|
| Reference | Age (yrs.)/sex | RP manifestations | Therapy prior to cardiac disease | Cardiac manifestations (duration of RP at the time of onset) | RP disease activity at time of cardiac diagnosis | Therapy | Response to therapy/clinical outcome |
|
| This case report | 55/M | Auricular chondritis
Nasal deformity
Polyarthritis
Respiratory chondritis | Adalimumab | Obliterative coronary arteritis | Well-controlled | Methotrexate
Etanercept
Mycophenolate
Mofetil
Lefulunomide
Minocycline
Prednisone
Etolac | Sudden cardiac death |
|
| Bowness et al. 1991 [8] | 33/M | Auricular chondritis
Nasal collapse
Polyarthritis
Respiratory chondritis | Prednisolone (10 mg/day) | Complete heart block (4 wks.)
Acute aortic incompetence (8 wks.)
Coronary vasculitis detected postmortem | Active: elevated CRP, right auricular chondritis, inflamed vocal cords, hoarseness | Pacemaker
AVR
Prednisolone (60 mg/day)
Cyclophosphamide (oral, 2 mg/kg/day) | Fatal acute heart failure (5 weeks postop.) |
|
| Vaidynathan et al. 2006 [12] | 26/F | Auricular chondritis
Nasal bridge depression | Prednisolone (10 mg/day for 1 year) | Severe aortic regurgitation (2 yrs.)
Anterior and lateral wall myocardial infarction with right and left coronary artery ostial stenosis (34 mos.) | Inactive | AVR
Prednisolone (20 mg/day) | Fatal cardiac arrest awaiting coronary artery bypass grafting |
|
| Stein et al. 2008 [11] | 30/M | Auricular chondritis
Nasal chondritis Uveitis | Prednisone (30 mg/day for 3 weeks) | Asymmetric proximal aorta and aortic root wall thickening, stenosis of ostium of left and right main coronary arteries, right external iliac artery stenosis (5 yrs.)
Positive cardiac stress test (5 yrs.)
Critical coronary stenosis (6 yrs.) | Active: auricular chondritis
Normal inflammatory markers | AVR
CABG
Prednisone (1 mg/kg/day)
Cyclophosphamide (oral, 100 mg/kg/day)
Infliximab (5 mg/kg/dose) | Improvement in symptoms following surgery |
|
| McCarthy and Cunnane 2010 [15] | 45/M | Auricular deformity
Nasal deformity
Arthralgia
Bilateral sensorineural deafness | Methotrexate
Azathioprine
Cyclosporine A
Infliximab
Corticosteroids
Rituximab | Severe aortic incompetence, normal aortic root, stenosis of main left and right coronary arteries (16 yrs.) | Active: elevated CRP | AVR
CABG
Azathioprine | Clinical remission after 18 mos. |
|
| Sugrue et al. 2014 [16] | 51/M | Auricular chondritis
Arthritis
Aortic root dilation | N/A | Aortic root dilation (at time of diagnosis)
Femoral bruit (4 mos.)
Critical left main stem ostial stenosis (8 mos.)
Progression of aortitis (36 mos.) | Active: elevated inflammatory markers that became quiescent with cardiac disease progression | Prednisolone (1 mg/kg)
MTX (25 mg/wk)
Infliximab
(IV 5 mg/kg, 6 weekly initially, increased to 10 mg/kg, 6 weekly) | Normalization of inflammatory markers Asymptomatic after 12 mos. |
|
