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.
What immunological markers are often found in patients with Relapsing Polychondritis?
Markers include antibodies to collagen type II, IX, and XI, matrilin-1, cartilage oligomeric matrix protein, and deposits of Ig and C3 at the perichondrium-cartilage junction.
What is the role of proteolytic enzymes in the pathogenesis of Relapsing Polychondritis?
Proteolytic enzymes such as MMP3, cathepsins K and L, MMP8, MMP9, and elastase contribute to cartilage degradation in RP.
What is the significance of the Relapsing Polychondritis Disease Activity Index (RPDAI)?
The RPDAI is used to assess disease activity in patients with Relapsing Polychondritis.
A patient with RP presents with dizziness, ataxia, and nausea. What is the likely cause?
The symptoms are likely due to vestibular dysfunction, which can occur in RP.
What are the histological findings in the inflammation associated with Relapsing Polychondritis?
Histological findings include perichondral inflammation with infiltrates of lymphocytes, neutrophils, macrophages, and plasma cells.
What are the systemic treatment options for severe cases of Relapsing Polychondritis?
Systemic treatments include methotrexate, cyclophosphamide, infliximab, rituximab, and autologous stem cell transplantation.
What is the role of T cells in the pathogenesis of Relapsing Polychondritis?
T cells, primarily CD4, play a role in the immune response in RP, with APCs activated by HLA-DR.
What are the common noncutaneous findings in Relapsing Polychondritis?
Noncutaneous findings include respiratory tract chondritis, costochondritis, joint pain, ocular inflammation, ear complications, and cardiovascular issues.
What is the typical age of onset for Relapsing Polychondritis in men and women?
The typical age of onset is around 55 years for men and 51 years for women.
What is the most frequent cutaneous finding in Relapsing Polychondritis, and how does it present?
The most frequent cutaneous finding is auricular chondritis, presenting with pain, redness, and swelling of the cartilaginous portion of the pinna, sparing the noncartilaginous lobe.
What is the significance of antibodies to collagen type II in Relapsing Polychondritis?
Antibodies to collagen type II are found in about one-third of patients with RP, especially during the acute phase, indicating a role in the humoral immune response.
What are the complications of respiratory tract chondritis in Relapsing Polychondritis?
Complications include upper airway collapse, obstructive respiratory insufficiency, and secondary infections.
What are the dermatologic manifestations of Relapsing Polychondritis?
Dermatologic manifestations include auricular chondritis (85%) and nasal chondritis (65%), which can lead to saddle-nose deformity.
What is the role of dapsone in the management of Relapsing Polychondritis?
Dapsone is used for mild auricular or nasal chondritis, arthralgia, and mild arthritis in RP.
What are the auditory complications of Relapsing Polychondritis?
Auditory complications include conductive hearing loss, sensorineural hearing loss, and vestibular dysfunction.
What is the role of methotrexate in the treatment of Relapsing Polychondritis?
Methotrexate is used as a systemic treatment option for severe cases of RP.
What are the indications for using pulse IV steroids in Relapsing Polychondritis?
Pulse IV steroids are indicated for acute airway obstruction, sudden hearing loss, and/or before surgical intervention.
What is the significance of HLA-DR4 in Relapsing Polychondritis?
HLA-DR4 is associated with a genetic predisposition to RP, particularly in German populations.
What are the ocular complications that can lead to blindness in Relapsing Polychondritis?
Ocular complications include corneal perforation, retinal vasculitis, optic neuritis, and severe keratitis.
What is the role of infliximab in the management of Relapsing Polychondritis?
Infliximab is used as a systemic treatment option for severe cases of RP.
What are the diagnostic criteria for Relapsing Polychondritis according to Damiana and Levine?
Damiana and Levine criteria require at least 3 of McAdam’s clinical criteria, or ≥1 McAdam clinical criterion plus biopsy confirmation of cartilage inflammation, or chondritis at ≥2 separate anatomic locations with response to steroids and/or dapsone.
What is the role of rituximab in the treatment of Relapsing Polychondritis?
Rituximab is used as a systemic treatment option for severe cases of RP.
