69: Relapsing Polychondritis Flashcards
What is the epidemiological trend regarding gender in Relapsing Polychondritis?
There is a slight female predilection, with men being affected at an average age of ~55 years and women at ~51 years.
What are the most common cutaneous findings in Relapsing Polychondritis?
The most common cutaneous findings include:
- Auricular chondritis (85%) - pain, redness, swelling of the pinna, which may become soft and ‘cauliflower’ shaped.
- Nasal chondritis (65%) - nasal pain, stuffiness, rhinorrhea, epistaxis, and saddle-nose deformity.
What are the key noncutaneous findings associated with Relapsing Polychondritis?
Key noncutaneous findings include:
- Respiratory tract chondritis (50%): Symptoms include hoarseness, cough, dyspnea, and wheezing.
- Costochondritis (35%): Parietal pain that can compromise respiration.
- Joint pain (30%): Intermittent, migratory arthritis affecting large and small joints.
- Ocular inflammation (60%): Includes episcleritis, scleritis, and keratoconjunctivitis.
What is the role of the immune response in the pathogenesis of Relapsing Polychondritis?
The immune response in Relapsing Polychondritis involves:
- T cells: Primarily CD4.
- Antibodies: Presence of antibodies to collagen type II in about a third of patients, especially in the acute phase.
- Proteolytic enzymes: Such as MMP3, cathepsins K and L, MMP8, MMP9, and elastase.
What are the diagnostic criteria for Relapsing Polychondritis according to the McAdam criteria?
According to the McAdam criteria, a diagnosis of Relapsing Polychondritis requires at least 3 of the following 6 findings:
- Bilateral auricular chondritis
- Nonerosive seronegative inflammatory polyarthritis
- Nasal chondritis
- Ocular inflammation
- Respiratory chondritis
- Cochlear and/or vestibular damage.
What is the typical course and prognosis of Relapsing Polychondritis?
The course of Relapsing Polychondritis is typically progressive with intermittent flares. The Relapsing Polychondritis Disease Activity Index (RPDAI) is used to assess disease activity.
What are the management strategies for Relapsing Polychondritis?
Management strategies include:
- NSAIDs, colchicine, dapsone: For mild auricular or nasal chondritis, arthralgia, and mild arthritis.
- Oral steroids: 0.3-1 mg/kg of body weight.
- Pulse IV steroids: For acute airway obstruction or sudden hearing loss.
- Methotrexate: 0.3 mg/kg/week.
- Cyclophosphamide, Infliximab, Rituximab: For more severe cases.
- Autologous stem cell transplantation: In selected cases.
What is the role of colchicine in the management of Relapsing Polychondritis?
Colchicine is used for mild auricular or nasal chondritis, arthralgia, and mild arthritis in RP.
What are the symptoms of respiratory tract chondritis in Relapsing Polychondritis?
Symptoms include hoarseness, nonproductive persistent cough, dyspnea, and wheezing.
What are the symptoms of vestibular dysfunction in Relapsing Polychondritis?
Symptoms include dizziness, ataxia, nausea, and vomiting.
What is the role of cyclophosphamide in the treatment of Relapsing Polychondritis?
Cyclophosphamide is used as a systemic treatment option for severe cases of RP.
What are the treatment options for mild auricular or nasal chondritis in Relapsing Polychondritis?
Treatment options include NSAIDs, colchicine, and dapsone.
What is the role of autologous stem cell transplantation in Relapsing Polychondritis?
Autologous stem cell transplantation is a treatment option for severe cases of RP.
A patient with RP has sudden hearing loss and acute airway obstruction. What is the recommended immediate treatment?
Pulse IV steroids are recommended for acute airway obstruction and sudden hearing loss.
What are the complications of costochondritis in Relapsing Polychondritis?
Costochondritis can cause parietal pain and may compromise respiration.
What are the symptoms of nasal chondritis in Relapsing Polychondritis?
Symptoms include nasal pain, stuffiness, rhinorrhea, and epistaxis.
Describe the genetic predispositions associated with Relapsing Polychondritis in German and Japanese populations.
In Germans, HLA-DR4 is associated with RP. In Japanese populations, HLA-DRB16:02, HLA-DQB105:02, and HLA-B*67:01 are associated.
A 55-year-old male presents with sudden onset of auricular pain, redness, and swelling, sparing the noncartilaginous lobe. What is the most likely diagnosis, and what is the typical progression of this condition?
The most likely diagnosis is Relapsing Polychondritis (RP). The condition is progressive with intermittent flares.
A patient with Relapsing Polychondritis develops hoarseness, persistent cough, and dyspnea. What complication should you be most concerned about?
The patient may develop upper airway collapse, obstructive respiratory insufficiency, or secondary infections due to respiratory tract chondritis.
What are the major diagnostic criteria for Relapsing Polychondritis according to McAdam’s criteria?
McAdam’s criteria include bilateral auricular chondritis, nonerosive seronegative inflammatory polyarthritis, nasal chondritis, ocular inflammation, respiratory chondritis, and cochlear and/or vestibular damage. A diagnosis requires at least 3 of these 6 criteria.
A patient with RP presents with nasal pain, stuffiness, and epistaxis. What deformity might develop if the condition progresses?
The patient might develop a saddle-nose deformity.
What are the common ocular manifestations of Relapsing Polychondritis?
Ocular manifestations include episcleritis, scleritis, keratoconjunctivitis sicca, iritis, retinopathy, keratitis, corneal perforation, retinal vasculitis, optic neuritis, and blindness.
What are the major and minor criteria in Michet’s diagnostic criteria for Relapsing Polychondritis?
Major criteria: Auricular chondritis, nasal chondritis, laryngotracheal chondritis. Minor criteria: Conjunctivitis, episcleritis, scleritis, or uveitis; hearing loss; vestibular dysfunction; seronegative polyarthritis. Diagnosis requires 2 major or 1 major + 2 minor criteria.
What are the cardiovascular complications associated with Relapsing Polychondritis?
Complications include aortic and/or mitral regurgitation, conduction system impairment, pericarditis, thoracic and abdominal aortitis leading to aneurysms, Takayasu-like aortic arch syndrome, medium- and large-vessel vasculitis, LCV, and thrombophlebitis.
