Relapsing polychondritis with large airway involvement

Yu, Christiaan; Joosten, Simon A.

doi:10.1002/rcr2.501

Cited by 8 publications

(11 citation statements)

References 7 publications

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“…Most published cases of RP have presented with auricular inflammation and/or respiratory symptoms, but only a few cases manifested idiopathic orbital inflammation [ 1 , 2 , 16 , 17 , 23 ]. Those cases were commonly associated with ANCA vasculitis, ankylosing spondylitis, systemic lupus erythematosus, or Sjogren’s syndrome; however, none have been reported with reactive arthritis [ 1 , 24 ].…”

Section: Discussionmentioning

confidence: 99%

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Unusual Presentation of Relapsing Polychondritis in a Patient with Human Immunodeficiency Virus and Reactive Arthritis

Alfraji

Alpert

2021

Am J Case Rep

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Patient: Female, 58-year-old Final Diagnosis: Relapsing polychondritis Symptoms: Ear pain • eye pain Medication: — Clinical Procedure: — Specialty: Rheumatology Objective: Rare disease Background: Relapsing polychondritis (RP) is an uncommon autoimmune condition that impacts cartilaginous structures involving the ears, nose, respiratory tract, and joints. Its etiology is unknown; however, it may be associated with other systemic autoimmune diseases, malignancy, and rarely with human immunodeficiency virus (HIV) infection. RP has a variable pattern at presentation and may be associated with constitutional symptoms such as fever and arthralgia, in addition to various auricular, ocular, respiratory, and cardiovascular manifestations. Auricular and ocular signs are the most common presenting features; however, idiopathic orbital inflammatory syndrome is considered a rare manifestation of the disease. Systemic corticosteroids are the mainstay of treatment, but immunomodulatory therapy may be required for refractory cases. Case Report: We present a challenging case of RP in a 58-year-old woman with HIV controlled by highly active anti-retroviral therapy (HAART) and stable chronic reactive arthritis on sulfasalazine who developed unilateral auricular chondritis associated with contralateral idiopathic orbital inflammation and scleritis as well as worsening arthralgia. She was initially treated empirically with antibiotics, without clinical improvement. The infectious diseases work-up was unrevealing, and other diagnostic possibilities were meticulously excluded. Clinical suspicion for RP ultimately led to appropriate therapy with corticosteroids and subsequent immunosuppression with methotrexate, resulting in clinical improvement and allowing for gradual tapering of steroids. Conclusions: RP is an uncommon multisystem disorder that can occur in the setting of other underlying chronic illnesses, as seen in our patient. It has a variable presentation and course, with no diagnostic laboratory tests; therefore, clinical suspicion is imperative for appropriate diagnosis and management.

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Section: Discussionmentioning

confidence: 99%

“…Only few reported an association with HIV infection [ 11 – 14 ]. The mainstay of treatment in most cases was systemic corticosteroids with or without immunosuppressive agents [ 1 , 2 , 16 , 17 , 23 ].…”

Section: Discussionmentioning

confidence: 99%

Unusual Presentation of Relapsing Polychondritis in a Patient with Human Immunodeficiency Virus and Reactive Arthritis

Alfraji

Alpert

2021

Am J Case Rep

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“…Hazra et al reported that respiratory symptoms were associated with the longest delay in diagnosis, with a median delay time of 10.4 years, while leaving 64% of patients pending diagnosis for more than two years [5]. The presence of respiratory symptoms in a patient known to have RP raises the clinical suspicion of airway involvement [15], but it would be challenging to establish the diagnosis of RP in someone presenting only with cough and dyspnoea. Considering the non‐specific and airway‐only manifestation of our patient, it was not helpful to diagnose his RP by applying those aforementioned sets of criteria.…”

Section: Discussionmentioning

confidence: 99%

“…Around 35–40% of patients with known airway involvement eventually required some form of interventions, such as tracheostomy, balloon dilatation, stenting, or a combination [2,3,10]. Moreover, for patients with long‐standing RP, life‐threatening airway collapse could be the first manifestation to unveil occult airway involvement [15]. The treatment of RP is largely based on experiences from previous case reports and small series, and there are still no evidence‐based guidelines [1,3,17].…”

Section: Discussionmentioning

confidence: 99%

Relapsing polychondritis with isolated tracheal involvement and airway‐only symptoms

Wang

Weng

Cheng

et al. 2020

Respirology Case Reports

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Relapsing polychondritis (RP) is a rare autoimmune disorder, characterized by the inflammation of cartilaginous structures and proteoglycan‐rich tissues. Due to its rarity and the notoriously variable presentations, the diagnosis of RP could be challenging. We report an unusual case of RP with isolated tracheal involvement and very non‐specific symptoms of exertional dyspnoea and dry cough. The initial chest radiograph showed long‐segment narrowing of the trachea, and the computed tomography of the chest revealed thickened cartilaginous walls of the trachea, while the posterior membranous portion was spared. The tracheal narrowing was readily observed under bronchoscopy. The patient was treated with oral prednisolone. Although the subsequent course was transiently complicated by an episode of severe Pneumocystis jirovecii pneumonia with acute respiratory distress syndrome, the patient overall responded well to systemic corticosteroid therapy. No new symptoms developed during a two‐year follow‐up.

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“…The diverse and nonspecific clinical features of RP alongside its relative rarity frequently lead to a diagnostic delay [ 8 ] which is associated with the lack of ear, nose, or joint involvement but presents with early signs of intermittent arthritis or eye involvement in such cases [ 4 ]. RP patients may develop significant disabilities during the course of the disease such as hearing and visual impairments, and 30–50% of patients suffer pulmonary complications [ 9 , 10 ].…”

Section: Introductionmentioning

confidence: 99%

Coexistence of Relapsing Polychondritis and Sickle Cell Disease in a Child

Tetteh

Yebuah

Amissah-Arthur

et al. 2021

Case Reports in Rheumatology

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Relapsing polychondritis (RP) is a rare, severe connective tissue disease of unknown etiology affecting cartilaginous and proteoglycan-rich structures in an episodic and inflammatory manner. Approximately a third of RP cases occur in conjunction with another disease usually systemic autoimmune rheumatic disease, or myelodysplastic syndrome. Sickle cell disease (SCD) is a common inherited hematologic condition characterized by the inheritance of two abnormal hemoglobins, of which one is a hemoglobin S, presenting with severe acute and chronic complications from vaso-occlusive phenomena, which can be difficult to differentiate from RP. The pathogenesis of RP is poorly understood but suggests an autoimmune mechanism with a link to sickle cell disease yet to be established. Treatment is empiric with steroids, anti-inflammatory, and disease-modifying antirheumatic drugs being the mainstay of therapy. Severe complications occur despite treatment, with respiratory involvement being the most catastrophic. This case report reviews a complex case of RP in an 11-year-old girl with sickle cell disease (SF genotype) presenting with bilateral red painful eyes, a painful swollen left ear, and knee pain. Laboratory findings revealed elevated inflammatory markers with negative immune serology. A diagnosis of RP was made based on the patient's symptomatology, presentation, and fulfillment of 5 out of the 6 clinical features using McAdam’s criteria. Management was instituted with a myriad of conventional and biologic DMARDs and other anti-inflammatory medications with no significant improvement and the development of complications of airway obstruction from disease activity and osteoporotic fracture from steroid therapy and underlying hemoglobinopathy. In children, the diagnosis of RP is delayed or overlooked due to its low incidence, variability in clinical symptoms, or sharing similar clinical features with other coexisting disease entities. This article reports its occurrence in the pediatric population and highlights the difficulty in managing such cases as there are no defined standard treatment protocols.

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Relapsing polychondritis with large airway involvement

Cited by 8 publications

References 7 publications

Unusual Presentation of Relapsing Polychondritis in a Patient with Human Immunodeficiency Virus and Reactive Arthritis

Unusual Presentation of Relapsing Polychondritis in a Patient with Human Immunodeficiency Virus and Reactive Arthritis

Relapsing polychondritis with isolated tracheal involvement and airway‐only symptoms

Coexistence of Relapsing Polychondritis and Sickle Cell Disease in a Child

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