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Relapsing polychondritis

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WHAT IS IT
Relapsing polychondritis (RP) is an immune-mediated condition associated with inflammation in cartilaginous structures. The clinical features and course of RP vary considerably from patient to patient. RP can lead to irreversible damage in the affected tissues and may be life threatening.

WHAT ARE THE MANIFESTATIONS of  RP AND HOW IS IT RECOGNIZED
RP can affect every cartilaginous structure in the body. However, the following are the most frequently affected organs:

  • ears: ear inflammation is the most common clinical feature of RP (up to 90% of patients); patients present with red, swollen and painful auricles; chronic inflammation may lead a permanent alteration of the structural integrity of cartilage, resulting in a "cauliflower" appearance;
  • joints: RP can cause arthritis in up to 75% of patients; affected joints present as red, swollen and painful;
  • nose: symptoms of stuffiness, crusting, rhinorrhea, and epistaxis may accompany nasal cartilage inflammation; cartilage destruction associated with sustained or recurrent episodes of inflammation can result in a characteristic “saddle nose” deformity;
  • eyes: ocular disease affects about 20 percent of RP patients and it may encompass scleritis, uveitis and keratitis; ocular inflammation can lead to loss of vision and a bluish discoloration of the sclera;
  • large airway: RP can cause an inflammatory process of larynx, trachea, and bronchi, thus leading to tracheal narrowing and airway compromise, a life threatening condition needing prompt treatment.

RP may also affect other organs, such as heart. Noteworthy, inflammation can also lead to nonspecific constitutional manifestations such as fever, malaise and weight loss. Up to one third of the patients with RP present with a recognizable form of systemic vasculitis, a conecctive tissue disease or other autoimmune disorder, or a malignat or premalignant condition.
The clinical course of RP is very heterogeneous, ranging from fulminant and life threatening forms to benign variants with favorable prognosis. Elevations in the acute phase reactants are associated with disease activity.
Imaging techniques such as Computed Tomography and 18-Fluorodeoxyglucose PET scan are useful to have a complete picture of the organs involved by RP.
The diagnosis of RP is established by the combination of clinical findings, laboratory data and imaging procedures.

WHO IS AFFECTED
RP is a rare disease that appears to occur among individuals of all races and age groups. Onset is most likely between the ages of 40 and 60.
Men and women are affected equally. The annual incidence has been estimated between 2 and 4 per million persons. RPC is not a familial disease, although studies indicate that some genetic contribution to susceptibility is likely. No environmental factor has been linked to the onset of RP yet.

HOW DO WE TREAT IT 
Early recognition of RP is extremely important in order to avoid complications and irreversible damage to affected organs. Glucocorticoids represent the first line treatment to induce disease remission in RP patients. Long-term use of glucocorticoids is associated with significant adverse effects. Methotrexate and monoclonal antibodies (e.g., infliximab and tocilizumab) are effectively used as steroid-sparing agents to treat RP patients.

SERVICES OFFERED TO PATIENTS WITH RP AT SAN RAFFAELE HOSPITAL: A MULTIDISCIPLINARY APPROACH
Patients with RP referred to San Raffaele Hospital are followed-up via the national health care system in a dedicated outpatient Clinic. Immunologists and Rheumatologists of our team have years of expertise in the field. Noteworthy, since RP can affect many different organs, each case is periodically discussed in multidisciplinary meetings within San Raffaele Hospital to provide the best therapeutic options for each patient. The multidisciplinary team includes ENT specialists, Ophthalmologists, Pulmonologists, Thoracic Surgeons and Radiologists among others. Being recognized as a national and international referral center for RP, San Raffaele Hospital also offers to patients the possibility to be enrolled in research projects and to benefit from cutting-edge off-label treatments.


REFERRING DOCTORS for this desease
at Ospedale San Raffaele

Ospedale San Raffaele - Milano, via Olgettina 60, 20132 Milano, Italia - Tel. 02 26 431
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