Polychondritis News
Serving the Polychondritis Community
Winter 2007 Issue.
- Optic neuropathy associated with periostitis in relapsing polychondritis.
- Diagnosis and treatment of relapsing polychondritis
- Neurologic involvement in relapsing polychondritis.
- Case of relapsing polychondritis followed by cognitive impairment.
- Clinical features and treatment of relapsing polychondritis with involvement of the respiratory tract-report of thirteen cases
C. Stephen Foster, M.D.
Keratoconjunctivitis sicca syndrome (KCS or dry eye) is a problem of major epidemiologic importance. It affects literally millions of people around the globe, with women dramatically over represented, particularly those women who have entered menopause. The problem may accompany dry mouth, and may be found in association with a systemic disease such as rheumatoid arthritis or systemic lupus erythematosus. It is, in many instances, far more than a simple "nuisance" problem. It has the potential for serious ocular consequences, beginning with the formation of dry spots on the cornea, progressing to epithelial defects or "abrasions" which resist healing, and then in some instances eventuating to ulceration of the cornea, sometimes even with frank perforation.
The mainstay of treatment for dry eye syndrome through the years has been replacement of fluid through the use of artificial tears. And while this is an important approach to the treatment of dry eye, it is by no means the only (or perhaps even the most important) approach. Conservation measures are also extremely important: the use of punctum plugs to reduce the lose of fluid from the eye through the nasolacrimal duct into the lacrimal sac and then down the throat, the use of side shield panels to spectacles to reduce the amount of air flow across the cornea and hence reduce evaporation, and the use of humidification techniques at home, again, in an effort to reduce the rate of loss of fluid from the surface of the eyes through evaporation. Additionally, we strongly believe that increasing the flow of oil from the oil ducts in the eyelids that supply a thin film of oil to the preocular tear film is extremely useful and important in the care of patients with KCS. Almost every patient whom I see with dry eye has a significant contribution from the oil component of the tear film being deficient. The use of warm compresses twice daily with gentle lid massage to dilate the oil ductules in the lids, further liquefy the oil, which sometimes "sets up" like toothpaste inside the oil ducts, promotes continual flow of oil to the tear film, producing a much more stable tear film and a tear film which is much more efficient at keeping the surface of the eye lubricated, retarding evaporation of the liquid from the surface of the eye, and promoting more uniform spreading of the tear film across the entire extent of the ocular surface.
The use of anti-inflammatory and immunomodulatory agents by topical application also play a role in many patients' dry eye care, since inflammation of the lacrimal gland has been shown to be present and to be hindering the normal function of that tear-producing gland in a significant proportion of patients with dry eye disease. Hence, topical Alrex (a weak corticorsteroid) and topical Restasis (containing cyclosporin) have been shown to be beneficial in such patients. Additionally, dietary supplementation with foods rich in omega 3 free fatty acids, such as cold water fish (eg, salmon) can also help in the restoration of the normal health and functioning of the dysfunctional lacrimal and meibomian glands. Finally, the use of specially constructed scleral lenses (see prior article from August on Contact Lenses) can additionally be helpful in retaining layer of liquid against the cornea in patients who have profound lack of adequate tears.
Reprint courtesy of: C. Stephen Foster, M.D. all rights reserved.
Although RP is rare, medical research is being done to try to expand our understanding of the disease. Physicians continue to observe patients and their response to different treatments, and report their findings in the medical literature. Several groups of scientists are examining animal models of the disease, and others are trying to identify new laboratory markers of disease activity.
The ideal approach to understanding an uncommon disease is to gather information from as many patients as possible. Dr. Jane Hoyt Buckner, M.D. has been studying RP since 1996 and has established a RP registry and sample repository. Her work is designed to examine both the clinical and immunologic questions important to understanding RP. Presently her work includes establishing a database of information on patients with RP, so that the number individuals affected and the course of disease can be better documented. Dr. Buckner is also using blood samples donated by patients for her studies in order to expand our understanding of what is causing relapsing polychondritis. These studies include studies of the genes that may contribute to disease, the antibodies that patients make against collagen and the T cells which may be driving the inflammation of cartilage. The goal of this work is to better be able to identify patients with relapsing polychondritis by developing better laboratory tests, to be able to better predict the potential disease activity in patients and to develop improved therapies and potentially a cure for patients with RP.
Relapsing Polychondritis Clinic at Virginia Mason Clinic
- Dr. Buckner has a weekly clinic devoted to seeing patients with relapsing polychondritis.
- When possible it is helpful to have a referral from your physician, and to bring your medical records at the time of your appointment.
- Visits to this clinic are considered medical consultations, and patients seen in the RP clinic will be billed for the visit unless they make specific arrangements with Dr. Buckner prior to their visit.
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- An appointment can be made by calling (206) 223-6824
- How you can participate in this research?
Any individual with relapsing polychondritis is welcome to take part in Dr. Buckner's research. There are several ways in which people may participate.
- Become part of the RP Registry - We have established the registry, which allows us to gather information from people with RP. To participate we ask that you fill out a form about yourself and your illness. Once we receive that information, we will then contact you to verify that information for our database. We will then contact you on an annual basis to gather up to date information on what has happened to you in the past year. All information is confidential.
- Donate a blood sample for the RP sample repository. Blood samples can be drawn by your local laboratory and sent to our laboratory via federal express. We supply the necessary kits for this process. These samples are used for studies of the immune response in relapsing polychondritis, and to evaluate potential genes involved in the disease. These are research studies, and although we hope to develop useful lab tests for clinical use in the future, these tests are not used in the management of individual patients.
Kelly Yim, CCRC, Clinical Research Coordinator Translational Research Program
Benaroya Research Institute at Virginia Mason
1201 Ninth Avenue Seattle, WA 98101-2795
P: 800-354-9527 x64297
F:206.223.7543
by: Dr. D’Cruz
FEBRUARY 2007
‘Relapsing polychondritis remains one of the rarest of the systemic vasculitides. However, over the last twenty years there has been increasing recognition of this distressing and occasionally life-threatening disorder. Clinical diagnostic criteria have been described and there have been a number of new treatments.
The rarity of RP often leads to delay in diagnosis unless the presenting symptoms are very characteristic. These typical features include collapse of the nasal bridge, inflammation of the ears and the main airways.
However, most patients present with symptoms such as cough, breathlessness, redness of the eyes and chest wall pains that are not specific to RP. Because these symptoms are very common, most physicians do not consider the possibility of RP and this may lead to considerable delay. This is further compounded by the complete lack of specific diagnostic blood tests.
Polychondritis may occur in other vasculitic diseases including Wegener’s granulomatosus and Churg Strauss syndrome. In these diseases the presence of the antineutrophil cytoplasmic antibodies (ANCA) is often helpful.
Corticosteroids including Prednisolone remain the most commonly prescribed drugs and are effective. The list of side-effects is however long and there is a tendency among non-specialists to use very large doses, increasing the risk of side-effects. In the Vasculitis Clinic at St Thomas’ Hospital, we commonly use steroid sparing drugs such as Methotrexate and Azathioprine. For very severe disease, Cyclosphosphamide given by drip in low doses has proved effective.
New agents such as Mycophenolate Mofetil and the so-call biological agents such as Infliximab and Anakinra may be useful but are very expensive and have not been tested in clinical trials. Indeed, there have been no clinical trials of any drugs in RP as the condition is so rare that it is difficult to enrol enough patients into research trial.
There is a team of interested physicians at St Thomas’ Hospital dedicated to assessing and treating patients with RP. Patients are initially seen in the vasculitis clinic and referrals made as appropriate to ENT, respiratory and cardiothoracic physicians and surgeons. As a large teaching hospital, St Thomas’ is fully equipped to deal with the potentially life-threatening complications of this disorder.
NHS bureaucracy is extensive but RP may fall into the category of diseases rare enough to warrant the Department of Health giving St Thomas’ Hospital recognition as a specialist centre for RP. This application is being put together but is very time-consuming.
RP remains a rare disorder but new treatments and a dedicated team of physicians may go some way to helping improve the quality of life for patients with this autoimmune disorder.’
Rheumatologist Consulstant,
D'Cruz, David MD FRCP
Systemic Vasculitis Clinic,
Louise Coote Lupus Unit,
St. Thomas Hospital, Lambeth Palace Road, London, SE1 7EH,
Tel +44 207 188 3569, Fax +44 207 620 2658
Reprint courtesy of D’Cruz all rights reserved.
J Neuroophthalmol. 2007 Mar;27(1):16-21
Kellogg Eye Center, Department of Ophthalmology and Visual Sciences, University of Michigan, Ann Arbor, Michigan 48109, USA.
Optic neuropathy is an uncommon manifestation of relapsing polychondritis (RPC), a rare systemic disease affecting cartilaginous and proteoglycan-rich structures. The optic neuropathy has been attributed to ischemia, intrinsic inflammation of the optic nerve, or spread of inflammation to the nerve from adjacent intraconal orbital tissues. We report a case of recurrent corticosteroid-responsive optic neuropathy in which MRI did not show ocular, optic nerve, or intraconal orbital abnormalities but did show periosteal thickening and enhancement in the apical orbit and adjacent intracranial space consistent with periostitis. The periostitis, which is a manifestation of a systemic vasculitis or an autoimmune reaction to progenitors of cartilage, probably caused the optic neuropathy by compression or inflammation. It is important to recognize this mechanism of optic neuropathy as its imaging features may be a subtle yet critical clue to an underlying systemic condition that can be life-threatening if not properly managed.
Zhongguo Yi Xue Ke Xue Yuan Xue Bao. 2007 Apr;29(2):171-3.
[Article in Chinese]
Xu KJ, Liu YH, Jiang M.
Department of Dermatology, PUMC Hospital, CAMS and PUMC, Beijing 100730, China.
OBJECTIVE: To explore the diagnosis and treatment of relapsing polychondritis (RP). METHODS: The clinical manifestations, diagnosis, and treatment of 24 patients with RP were retrospectively analyzed. Twenty-four RP patients were examined for autoimmune antibody. Laryngoscopy was performed in 4 out of 6 RP patients with laryngeal involvement. Bronchoscopy was performed in 12 out of 19 RP patients with lower respiratory tract involvement. Biopsies were taken from the cartilage involved in 16 out of 24 patients. RESULTS: Five patients were ANA positive at present, 2 patients were SSA antibody positive, 2 patients were anti-RNP antibody positive, and 2 patients were anti-Sm antibody positive. Laryngoscopy revealed vocal fold edema and laryngeal stenosis. Bronchoscopy showed stenosis of trachea and both main bronchi and destruction of tracheobronchial cartilage. Histopathology revealed chronic inflammation of cartilage. Treatment included immunosuppressants (cyclophosphamide, methotrexate) together with prednisone. Eleven patients with respiratory tract involvement had laryngotracheostomy or airway stenting. Twenty-three patients alleviated after treatment, and 1 patients died. CONCLUSIONS: RP involves cartilage and connective tissue. Laryngotracheobronchial complications are the most severe manifestations of this disease. Corticosteroids and immunosuppressive drugs are effective treatment options. Patients with laryngotracheal stenosis and collapsed tracheobronchial wall should receive laryngostomy, tracheostomy, or airway stenting to improve airway obstruction symptoms.
Medicina (B Aires). 2007;67(2):156-8. [Article in Spanish]
Requena MA, Palma DA, Popescu BM.
Hospital Escuela José Francisco de San Martin de la Facultad de Medicina de la UNNE, Corrientes.
Relapsing polychondritis is a multysistemic inflammatory disease, of unknown etiology. It is characterized by recurrent episodes of inflammation with progressive destruction of cartilaginous structures and connective tissue. It may also affect tissues that contain proteoglycan like eyes, heart, kidney, and blood vessels causing a picture of vasculitis. We report here the case of a patient with relapsing polychondritis and seizures. The magnetic resonance imaging of the brain showed multiple hyperintense signals in the T2 weighted images and fluid-attenuated inversion recovery (FLAIR), suggesting ischemic injuries, that improved with high doses of glucocorticoids. We remark the unusual presentation with neurological involvement in this uncommon disease that has improved with treatment.
Rinsho Shinkeigaku. 2007 Jun;47(6):353-5.
[Article in Japanese]
Ochi M, Kawajiri M, Igase M, Takada K, Kohara K, Miki T.
Department of Geriatric Medicine, Medicine and Bioscience, Graduate School of Medicine, Ehime University.
A 64-year-old man developed cognitive impairment. A neurological examination showed hypereactive tendon reflex. A cerebrospinal fluid (CSF) examination showed a mild increase in both the number of mononuclear cells (14 cells/mm3) and the protein level (45 mg/dl). An electroencephalogram showed diffuse slow wave activity. Brain MR images revealed multifocal lesions in the limbic area and white matter regions. He was suspected to have autoimmune encephalitis and his symptoms improved after treated with methylprednisolone (1 g/day for 3 days). Despite this treatment, his mental disorders later relapsed. His condition improved after undergoing repeated treatment. Thereafter, he developed bacterial meningoencephalitis which had been caused by Listeria monocytogenes during the period of the internal administration of steroids. Antibiotics successfully cured his Listeria meningoencephalitis. He thereafter developed conjunctivitis, polyarthritis, hearing difficulties, dizziness and auricular chondritis. Based on these findings, we diagnosed the patient to have relapsing polychondritis (RP). It was thus conceivable that his cognitive impairment has been caused by CNS vasculitis associated with RP. RP therefore should be carefully taken into consideration and be included in the differential diagnosis as a possible cause for CNS vasculitis.
Zhonghua Jie He He Hu Xi Za Zhi. 2007 Mar;30(3):173-7.
[Article in Chinese]
Zhang JQ, Li Q, Bai C, Huang Y, Dong YC, Yao XP, Wang Q.
Department of Respiratory, Changhai Hospital, Second Military Medical University, Shanghai 200433, China.
OBJECTIVE: To describe the clinical features and treatment of relapsing polychondritis with involvement of the respiratory tract. METHOD: Thirteen cases (admitted from Aug 2000 to Oct 2006) of relapsing polychondritis with involvement of the respiratory tract treated in our hospital were retrospectively analyzed. RESULTS: There were 9 males and 4 females, with ages ranging from 30 to 61 years (mean 50 years). At early stage of the disease, clinical manifestations included cough, throat pain and hoarseness. Patients in later stage usually complained of chest distress, shortness of breath and dyspnea. Severe complications were repeated lower respiratory tract infections and/or respiratory failure. Bronchoscopic examination revealed an edematous larynx, narrowing of the glottis, tracheobronchial edema, turgescence of bronchial cartilage rings and airway stenosis at early stage. At later stage of the disease, malacia of trachea and bronchi due to disappearance of bronchial cartilage rings, and partly obliteration of the both trachea and main bronchus on expiration were demonstrated. Thoracic CT scan, with three-dimensional reconstruction of the airways, demonstrated a diffusely thickened tracheobronchial wall with tracheobronchial stenosis in earlier period of the disease and showed severe narrowing of both trachea and main bronchi in later period. Lung function measurements showed a moderate obstructive ventilatory disorder in 5 patients. Medical treatment with corticosteroids and immunosuppressive drugs was given in 12 patients. Symptoms were improved in 6 patients in earlier period of the disease, however, 6 patients in later period were not relieved. Twelve self-expanding metallic stents were placed in the airways (trachea and/or main bronchi), and obstruction of the respiratory tract was relieved in 5 patients, but there was no improvement in 1 patient who later was treated with positive airway pressure support. The 13 patients were followed for 1 to 48 months, and 12 patients survived, but one patient died 2 years after diagnosis. CONCLUSIONS: Corticosteroid therapy is effective in improving the symptoms and delaying the progression of relapsing polychondritis with involvement of the respiratory tract at early stage. At later stage of the disease, airway interventional therapy, such as metallic stent placement, tracheostomy or positive airway pressure support, can be used to treat airway obstruction and to improve the survival.
It can be one of your most frustrating experiences. For years your family and friends have patiently and caringly comforted you as you have experienced "phantom" pains, unexplained illnesses, and the overall disgust that comes with not being able to do what you used to do. And finally, after months and years of searching for an answer, trying to prove to yourself and to others that this isn't in your head, that you don't want to feel this way, you have a DIAGNOSIS! Finally, you can put a name to what ails you, an identifier, something to say to "what's wrong with you?", you can say
I HAVE RELAPSING POLYCHONDRITIS
You are not alone!
Relapsing Polychondritis On-line
Support Group - A mutual support resource for those with RPC. Our goal is to support those with RP by sharing our accurate information and experiences of this rare disease. Sharing with others, our own personal experiences and discussing different medical treatments and medical problems associated with this disease. (global membership)
Our aim is to locate RP suffers, offer support and an exchange of information. Founded in 1987 by Clifford Ashworth (global membership with UK focus)
Deutschsprachige Selbshilfegruppe für Betroffene und Angehörige der seltenen Krankheit rezidivierende Polychondritis (RP). Alle von der Krankheit betroffene sowie Angehörige sind herzlich willkommen. Wir wollen uns gegenseitig helfen, trösten, austauschen, Infos sammeln, Umfragen erstellen und vieles mehr.
(German support group for those with the rare disease named Relapsing Polychondritis)
Community Based
Keep-in-Touch (KIT) was developed to link persons with Relapsing Polychondritis (RPC) together with others in their community or region when possible. To establish community or regional support groups that help support those with RPC and to bring awareness of RPC to you and your community. To help you understand the disease at various stages and in various people, several support groups are available. One of the most important functions of a support group in Relapsing Polychondritis is to be able to talk with others who have this disease. As rare as this disease is, it is not uncommon to find that no one you know has ever heard of it.
All support groups can be verified through the Polychondritis Educational Society, Ltd. At www.Polychondritis.com
Clifford and Nellie Ashworth
2007 marks the 20th anniversary of the Relapsing Polychondritis Support Group.
We could not let the occasion go by without paying tribute to Clifford and Nellie Ashworth and remembering it was their hard work and determination that brought us to where we are today.
Clifford and Nellie would be proud.
This page was last updated: March 28, 2008
Polychondritis News, formerly the Support Report is the official publication of the
Polychondritis Educational Society, ltd. Copyright 2007- 2008. All rights reserved.
