history of tracheobronchial stenosis, clinical chondritis, saddleback nose and neurosensorial hypoacusis. The diagnosis of Relapsing Polychronditis was reached. Due to its low incidence rate, clinical diagnosis and similarity to other diseases, the diagnosis of this disease always represents a challenge.

Relapsing Polychondritis: A Case Report.

Relapsing polychondritis is a chronic, recurrent, episodic, inflammatory disease of unknown cause affecting predominantly the cartilaginous tissues of the body. Episodes last few days to weeks. All types of cartilages may be involved. The most common presentation is the involvement of pinnae cartilages. The term was coined by Pearson and was first described by Jacksch-Wartenhorst in 1923, who called it polychondropathies. Annual incidence is about 3.5/million with the peak age at onset ranging from 40 to 50 years. Autoimmune etiology is suspected. Diagnosis is based on McAdam et al., criterion. Corticosteroid remains the mainstay of treatment along with the anti-inflammatory drugs. Here we present a case of 18-year-old male who presented with the involvement of the laryngeal cartilages, which is a rare presentation and later there was an involvement of other cartilages too. Our patient had four of McAdams criteria and also responded to steroids.

Syndrome In Question

The authors present a male 40-year-old patient with established diagnosis of Behçet's disease which had evolved to recurrent bilateral auricular polychondritis crises. MAGIC syndrome (mouth and genital ulcers with inflamed cartilage) is rare and groups together patients with this clinical picture without necessarily fulfilling the clinical criteria for Behçet's disease or relapsing polychondritis, demonstrating an independent disorder.

Central nervous system involvement in relapsing polychondritis: 5 case reports and review of literatures / 中华风湿病学杂志

Objective To study the clinical profile of patients with central nervous system (CNS) involvement in relapsing polychondritis (RP).Methods Clinical data of five patients of RP with CNS lesions were collected,and compared with those from the literatures.T test and Fisher exact test were used for statistical analysis.Results Among 205 patients with RP in Peking Union Medical College Hospital,five cases (2.4％) had CNS damage.All 5 cases presented an active onset and occurred in the active phase of RP.All of them manifested as meningoencephalitis,complicating cranial neuropathies in 2 cases (2/5).Cerebrospinalfluid examination revealed non-specific meningeal inflammation,and magnetic resonance image (MRI) showed long T2 signals in brain lesions.Four patients (4/5) showed good response to high-dose glucocorticosteroid plus immunosuppressive agent combined therapy.The average age of our patients was younger than those in the literatures [(44± 14),(58± 11) years,respectively; t=2.547,P＜0.05],while other clinical features was not significantly different between the two groups.Conclusion CNS involvement is a rare condition in RP patients,and usually occurrs in the early course of active RP.Meningoencephalitis/ meningitis is the major clinical manifestations.MRI and cerebrospinal fluid examination may help to confirm the diagnosis.Treatment with corticosteroid and immunosuppressant can result in favorable response.

The correlation between aggrecan degradation and the progression in relapsing polychondritis disease / 中华检验医学杂志

Objective To explore the significance in judging the different clinical stages of relapsing polychondritis (RP) patients through examining the changes of aggrecanase and metabolic fragments of aggrecan.MethodsIn comparison with the control group (20 cases),40 patients were divided into the stable stage group (22 cases) and the active stage group (18 cases).The aggrecanase-generated neoeptitopes in cartilage matrix were analysed by immunohistochemistry and Western blot(WB) respectively.The mRNA and protein levels of aggrecanase-1,2 expressed in cartilage cells were measured by real-time reverse transcriptional polymerase chain reaction(RT-PCR) and WB respectively.The difference of these results among these three groups was analyzed accordingly.ResultsThe expression of aggrecanase-1,2 in mRNA level was measured by real-time RT-PCR.The values of aggrecanase-1,2 mRNA 2 -ΔΔC1 were 1.00 ± 0.26 and 1.00 ± 0.27 in control group,1.47 ± 0.11 and 1.57 ± 0.13 in stable stage group,2.09 ±0.12 and 2.09 ± 0.19 in active stage group respectively.By one-way ANOVA analysis,the difference between every two groups was statistically significant (F was 299.113 and 459.013,P ＜ 0.01 ).In comparison with control group,aggrecanase-1,2 increased significantly in both stable and active stage group (P ＜ 0.01 ) and aggrecanase-1,2 increased more significantly in active stage group than in stable stage group (P ＜ 0.01 ).The results from WB analysis indicated that aggrecanase-1,2 could not be detected in control group,and they were detectable in stable stage group and increased in active stage group at the relative molecular of 68 000 Da or 73 000 Da respectively.The aggrecanase-generated neoeptitopes were analyzed by WB as well.The results indicated that NITEGE and ARGSV could be detected in stable stage group and increased in active stage group at the relative molecular of 70 000 Da or 48 000 Da respectively,but there were no signals in control group.Similar with the previous WB results,no signals of NITEGE or ARGSV eptitopes were detected in normal cartilage matrix ( no red staining) by use of immunohistochemical staining.However,in stable stage group and active stage group,these eptitopes were apparently detected (obviously red staining).ConclusionWith the progression of the RP,the activity of the aggrecanase is enhanced,and the degradation of the aggrecan is increased,associated with the severity of the disease.

Síndrome de Sweet e policondrite recidivante reveladores de síndrome mielodisplásica / Sweet's Syndrome and relapsing polychondritis signal myelodysplastic syndrome

Certas dermatoses, pertencentes ao grupo das síndromes paraneoplásicas mucocutâneas, podem ser o prenúncio de uma neoplasia previamente não conhecida. Tanto a síndrome de Sweet como a policondrite recidivante incluem-se neste grupo. A síndrome de Sweet e a PR são raramente encontradas em um mesmo paciente. A presença de policondrite recidivante e síndrome de Sweet em um mesmo paciente tem se revelado mais frequente em pacientes com neoplasias associadas, sobretudo hematológicas. Relata-se o caso de paciente do sexo masculino, 79 anos, com síndrome de Sweet e policondrite recidivante, em quem, subsequentemente, foi diagnosticada uma síndrome mielodisplásica.

The emergence of certain skin conditions belonging to the group of mucocutaneous paraneoplastic syndromes may indicate the future appearance of a previously unknown malignancy. Sweet's Syndrome and relapsing polychondritis are included in this group. Sweet's Syndrome and relapsing polychondritis are very rarely found together in the same patient. This dual occurrence is more commonly found in cancer patients with associated hematological malignancies. We report the case of a 79year-old male with Sweet's Syndrome and relapsing polychondritis, who was subsequently diagnosed with a myelodysplastic syndrome.

Clinical analysis of 11 patients with relapsing polychondritis / 临床耳鼻咽喉头颈外科杂志

Objective: To improve the understanding of the clinical feature and early diagnosis of relapsing polychondritis(RP). Method: Eleven patients with RP were analyzed retrospectively and the reported literatures were reviewed. Result: Nine cases had initial involvement of auricular cartilage while two polyarthritis. The treatment of 2RP patients with respiralory tract involvement was invalid and 1 patient died. Nine cases were initially misdiagnosed, with a misdiagnosis rate of 81.82%. Conclusion: RP involves cartilage and connective tissue. The prognosis with respiralory tract involvement is poor. RP is a sort of paroxysmal and progressive inflammation involving cartilages all over the body with a variety of clinical manifestations. Early diagnosis of RP is based on full understanding of its clinical features.

Clinical analysis of 17 patients with relapsing polychondritis / 中国医师进修杂志

Objective To enhance the level of the diagnosis and treatment of relapsing polychondritis(RP).Methods Clinical data of 17 patients of RP were studied retrospectively. Results Of the patients,ear was involved in 16 cases, nose in 10 cases, laryngotracheobronchial region in 9 cases, eyes in 8 cases, joints in 4 cases, cardiovascular system in 1 case.Of 17 cases-1 died from respiratory complications. Fifteen patients had recurrence over twice,1 patient had sixth recurrence. Conclusions RP is rare autoimmune system disease, early clinical manifestation are nontypical, misdiagnosis and missed diagnosis. Corticosteroids, immunosuppressive agents, dapsone and surgical operation are used in these patients to control symptoms.

Treatment of laryngotracheal stenosis due to relapsing polychondritis / 中国耳鼻咽喉头颈外科

OBJECTIVE To investigate the clinical characteristics,treatment and prognosis of laryngotracheal stenosis due to relapsing polychondritis (RP).METHODS The clinical data of 6 patients with laryngotracheal stenosis due to RP were reviewed retrospectively.RESULTS From 1996 to 2006,6 patients with laryngotracheal stenosis due to RP were managed in our hospital.Respiratory tract lesions were diffuse and extensive.Of them,4 patients accepted laryngotracheal reconstruction using surgical flaps with or without pedicle,2 accepted dilation of trachea in thoracic segments.Five patients were cured without recurrence.CONCLUSION Treatment of laryngotracheal stenosis due to RP is very difficult.If the surgical opportunity and methods were selected correctly according to each patient,good results can also be obtained.

Clinical analysis of 56 patients with relapsing polychondritis / 中华全科医师杂志

Objective To investigate clinical characteristics of relapsing polychondritis(RP)and to improve early recognition for it.Methods Clinical and laboratory data of 56 patients with RP were analyzed retrospectively.Results Ratio of number of male patients to female ones was 1.2.Age at onset was(46?11)years(ranging from 27 to 71)and average interval between onset and diagnosis was(21? 35)months,(8?6),(16?31)and(29?37)months for patients initial onset with auricle,respiratory tract and joints involved,respectively.Site involved included airway in 40 patients(71.4%),auricle in 32 (57.1%),joints in 32(57.1%),eyes in 27(48.2%),nasal chondritis in 25(44.6%)and inner ear in 13(23.2%).At initial stage of the course,17 patients were misdiagnosed as respiratory infection (30.4%),nine as perichondritis(16.1%),six as pulmonary tuberculosis(10.7%),five as rheumatoid arthritis(8.9%).Seven of 40 patients with airway involvement received metallic stents for their tracheobronchial stenosis.Four patients whose condition never improved after regular therapy all had respiratory involvement.Conclusions Patients of RP with initial onset at non-auricle,non-nasal sites tended to be misdiagnosed.Prevalence of airway involvement was not so low with a poor prognosis in patients of RP.