Refractory relapsing polychondritis: challenges and solutions

Lekpa, Fernando Kemta; Chevalier, Xavier

doi:10.2147/oarrr.s142892

Cited by 32 publications

(22 citation statements)

References 74 publications

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“…The diagnostic delay is associated with the lack of ear, nose or joint involvement. 10–13 Early signs of RP in such cases may be intermittent arthritis or eye involvement such as episcleritis and scleritis, which may point a search for RP. However, even in the presence of external ear inflammation, an infection, local trauma, insect bite and chondrodermatitis nodularis helicis (Winkler’s disease) are often suspected first.…”

Section: Resultsmentioning

confidence: 99%

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Relapsing polychondritis: state of the art on clinical practice guidelines

Rednic

Damian

Talarico³

et al. 2018

RMD Open

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Due to the rarity of relapsing polychondritis (RP), many unmet needs remain in the management of RP. Here, we present a systematic review of clinical practice guidelines (CPGs) published for RP, as well as a list of the most striking unmet needs for this rare disease. We carried out a systematic search in PubMed and Embase based on controlled terms (medical subject headings and Emtree) and keywords of the disease and publication type (CPGs). The systematic literature review identified 20 citations, among which no CPGs could be identified. We identified 11 main areas with unmet needs in the field of RP: the diagnosis strategy for RP; the therapeutic management of RP; the management of pregnancy in RP; the management of the disease in specific age groups (for instance in paediatric-onset RP); the evaluation of adherence to treatment; the follow-up of patients with RP, including the frequency of screening for the potential complications and the optimal imaging tools for each involved region; perioperative and anaesthetic management (due to tracheal involvement); risk of neoplasms in RP, including haematological malignancies; the prevention and management of infections; tools for assessment of disease activity and damage; and patient-reported outcomes and quality of life indicators. Patients and physicians should work together within the frame of the ReCONNET network to derive valuable evidence for obtaining literature-informed CPGs.

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Section: Resultsmentioning

confidence: 99%

“…Cases of necrotising scleritis treated with biologics (infliximab or adalimumab) have also been reported. 10 11 The use of cyanoacrylate glue repairment has been reported for peripheral corneal perforations. 22 …”

Section: Resultsmentioning

confidence: 99%

Relapsing polychondritis: state of the art on clinical practice guidelines

Rednic

Damian

Talarico³

et al. 2018

RMD Open

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“…The chemical therapy treatment has been applied according to the indicators obtained from the patient's anamnesis. In case of pain control and inflammation in nonsevere forms, non-steroidal anti-inflammatory drugs (NSAIDs) were used [14,[17][18][19]. For mild manifestations chemical treatment is with Dapsone or Colchicine [20][21][22].…”

Section: Resultsmentioning

confidence: 99%

“…The relative rarity of the disease causes difficulties in evaluating potential therapies. Biologic therapy brings hope in conventional immunosuppressant resistant cases [18,23,26]. The anti-TNF-α agents are also of interest in cases with pulmonary involvement, the leading cause of mortality and morbidity in RP.…”

Section: Figure 2 Intraocular Pressure Evolution Under Glaucoma Therapymentioning

confidence: 99%

Importance of Chemical Therapy in Relapsing Polychondritis � a Disease with Impact on the Eye Tissues

Jurja¹,

Mehedinți²,

Sîrbu³

et al. 2020

Rev. Chim.

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Relapsing polychondritis (RP), falls into the category of rare diseases. The true incidence and prevalence of this rare disease is unknown. The ocular implications in relapsing polychondritis (RP) are numerous and variable, including mainly inflammation in different structures of the eye. As a complication of this inflammatory condition, a closed secondary angle glaucoma has been described. The purpose of our work is to highlight the diversity of ocular determinations of the same rare disease, including different types of glaucoma that may occur under the same circumstances and to make a detailed analysis of chemical therapies based on drug treatment pathways. The paper includes a report of the cases series admitted to the Clinic of Ophthalmology in St. Andrew Emergency Clinical Hospital, Constanta, between 2007 and 2018, cases analyzed and compared with international literature. One of the cases is a 43-year-old male patient with bilateral open-angle bilateral glaucoma. Other cases with RP, are patients of 41 and 46 years old, respectively, presented with unilateral episcleritis. This case series report aims to show that RP can associate even more protean ocular manifestations than already discussed in the literature, with specific chemical therapies and to emphasize the need for team approach and ophthalmological monitoring in the care of RP patients with chemical therapy (drug treatment) for each patient.

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“…Larynx, trachea, and bronchi are most frequently affected. Due to the rarity of the disease, there is no standardized treatment approach, and the treatment is guided by the clinical presentation and the severity of the disease [9]. The presented case was a patient who was diagnosed with relapsing polychondritis 2 years ago and developed tracheal involvement while under treatment, resulting in death.…”

Section: Discussionmentioning

confidence: 99%