What is Relapsing Polychondritis?
Relapsing Polychondritis is a rare, chronic, multi-system, inflammatory/degenerative disease with varying clinical manifestations that can compromise the structural and functional integrity of cartilage and other connective tissue. It is part of the rheumatic disease family. The classic hallmarks of RPC are repeated episodes of chondritis (inflammation of cartilage) involving the ear, nose and trachea. Inflammation at these sites can lead to cartilage destruction and result in deformity of the ears, the nose, and obstruction of the trachea, which leads to difficulty breathing. [TOP]
Does Relapsing Polychondritis affect only cartilage?
No. In addition, it can affect proteoglycan-rich tissues, such as the eyes, aorta, heart, and skin. Other problems include pain, arthritis, eye inflammation, hearing and balance disorders, vasculitis, endocrine system, skin disorders and cranial nerve or brain involvement. Additionally, up to 30% of persons with RPC have another autoimmune disease. TOP]
What causes RPC?
The cause of RPC is still unknown, but there are several lines of evidence that suggest that an autoimmune response directed against cartilage may lead to this disease.
Trauma to the cartilage structure has also been speculated to cause RPC to develop in that area. One medical journal noted that a women developed RPC in her upper ear after having the cartilage portion of the ear pierced. [TOP]
Does Everyone Progress the Same?
The course of this disease is poorly understood. In some persons RPC assumes a fluctuating but progressive course in which repeated bouts of inflammation may lead to permanent destruction of the involved structure. While others, may have a more limited pattern of disease. While RPC has classic hallmart features these may not be the presenting symptoms of the disease. Nor do all RPCer's develop these symptoms during the course of their disease. [TOP]
Does it affect any particular age groups?
No, RPC can develop at any age. There are reports of children as young as 18months old developing RPC and well as persons in their '90's. The mean age of diagnosis is in the 40's. Although symptoms may have appeared years earlier. [TOP]
I read where younger persons who develop RPC have a worse prognosis is this true?
With better awareness of RPC doctors are now diagnosis it earlier and at much younger ages. While it may be true for some young persons depending on the severity of there case. Properly treated people with RPC are now living longer and more actively then 5 years ago. [TOP]
Is there a cure?
No, however there are medications and treatments available that can effectively control your RPC. [TOP]
What is a flare?
A flare is a reoccurrence of RPC after a period of months of non disease activity. [TOP]
Why am I flaring 2 or 3 times a week or a month?
Disease activity that appears in this fashion indicates your RPC is not under control yet and that your medical treatment plan may need to be adjusted to quiet your RPC [TOP]
What is remission?
Remission is the disappearance of symptoms during the course of an illness. When this happens, the illness is said to be "in remission". This condition may be temporary, sometimes lasting for years before symptoms reoccur. [TOP]
Is it hereditary?
At this time RPC is not believed to be hereditary, however there are several families with documented and diagnosed RPC in them. Siblings are the most commonly found grouping, although several twins only one twin developed RPC. The next factor seen is parent and child, then grandparent and child. TOP]
I have RPC, will my children get it also?
Most likely no. At this point in time RPC is not believed to be hereditary. While we do have some families with proven RPC in their family these are exceptions. [TOP]
Does a particular diet help?
A healthy balanced diet well improve your overall health. To date there has been no provable research or evidence that any particular diet works to cure or control RPC.
What are the treatments available?
Presently treatments for RPC very with each person depending on their specific case, and can consist of the use of prednisone, NSAIDs, DMARDs, TNF's and/or Biologics. To control the RPC, organ specific medications such as medications to control blood pressure, breathing etc may also be needed. [medications] [TOP]
Who should I see for treatment of my RPC?
RPC is a systemic disease the best approach is to use a team of doctors in the specialities that your RPC has affected. Generally a Rheumatology will take the lead in controlling the overall course of your RPC. However, he/she will often refer you to ENT's, pulmonary doctors or other specialist as needed. [TOP]
Are there any doctors who specialize in RPC?
RPC is a very rare disease many doctors have only read about it and have not had patients with RPC. There are doctors that have an interest in RPC and have vast experience in treating RPC. When possible it is highly recommended to us a doctor that had current knowledge of RPC and knows how to treat this disease.
What is a Rare Disease or Disorder?
Rare diseases and disorders are those which affect small patient populations, defined as fewer than 200,000 individuals in the United States. Taken together 25,000,000 Americans suffer from one of 6,000 rare diseases or disorders.
Rheumatic disease represents more that 100 different diseases that are chronic and unpredictable. More than 40 million Americans, or one in six, have a rheumatic disease, including more than 285,000 children. Rheumatic disease as a group are the most frequently occurring chronic illness in the United States. It is a leading cause of work-related disability in people age 16 -72 and is the leading cause of disability for Americans over 65. [TOP]
Are there any support groups for RPC?
Yes, PES hosts two on-line support groups and is in the process of starting community based support groups.
