Relapsing Polychondritis: Comprehensive Analysis of a Rare Disease

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This essay provides a comprehensive overview of relapsing polychondritis, a rare autoimmune rheumatic disorder characterized by recurrent episodes of inflammation affecting cartilage throughout the body, particularly in the ears, nose, and airways. The essay explores the unknown causes and risk factors associated with the disease, emphasizing the role of the immune system. It details common symptoms such as fatigue, dizziness, and inflammation in affected areas, along with diagnostic methods like biopsies and blood tests. The essay also discusses the disease's impact on life expectancy and examines treatment strategies, including corticosteroids and other medications aimed at managing symptoms and protecting affected cartilage. The content is supported by several research papers, offering an evidence-based approach to understanding and managing this complex condition. The essay highlights the importance of early detection and treatment to prevent severe complications and improve patient outcomes.
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Running head: RELAPSING POLYCHONDRITIS
RELAPSING POLYCHONDRITIS
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1RELAPSING POLYCHONDRITIS
Introduction:
Relapsing polychondritis is demarcated as a rare autoimmune rheumatic disorder which
is characterized by the recurrent episodes of destructive and painful inflammation of the
connective tissue and cartilage in different organs. Conferring to the study piloted by Longo et
al., (2016), this autoimmune disorder primarily affects the men and women of middle age. The
primary cause of the disorder is unknown, however as immunity system of the body is supposed
to be responsible for the prevalence of the disease. This essay primarily focusses on the
rheumatic disorder of polychondritis, its cause, risk factor and symptoms. Apart from this the
discussion also includes diagnosis, treatment and prevention of the disorder to avoid the adverse
impact of the illness.
Cause;
Relapsing polychondritis is a defined as a rare disorder in which the cartilage of different
organ gets inflamed. It is a rare autoimmune rheumatic disorder where nose, airway to the lungs
and the ear gets mostly affected. Rendering to the study performed by Vitale et al., (2016), the
primary cause of the disorder is not known clearly, however immune system of the body is
supposedly to be responsible for the disorder. According to Lekpa and Chevalier (2018), it is an
autoimmune disorder where the immune system of the body attacks the body itself rather than
attacking the foreign agents such as virus. According to some researcher, there is a possibility
that there might be a triggering event such as infection or illness or injury which initiates a set of
reaction by immunity system of the body which tends to attack the cartilage of the body.
It is primarily observed in the case of individual both men and women of middle age
(individual who are in their 50s and 60s). The disease is also considered to be transferred from
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2RELAPSING POLYCHONDRITIS
one generation to another. Genetic makeup of some individual makes them more prone to the
disease than other individuals. The individual suffering from illness such as rheumatoid arthritis,
systemic lupus and vasculitis are supposed to be more vulnerable for getting affected with the
relapsing polychondritis.
Symptoms:
Relapsing polychondritis is observed to possess negative impact on the whole body
similarly in case of systemic illness. The common symptoms which are generally observed in
case of individual suffering from the autoimmune disorder includes, malaise or fatigue,
dizziness, arthritis, cough, shortness of breathing and stridor. As stated above that the disorder
mainly attacks the cartilage of ear, nose and airways of lungs, that part of the gets swollen up.
The ear appears red and becomes painful and can even lead to hear loss in the Patient. The ear
when touched appears floppy, which means they become softer as compared to normal
individual and becomes limp or droopy (Kingdon et al., 2017). The nasal part of the individual
also gets inflamed which can lead to nasal congestion. In some patient more chronic symptoms
are observed such as, leaky heart valves, neurological complications, eye inflammation and
kidney complications.
Diagnosis:
By analyzing the above listed symptoms, it can be stated that if the disorder is not treated
immediately it can prove fatal. Hence, to avoid such adverse impact early diagnosis of the
disorder is vital. Rendering to the study conducted by Zampeli and Moutsopoulos (2018), it is
observed that in most of the cases, the diagnosis of illness get delayed as the early signs and
symptoms of the disease are similar to that of the other disease. To confirm the occurrence of the
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3RELAPSING POLYCHONDRITIS
disease in the Patient, biopsy of the inflamed area is performed. During the biopsy, a part of the
tissue is tissue is removed and is then examined under the microscope. After examination it helps
in the evaluation of the type and severity of the infection. Apart from these, sometimes the
disorder can also be confirmed by the critical analysis of the symptoms such as, inflammation of
the ears, painful swelling in the joints, inflammation of the nose and eye, cartilage of the
respiratory tract is damaged and balance or hearing complications is observed. If the patient is
observed to be suffering from any three of the above listed symptoms, the disorder is confirmed.
In addition to that blood test is also used to evaluate the occurrence of the disease where the
erthrocyte sedimentation rate helps in the detection of the severity of the inflammation (Mathian
et al., 2016).
Prognosis:
According to the research piloted by Hazra et al., (2015), individual suffering from
polychondritis have lower life expectancy rate as compared to the normal healthy individual
because occurrence of the disease tends to increase the risk of heart, lungs and the blood vessel
damage. Therefore, it is also contributes to increase the death rate of individual suffering from
immune mediated disorder. However in recent years several advancement has made which has
contributed to decrease the death rate and aids in increasing the health outcomes of the patient
suffering relapsing polychondritis.
Treatment:
The primary aim of treatment in the Patient suffering from relapsing polychondritis is to
deliver relive to the patient from the chronic symptoms which is to protect the structures of thr
cartilages affected from illness. In order to do that, the Patient is administered with corticosteroid
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along with predisnone which tends to decrease the severity of the infection (Moore et al., 2016).
Apart from corticosteroid other medications such as, colchicine, dapson, infliximab are also there
which tends to reduce the severity of the infection.
Conclusion:
From the above discussion it is observed that relapsing polychondritis is a rare
autoimmune rheumatic disorder with unknown cause. It mainly affects the cartilage portion of
the body especially ears, nose and airways lining of the lungs. The person suffering from the
disease have low life expectancy rate as compared to other individuals therefore early detection
is must to avoid the future complications. Early detection can be performed by the aid of biopsy,
physical examination and blood test. From the above discussion it can be concluded that to avoid
the future complications the patient must be treated by the aid of corticosteroid and other
medications.
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Reference:
Hazra, N., Dregan, A., Charlton, J., Gulliford, M. C., & D’Cruz, D. P. (2015). Incidence and
mortality of relapsing polychondritis in the UK: a population-based cohort
study. Rheumatology, 54(12), 2181-2187.
Kingdon, J., Roscamp, J., Sangle, S., & D’Cruz, D. (2017). Relapsing polychondritis: a clinical
review for rheumatologists. Rheumatology, 57(9), 1525-1532.
Lekpa, F. K., & Chevalier, X. (2018). Refractory relapsing polychondritis: challenges and
solutions. Open access rheumatology: research and reviews, 10, 1.
Longo, L., Greco, A., Rea, A., Vasco, V. R. L., De Virgilio, A., & De Vincentiis, M. (2016).
Relapsing polychondritis: a clinical update. Autoimmunity Reviews, 15(6), 539-543.
Mathian, A., Miyara, M., Cohen-Aubart, F., Haroche, J., Hie, M., Pha, M., ... & Amoura, Z.
(2016). Relapsing polychondritis: a 2016 update on clinical features, diagnostic tools,
treatment and biological drug use. Best practice & research Clinical
rheumatology, 30(2), 316-333.
Moore, G. H., Rootman, D. B., Roybal, C. N., & Goldberg, R. A. (2016). Orbital relapsing
polychondritis: a unique presentation, complication, and treatment. Ophthalmic plastic
and reconstructive surgery, 32(2), e34-e36.
Vitale, A., Sota, J., Rigante, D., Lopalco, G., Molinaro, F., Messina, M., ... & Cantarini, L.
(2016). Relapsing polychondritis: an update on pathogenesis, clinical features, diagnostic
tools, and therapeutic perspectives. Current rheumatology reports, 18(1), 3.
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Zampeli, E., & Moutsopoulos, H. M. (2018). Relapsing polychondritis: a diagnosis not to be
missed. Rheumatology, 57(10), 1768-1768.
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