International Journal of Current Research and Review
ISSN: 2231-2196 (Print)ISSN: 0975-5241 (Online)
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IJCRR - Vol 11 Issue 07, April, 2019

Pages: 09-11

Date of Publication: 12-Apr-2019


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Relapsing Polychondritis with Seronegative Spondyloarthritis: A Rare Case Report

Author: Archana U., Raju H. B., Sreedhar J., Jyoti H., Shama B., Abhiram M., Rishab A., Rajani D.

Category: Healthcare

Abstract:The aim of this paper is to present a case of relapsing polychondritis and response to cyclophosphamide pulse therapy. A 19 years old female patient presented with inflammatory back pain, asymmetrical oligoartheritis and acute anterior uveitis. CT thorax revealed edema in the glottis and trachea causing narrowing of lumen. Based on the investigations and clinical finding a final diagnosis of relapsing polychondritis was done and started with 5 pulse of cyclophosphamide which showed improvement. Relapsing polychondritis is a rare condition commonly affecting 40-50 years age group. But can be seen in younger age too in both the sexes. Laryngobronchial involvement occurs in approximately 50% of patients, and is among the most serious complications. Here we present the case of a 19 year old female with relapsing polychondritis presenting with stridor due to tracheal involvement.

Keywords: Relapsing Polychondritis, Cyclophosphamide

Full Text:

INTRODUCTION

Relapsing polychondritis is an uncommon disease with an incidence of about 3.5 cases per million population per year. The peak age of onset is between the ages of 40 and 50 years. But relapsing polychondritis may affect both children and the elderly, and affects both sexes equally. Approximately 30% of patients with relapsing polychondritis will have another rheumatologic disorder, the most frequent is systemic vasculitis, followed by rheumatoid arthritis and systemic lupus erythematosus. Diagnosis is based on typical clinical features. McAdam et al. proposed a diagnostic criteria in 1976, which was further modified by Damiani and Levine in 1979.

McAdam et al, proposed the following:-

  1. Recurrent chondritis of both auricles

  2. Nonerosive inflammatory arthritis

  3. Chondritis of nasal cartilage

  4. Inflammation of ocular structures, including conjunctivitis, keratitis, scleritis/episcleritis, and/or uveitis

  5. Chondritis of laryngeal and/or tracheal cartilages

  6. Cochlear and/ or vestibular damage manifested by neurosensory hearing loss, tinnitus, and/or vertigo

Diagnosis is certain when 3 or more of these features are present along with a positive biopsy from the ear, nasal or respiratory cartilage1.

Damiani and Levine later suggested that the diagnosis could be made when;

  1. One of the features and a positive biopsy

  2.  Two or more separate sites of cartilage involvement present that responded to glucocorticoids or dapsone

  3. Three or more of the features mentioned were present.

We are reporting the case of a 19 year old female, who presented with inflammatory arthritis and uveitis, and later developed hoarseness of voice and an acute episode of choking.

CASE REPORT

A 19 year old female presented with symptoms suggestive of inflammatory back pain, asymmetric oligoarthritis along with acute anterior uveitis. MRI pelvis showed evidence of bilateral sacroilitis and she tested negative for Human Leukocyte Antigen B27 (HLAB27). Treatment was initiated with Non steroidal Anti-inflammatory Drugs (NSAIDs) and Sulfasalazine, further in view of lack of response to treatment, the patient was started on Infliximab therapy after a thorough work up. Following the commencement of Infliximab therapy, the joint pains subsided but, developed hoarseness of voice soon after the 1st pulse. In view of hoarseness of voice, an ENT opinion was taken, and the patient was put on steroids considering it to be Reinke’s edema, symptomatic improvement in hoarseness of voice was noted. Upon tapering steroids, the hoarseness of voice returned. In view of relapse of symptoms, an endocrinology opinion was taken to look for an endocrinologic cause for hoarseness, and following thorough evaluation, any endocrinological cause was ruled out.

Three pulses of Infliximab were completed (as per protocol). Later the patient developed an acute choking episode at her college, wherein she was referred to a local hospital and subsequently intubated, and further referred to our hospital. After admission to our hospital, and stabilization of the patient, she was extubated. General physical examination at this juncture revealed a depressed nasal bridge, and tenderness over the larynx. There was no evidence of chondritis of auricles, and no history of any previous episodes of the same. No history suggestive of cochlear or vestibular involvement was elicited. No features suggestive of vasculitis were noted.

With the suspicion of Relapsing Polychondritis, the patient was subjected to a CT Thorax/Neck which revealed edema in the glottis region and trachea from C6 to D5, causing luminal narrowing. A respiratory medicine opinion was taken and a bronchoscopy performed. The bronchoscopy showed severe narrowing of the tracheal lumen. A 2 Dimensional Echocardiogram (2DECHO) performed showed anterior mitral leaflet prolapse. Positron Emission Tomography- Computed Tomography (PET CT) done, showed increased uptake in the tracheal and laryngeal cartilage.

Based on these clinical findings a diagnosis of Relapsing Polychondritis was made. She was started on Cyclophosphamide pulse therapy under the cover of Luperolide, to prevent gonadal toxicity. A repeat bronchoscopy was performed after 5 pulses of cyclophosphamide which showed improvement. A follow up CT scan showed narrowing at C4 level with no signs of edema. Currently the patient is on regular follow up on an outpatient basis, the cyclophosphamide pulses have been tapered, and her condition is stable, with no further episodes of acute respiratory distress.

DISCUSSION

Relapsing polychondritis is a systemic disease causing inflammation of cartilaginous structures in multiple organs2

Approximately one-third of patients with relapsing polychondritis suffer from another underlying autoimmune disease such as systemic vasculitis. There is genetic risk for relapsing polychondritis, as suggested by its association with Human Leukocyte Antigen- DR4 isotope (HLA-DR4), which is associated with multiple autoimmune diseases such as rheumatoid arthritis, multiple sclerosis and others. It is suggested that in RP there are circulating antibodiesagainst type II collagen, which leads to cell-mediated injury to targeted organs in the diseaseprocess3.

Clinical phenotypes of relapsing polychondritis follow two patterns; either nasal cartilage/airway involvement or external ear involvement4. Typically, auricular chondritis occurs in almost 90% of the patients5. However, as demonstrated in our patient, its absence does not rule out the diagnosis of relapsing polychondritis.The respiratory tract is affected in more than 50% of the cases5. Respiratory disturbance results from airway collapse secondary to destruction of tracheal rings or narrowingof the tracheal lumen caused by fibrous or inflammatory edema. Airway manifestations are the commonest cause of morbidity and mortality in the disease6. The management of respiratory tract involvement in relapsing polychondritis is important because sudden respiratory arrest mayoccur5, as manifested by an acute episode of choking in our patient. Failure of early diagnosis of airway involvement in relapsing polychondritis can lead to irreversible cartilage damage. Steroids or other immunosuppressants are ineffective in these advanced cases, and would require airway interventions such as tracheostomy or tracheobronchial stents6.

There is no current standard treatment for relapsing polychondritis, owing in part to its rarity. Corticosteroids are considered to be the first line treatment.6 Steroid sparing agents, such as cyclophosphamide, azathioprine, methotrexate and cyclosporine are also found to be helpful. Our patient achieved remission following cyclophosphamide pulse therapy.

CONCLUSION

Relapsing Polychondritis, is a rare condition, which can present without the classical symptom of auricle chondritis, and as involvement of the laryngotracheobronchial tree alone. In the absence of any specific diagnostic test, a high degree of clinical suspicion is required to arrive at the diagnosis. Recognition and prompt treatment of the airway symptoms of relapsing polychondritits, is beneficial so as to avoid the more invasive airway interventions at more advanced stages of the disease.

Conflict of interest: None

References:

  1. Harrison’s Principles of Internal Medicine, 20th Edition, Chapter 359, Page no 2597-600

  2. Ferrada MA, Grayson PC, Banerjee S et al. Patient-perceptionof disease-related symptoms and complications in relapsing polychondritis. Arthritis Care Res 2017; Advance Access published 15 December 2017, doi: 10.1002/acr.23492.

  3. Maha Almackenzie, Ahmad Alharbi, Sharifa Alhassan, Eleanor Cook and Nezam Altorok, Central Nervous System Vasculitis Associated with Relapsing Polychondritis, Successful Treatment with Cyclophosphamide, The American Journal of the Medical Sciences, http://dx.doi.org/10.1016/j.amjms.2016.11.023.

  4. Shimizu J, Yamano Y, Yudoh K, Suzuki N. Organ involvementpattern suggests subgroups within relapsing polychondritis: comment on the article by Dion et al. Arthritis Rheumatol 2018;70:1489

  5. Vito Sabato, Olivier M Vanderveken, Tim Van den Wyngaert, Carl Van Laer & Didier Ebo (2016): A patient with a severe glottic stenosis and saddle nose, Acta Clinica Belgica, DOI: 10.1080/17843286.2015.1111657

  6. Gorard C, Kadri S. Critical airway involvement in relapsing polychondritis. BMJ Case Rep. 2014; 2014. Published 2014 Sep 11. doi:10.1136/bcr-2014-205036

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One article from every issue is selected for the ‘Best Article Award’. Authors of selected ‘Best Article’ are rewarded with a certificate. IJCRR Editorial Board members select one ‘Best Article’ from the published issue based on originality, novelty, social usefulness of the work. The corresponding author of selected ‘Best Article Award’ is communicated and information of award is displayed on IJCRR’s website. Drop a mail to editor@ijcrr.com for more details.

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This award is instituted to encourage women researchers to publish her work in IJCRR. Women researcher, who intends to publish her research work in IJCRR as the first author is eligible to apply for this award. Editorial Board members decide on the selection of women researchers based on the originality, novelty, and social contribution of the research work. The corresponding author of the selected manuscript is communicated and information is displayed on IJCRR’s website. Under this award selected women, the author is eligible for publication incentives. Drop a mail to editor@ijcrr.com for more details.

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Best Article Award

A Study by Amr Y. Zakaria et al. entitled "Single Nucleotide Polymorphisms of ATP-Binding Cassette Gene(ABCC3 rs4793665) affect High Dose Methotrexate-Induced Nephrotoxicity in Children with Osteosarcoma" is awarded Best Article for Vol 13 issue 19
A Study by Kholis Ernawati et al. entitled "The Utilization of Mobile-Based Information Technology in the Management of Dengue Fever in the Community Year 2019-2020: Systematic Review" is awarded Best Article for Vol 13 issue 18
A Study by Bhat Asifa et al. entitled "Efficacy of Modified Carbapenem Inactivation Method for Carbapenemase Detection and Comparative Evaluation with Polymerase Chain Reaction for the Identification of Carbapenemase Producing Klebsiella pneumonia Isolates" is awarded Best Article for Vol 13 issue 17
A Study by Gupta R. et al. entitled "A Clinical Study of Paediatric Tracheostomy: Our Experience in a Tertiary Care Hospital in North India" is awarded Best Article for Vol 13 issue 16
A Study by Chandran Anand et al. entitled "A Prospective Study on Assessment of Quality of Life of Patients Receiving Sorafenib for Hepatocellular Carcinoma" is awarded Best article for Vol 13 issue 15
A Study by Rosa PS et al. entitled "Emotional State Due to the Covid – 19 Pandemic in People Residing in a Vulnerable Area in North Lima" is awarded Best Article for Vol 13 issue 14
A Study by Suvarna Sunder J et al. entitled "Endodontic Revascularization of Necrotic Permanent Anterior Tooth with Platelet Rich Fibrin, Platelet Rich Plasma, and Blood Clot - A Comparative Study" is awarded Best Article for Vol 13 issue 13
A Study by Mona Isam Eldin Osman et al. entitled "Psychological Impact and Risk Factors of Sexual Abuse on Sudanese Children in Khartoum State" is awarded Best Article for Vol 13 issue 12
A Study by Khaw Ming Sheng & Sathiapriya Ramiah entitled "Web Based Suicide Prevention Application for Patients Suffering from Depression" is awarded Best Article for Vol 13 issue 11
A Study by Purushottam S. G. et al. entitled "Development of Fenofibrate Solid Dispersions for the Plausible Aqueous Solubility Augmentation of this BCS Class-II Drug" is awarded Best article for Vol 13 issue 10
A Study by Kumar S. et al. entitled "A Study on Clinical Spectrum, Laboratory Profile, Complications and Outcome of Pediatric Scrub Typhus Patients Admitted to an Intensive Care Unit from a Tertiary Care Hospital from Eastern India" is awarded Best Article for Vol 13 issue 09
A Study by Mardhiah Kamaruddin et al. entitled "The Pattern of Creatinine Clearance in Gestational and Chronic Hypertension Women from the Third Trimester to 12 Weeks Postpartum" is awarded Best Article for Vol 13 issue 08
A Study by Sarmila G. B. et al. entitled "Study to Compare the Efficacy of Orally Administered Melatonin and Clonidine for Attenuation of Hemodynamic Response During Laryngoscopy and Endotracheal Intubation in Gastrointestinal Surgeries" is awarded Best Article for Vol 13 issue 07
A Study by M. Muthu Uma Maheswari et al. entitled "A Study on C-reactive Protein and Liver Function Tests in Laboratory RT-PCR Positive Covid-19 Patients in a Tertiary Care Centre – A Retrospective Study" is awarded Best Article of Vol 13 issue 06 Special issue Modern approaches for diagnosis of COVID-19 and current status of awareness
A Study by Gainneos PD et al. entitled "A Comparative Evaluation of the Levels of Salivary IgA in HIV Affected Children and the Children of the General Population within the Age Group of 9 – 12 Years – A Cross-Sectional Study" is awarded Best Article of Vol 13 issue 05 Special issue on Recent Advances in Dentistry for better Oral Health
A Study by Alkhansa Mahmoud et al. entitled "mRNA Expression of Somatostatin Receptors (1-5) in MCF7 and MDA-MB231 Breast Cancer Cells" is awarded Best Article of Vol 13 issue 06
A Study by Chen YY and Ghazali SRB entitled "Lifetime Trauma, posttraumatic stress disorder Symptoms and Early Adolescence Risk Factors for Poor Physical Health Outcome Among Malaysian Adolescents" is awarded Best Article of Vol 13 issue 04 Special issue on Current Updates in Plant Biology to Medicine to Healthcare Awareness in Malaysia
A Study by Kumari PM et al. entitled "Study to Evaluate the Adverse Drug Reactions in a Tertiary Care Teaching Hospital in Tamilnadu - A Cross-Sectional Study" is awarded Best Article for Vol 13 issue 05
A Study by Anu et al. entitled "Effectiveness of Cytological Scoring Systems for Evaluation of Breast Lesion Cytology with its Histopathological Correlation" is awarded Best Article of Vol 13 issue 04
A Study by Sharipov R. Kh. et al. entitled "Interaction of Correction of Lipid Peroxidation Disorders with Oxibral" is awarded Best Article of Vol 13 issue 03
A Study by Tarek Elwakil et al. entitled "Led Light Photobiomodulation Effect on Wound Healing Combined with Phenytoin in Mice Model" is awarded Best Article of Vol 13 issue 02
A Study by Mohita Ray et al. entitled "Accuracy of Intra-Operative Frozen Section Consultation of Gastrointestinal Biopsy Samples in Correlation with the Final Histopathological Diagnosis" is awarded Best Article for Vol 13 issue 01
A Study by Badritdinova MN et al. entitled "Peculiarities of a Pain in Patients with Ischemic Heart Disease in the Presence of Individual Combines of the Metabolic Syndrome" is awarded Best Article for Vol 12 issue 24
A Study by Sindhu Priya E S et al. entitled "Neuroprotective activity of Pyrazolone Derivatives Against Paraquat-induced Oxidative Stress and Locomotor Impairment in Drosophila melanogaster" is awarded Best Article for Vol 12 issue 23
A Study by Habiba Suhail et al. entitled "Effect of Majoon Murmakki in Dysmenorrhoea (Usre Tams): A Standard Controlled Clinical Study" is awarded Best Article for Vol 12 issue 22
A Study by Ghaffar UB et al. entitled "Correlation between Height and Foot Length in Saudi Population in Majmaah, Saudi Arabia" is awarded Best Article for Vol 12 issue 21
A Study by situs slot and Siti Sarah Binti Maidin entitled "Sleep Well: Mobile Application to Address Sleeping Problems" is awarded Best Article for Vol 12 issue 20
A Study by Avijit Singh et al. situs slot "Comparison of Post Operative Clinical Outcomes Between “Made in India” TTK Chitra Mechanical Heart Valve Versus St Jude Mechanical Heart Valve in Valve Replacement Surgery" is awarded Best Article for Vol 12 issue 19
A Study by Sonali Banerjee and Mary Mathews N. entitled "Exploring Quality of Life and Perceived Experiences Among Couples Undergoing Fertility Treatment in Western India: A Mixed Methodology" is awarded Best Article for Vol 12 issue 18
A Study by Jabbar Desai et al. entitled "Prevalence of Obstructive Airway Disease in Patients with Ischemic Heart Disease and Hypertension" is awarded Best Article for Vol 12 issue 17
A Study by Juna Byun et al. entitled "Study on Difference in Coronavirus-19 Related Anxiety between Face-to-face and Non-face-to-face Classes among University Students in South Korea" is awarded Best Article for Vol 12 issue 16
A Study by Sudha Ramachandra & Vinay Chavan entitled "Enhanced-Hybrid-Age Layered Population Structure (E-Hybrid-ALPS): A Genetic Algorithm with Adaptive Crossover for Molecular Docking Studies of Drug Discovery Process" is awarded Best article for Vol 12 issue 15
A Study by Varsha M. Shindhe et al. entitled "A Study on Effect of Smokeless Tobacco on Pulmonary Function Tests in Class IV Workers of USM-KLE (Universiti Sains Malaysia-Karnataka Lingayat Education Society) International Medical Programme, Belagavi" is awarded Best article of Vol 12 issue 14, July 2020
A study by Amruta Choudhary et al. entitled "Family Planning Knowledge, Attitude and Practice Among Women of Reproductive Age from Rural Area of Central India" is awarded Best Article for special issue "Modern Therapeutics Applications"
A study by Raunak Das entitled "Study of Cardiovascular Dysfunctions in Interstitial Lung Diseas epatients by Correlating the Levels of Serum NT PRO BNP and Microalbuminuria (Biomarkers of Cardiovascular Dysfunction) with Echocardiographic, Bronchoscopic and HighResolution Computed Tomography Findings of These ILD Patients" is awarded Best Article of Vol 12 issue 13 
A Study by Kannamani Ramasamy et al. entitled "COVID-19 Situation at Chennai City – Forecasting for the Better Pandemic Management" is awarded best article for  Vol 12 issue 12
A Study by Muhammet Lutfi SELCUK and Fatma situs slot entitled "Distinction of Gray and White Matter for Some Histological Staining Methods in New Zealand Rabbit's Brain" is awarded best article for  Vol 12 issue 11
A Study by Anamul Haq et al. entitled "Etiology of Abnormal Uterine Bleeding in Adolescents – Emphasis Upon Polycystic Ovarian Syndrome" is awarded best article for  Vol 12 issue 10
A Study by situs slot et al entitled "Estimation of Reference Interval of Serum Progesterone During Three Trimesters of Normal Pregnancy in a Tertiary Care Hospital of Kolkata" is awarded best article for  Vol 12 issue 09
A Study by Ilona Gracie De Souza & Pavan Kumar G. entitled "Effect of Releasing Myofascial Chain in Patients with Patellofemoral Pain Syndrome - A Randomized Clinical Trial" is awarded best article for  Vol 12 issue 08
A Study by Virendra Atam et. al. entitled "Clinical Profile and Short - Term Mortality Predictors in Acute Stroke with Emphasis on Stress Hyperglycemia and THRIVE Score : An Observational Study" is awarded best article for  Vol 12 issue 07
A Study by K. Krupashree et. al. entitled "Protective Effects of Picrorhizakurroa Against Fumonisin B1 Induced Hepatotoxicity in Mice" is awarded best article for issue Vol 10 issue 20
A study by Mithun K.P. et al "Larvicidal Activity of Crude Solanum Nigrum Leaf and Berries Extract Against Dengue Vector-Aedesaegypti" is awarded Best Article for Vol 10 issue 14 of IJCRR
A study by Asha Menon "Women in Child Care and Early Education: Truly Nontraditional Work" is awarded Best Article for Vol 10 issue 13
A study by Deep J. M. "Prevalence of Molar-Incisor Hypomineralization in 7-13 Years Old Children of Biratnagar, Nepal: A Cross Sectional Study" is awarded Best Article for Vol 10 issue 11 of IJCRR
A review by Chitra et al to analyse relation between Obesity and Type 2 diabetes is awarded 'Best Article' for Vol 10 issue 10 by IJCRR. 
A study by Karanpreet et al "Pregnancy Induced Hypertension: A Study on Its Multisystem Involvement" is given Best Paper Award for Vol 10 issue 09
Late to bed everyday? You may die early, get depression situs slot
Egg a day tied to lower risk of heart disease situs slot
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A Study by Ese Anibor et al. "Evaluation of Temporomandibular Joint Disorders Among Delta State University Students in Abraka, Nigeria" from Vol 13 issue 16 received Emerging Researcher Award


A Study by Alkhansa Mahmoud et al. entitled "mRNA Expression of Somatostatin Receptors (1-5) in MCF7 and MDA-MB231 Breast Cancer Cells" from Vol 13 issue 06 received Emerging Researcher Award


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