|Year : 2021 | Volume
| Issue : 2 | Page : 76-79
Relapsing polychondritis in an asthma clinic: a pulmonologist perspective
Ravi Dosi1, Gaurav Jain1, Kamendra Singh Pawar1, Nirmal Jain2, Parvez Khan3
1 Department of Pulmonary Medicine, SAMC and PGI, Indore, India
2 Sharda Hospital, Greater Noida, Uttar Pradesh, India
3 Department of Pulmonary Medicine, Datia Government Medical College, Madhya Pradesh, India
|Date of Submission||07-Aug-2019|
|Date of Acceptance||21-Apr-2021|
|Date of Web Publication||4-Aug-2021|
Dr. Gaurav Jain
Junior Resident, Department of Pulmonary Medicine, SAMC and PGI, Indore, Madhya Pradesh
Source of Support: None, Conflict of Interest: None
The first case of relapsing polychondritis (RP) was described in 1923, and yet it remains a “medical mystery”, a much “overlooked” entity. It is an uncommon systemic disorder causing inflammation of cartilage affecting ear, nose, laryngotracheobronchial tree, eyes, joints, heart, blood vessels, kidney, and skin. Being a rheumatological entity presenting with ear and joint involvement most frequently, these patients land up in Otolaryngology and Rheumatology clinics mostly, though tracheobronchial involvement is among the most serious manifestation causing substantial morbidity and mortality. Herein, we report a case of RP and discuss available diagnostic techniques and airway interventions under the ambit of a pulmonologist, as airway involvement severely limits its prognosis.
Keywords: Airway involvement, McAdam criteria, relapsing polychondritis
|How to cite this article:|
Dosi R, Jain G, Pawar KS, Jain N, Khan P. Relapsing polychondritis in an asthma clinic: a pulmonologist perspective. J Assoc Chest Physicians 2021;9:76-9
|How to cite this URL:|
Dosi R, Jain G, Pawar KS, Jain N, Khan P. Relapsing polychondritis in an asthma clinic: a pulmonologist perspective. J Assoc Chest Physicians [serial online] 2021 [cited 2021 Dec 1];9:76-9. Available from: https://www.jacpjournal.org/text.asp?2021/9/2/76/323092
Key Message: Airway involvement is seen in about 21% to 50% patients of relapsing polychondritis with it being the first manifestation in 11% to 30% patients, leading to substantial morbidity and mortality. The diagnosis becomes challenging when chondritis of respiratory tract is an isolated feature. Thus, it is important for pulmonologists to ensure timely diagnosis and offer appropriate therapeutic interventions.
| Introduction|| |
Relapsing polychondritis (RP), first described by Jaksch-Wartenhorst in 1923, is a rare inflammatory disease of unknown cause predominantly affecting cartilaginous tissues of ear, nose, laryngotracheobronchial tree, costal cartilages, and joints. It affects 3.5 per million population per year. Diagnosis of RP is challenging because of its pleomorphic nature and absence of specific laboratory or radiological tests, justifying mean diagnostic delay of 2.9 years. Diagnosis is mainly based on clinical criteria such as that of McAdam, Damiani/Levine, and Michet. Airway involvement poses a great threat in a way that patients are often misdiagnosed, which leads to serious organ-threatening complications later in life, adding to morbidity and mortality and also poor response to medical therapy. This case highlights the increasingly important role of pulmonologists in timely diagnosing of RP using pulmonary function tests, imaging, and bronchoscopy as well as offering appropriate therapeutic interventions in selected cases.
| Case report|| |
A 45-year-old female, with no known comorbidities, was referred from ENT to Department of Pulmonary Medicine with persistent complaints of worsening dyspnea, cough, hoarseness of voice, and wheezing for the last 18 months.
The patient developed complaints of dyspnea 1.5 years back, which was associated with dry cough. She consulted multiple physicians and was prescribed inhalers and short-course steroids which provided a temporary relief and led to the diagnosis of bronchial asthma. But her symptoms persisted and eventually worsened. Meanwhile, she also developed complaint of pain and redness in right eye which was diagnosed as corneal ulcer, was treated conservatively, and took around 1 month to resolve. In the ensuing 12 months, her dyspnea worsened, she developed stridor, and also complained of decreased hearing, tinnitus, and vertigo.
She was also found to have saddle-nose deformity ([Figure 1]) and swelling in distal 3rd and 5th interphalangeal joints with no history of trauma, accident, or surgery. There was no history of intubation in the past. Respiratory examination was suggestive of bilateral wheeze. There was no abnormal neurological sign except possibility of 8th nerve palsy. Her blood examination and chest roentgenogram were normal except erythrocyte sedimentation rate (ESR), which was 58 mm in 1st hour (Wintrobe method).
Her pure tone audiometry was suggestive of mixed hearing loss in right ear and conductive hearing loss in left ear. Her video laryngoscopy report was suggestive of edema and bilateral abductor palsy with possibility of subglottic stenosis after which she was referred to Department of Pulmonary Medicine [Figure 2]. CT neck and chest was suggestive of luminal narrowing at the level of larynx and subglottic region with edema of both false and true vocal cords [Figure 3].
Bronchoscopy also confirmed subglottic stenosis with vocal cord paresis. Pulmonary function test and impulse oscillometry showed the presence of small airway limitation with restrictive ventilatory defect, whereas loop was suggestive of fixed airway obstruction. Connective tissue profile, veneral disease research laboratory (VDRL), and slit and skin smear for Hansen were also negative.
Hence, based on the four McAdams criteria, including Saddle-nose deformity of the nasal cartilage, laryngotracheal involvement in the form of subglottic stenosis, cochleo-vestibular damage shown by right-sided sensorineural hearing loss with history of tinnitus and vertigo and seronegative, nondestructive, asymmetrical arthritis, and by principle of exclusion, diagnosis of RP was made.
| Discussion|| |
Presence of airway symptoms, such as progressive dyspnea, cough, stridor, hoarseness, chest discomfort, tenderness over laryngotracheal cartilages, and features of respiratory failure, is seen in 21% to 50% patients of RP with them being the first manifestation in about 11% to 30% patients., Patients who present with respiratory tract involvement have worse prognosis than those in whom respiratory signs develop later. Though the disease has no sex predilection, airway involvement is found more commonly in females.
From a pulmonologist point of view, it is very important to look at airway involvement in such patients, since it carries poorer prognosis. Airway involvement can be diffuse or localized, involving the extrathoracic airway, intrathoracic trachea, or bronchi. Laryngeal and upper tracheal involvement is more common. Involvement of lower airway may be asymptomatic and found only on CT and pulmonary function testing.
Upper airway obstruction in RP happens as a result of subglottic stenosis, tracheal wall thickening, and tracheobronchomalacia due to collapse of laryngeal or tracheal cartilage. Disruption of mucociliary escalator mechanism at the site of scarring/inflammation and ineffectiveness of cough in clearing secretions due to dynamic airway collapse may also result in pneumonia.
Apart from localizing site of involvement, it is equally important to understand if the airway involvement is dynamic or fixed to plan management and monitor the disease. Thus, interpreting flow volume loops with pulmonary function testing becomes pertinent.
Bronchoscopy may reveal severity and extent of inflammation of tracheobronchial tree and dynamic collapse of airway. Typical findings include supraglottic and false vocal cord edema, subglottic edema, tracheomalacia, or focal airway stenosis, but biopsies do not disclose any distinctive finding in presence of typical clinical presentation. Bronchoscopy carries a serious risk as margin of safety is very narrow in patients of chronic hypoxia.
Trachea is no more a “blind spot” or “forgotten zone” with development of imaging modality such as thoracic CT. CT neck and chest should be performed at the time of diagnosis, including dynamic scans. Typical CT findings may include the presence of tracheobronchial stenosis, airway collapse or narrowing, thickening of walls of trachea and proximal bronchi, and calcified cartilaginous rings. Posterior tracheal membrane is classically spared.
The backbone of medical management in suppressing disease activity of RP lies on steroids. Role of other immunosuppressive or immunomodulatory agents such as colchicine, dapsone, methotrexate, cyclophosphamide, cyclosporine, chlorambucil, azathioprine, mycophenolate mofetil, intravenous immunoglobulin, minocycline, and leflunomide are limited to patients not responding to steroids, requiring very high rose of steroids or having organ-threatening disease. Biological agents like tumor necrosis factor inhibitors may prove to be effective, but their efficacy still needs to be proven. However, medical therapies have not been proven to be effective in stabilizing the airways.
Continuous positive airway pressure provides symptomatic benefit in patients with tracheobronchomalacia.
Invasive interventions such as tracheostomy, balloon dilation, and airway stenting can provide symptomatic relief to carefully selected patients. Tracheostomy is required in the setting of respiratory distress and localized subglottic involvement.
Wu et al reported improvement in spirometry and Six minute walk distance (6MWD) values post airway metallic stenting in RP, with cough, foreign body sensation, mucus production and granulomas as common adverse events not warranting bronchoscopic intervention. Other reported serious complications are stent migration, airway infection, stent obstruction, aspiration pneumonia, and massive hemorrhage.Laryngotracheal tracheal resection and reconstruction and tracheal resection and reconstruction have a decent success rate (>95%) but only at an expense of a high complication rate of 15% to 39%.
Airway involvement has been found to be one of the leading causes of death. The 10-year survival rate has improved considerably, from 55% in 1986 to more than 90% in 1998, as reported by Trentham and Le. It has improved partially due to the availability of interventional treatments, but related morbidity and mortality due to airway involvement are still elevated.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]