Relapsing polychondritis: clinical presentations, disease activity and outcomes

Sharma, Aman; Law, Arjun; Bambery, P; Sagar, Vinay; Wanchu, Ajay; Dhir, Varun; Vijayvergiya, Rajesh; Sharma, Kusum; Gupta, Ashok; Panda, Naresh K.; Singh, Surjit

doi:10.1186/s13023-014-0198-1

Cited by 33 publications

(22 citation statements)

References 30 publications

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“…The image of tracheobronchial amyloidosis on CT appears as circumferential wall thickening with calcifications as well as luminal narrowing at various levels of the tracheobronchial tree, as was seen in our patient(4). However, the diseases related with tracheobronchial involvement such as tracheobronchial tuberculosis or polychondritis should be considered for differantial diagnosis(9)(10)(11).…”

supporting

confidence: 60%

A Case of Tracheal Amyloidosis Admitted with Rectus Abdominis Muscle Hematoma

Baris¹,

Turhan²,

Yildiz³

et al. 2015

West Indian Med J

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Tracheobronchial amyloidosis is often misdiagnosed as chronic airway diseases in relation to the symptoms of chronic cough and dyspnea. Here, we presented a case admitted to emergency department with severe cough and stomachache and diagnosed with rectus abdominis muscle hematoma due to chronic cough. The case had a history of asthma and no clinical improvement despite the use of inhaled drugs nearly five years. He was diagnosed with tracheobronhial amyloidosis after bronchoscopic and histopathologic evaluation. This case presented to remind the tracheal pathologies in differential diagnosis of chronic cough and emphasize the importance of bronchoscopic evaluation.

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supporting

confidence: 60%

A Case of Tracheal Amyloidosis Admitted with Rectus Abdominis Muscle Hematoma

Baris¹,

Turhan²,

Yildiz³

et al. 2015

West Indian Med J

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“…In these cases, methotrexate, azathioprine, cyclosporine, and chlorambucil may be used. 24 , 25 Intravenous cyclophosphamide and plasmapheresis are used in life-threatening situations, or in organ-threatening disease including acute airway obstruction or glomerulonephritis. 26 A number of biologics targeting the B cells and the pathways of cell-mediated immunity (anti-CD4, anti-CD20, anti-TNF agents, IL-1 receptor antagonist, CTLA4-IgG1 fusion protein, or anti-IL-6 receptor antibody) are applied if RP is not responding to other medical therapy.…”

Section: Introductionmentioning

confidence: 99%

A nationwide study of the epidemiology of relapsing polychondritis

Horváth¹,

Páll²,

Molnár³

et al. 2016

CLEP

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ObjectiveRelapsing polychondritis (RP) is a rare autoimmune inflammatory disease that attacks mainly cartilaginous structures or causes serious damage in proteoglycan-rich structures (the eyes, heart, blood vessels, inner ear). This study shows results regarding the epidemiology, progression, and associations of this highly variable disease by collecting all cases from a 124-million-person-year Central European nationwide cohort.MethodsWe used the Hungarian Health Care Database to identify all persons with possible RP infection. We followed patients who had International Classification of Diseases 10th edition code M94.1 at least once in their inpatient or outpatient records between January 1, 2002 and December 31, 2013 in Hungary. We classified these patients into disease severity groups by their drug consumption patterns between January 1, 2010 and December 31, 2013. We analyzed the regional distribution of RP incidences as well. Overall maps of comorbidity are presented with network layouts.ResultsWe identified 256 patients with RP among cumulatively 11.5 million registered inhabitants. We classified these patients into four severity classes as “extremely mild” (n=144), “mild” (n=22), “moderate” (n=41), and “severe” (n=4). Two additional groups were defined for patients without available drug data as “suspected only” (n=23) and “confirmed but unknown treatment” (n=22). The age and sex distributions of patients were similar to worldwide statistics. Indeed, the overall survival was good (95% confidence interval for 5 years was 83.6%–92.9% and for 10 years was 75.0%–88.3% which corresponds to the overall survival of the general population in Hungary), and the associations with other autoimmune disorders were high (56%) in Hungary. Almost any disease can occur with RP; however, the symptoms of chromosomal abnormalities are only incidental. Spondylosis can be a sign of the activation of RP, while Sjögren syndrome is the most frequent autoimmune association. Regional distribution of incidences suggests arsenic drinking water and sunlight exposure as possible triggering factors.ConclusionThe good survival rate of RP in Hungary is probably associated with the early diagnosis of the disease.

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“…RP is defined by inflammation of cartilaginous structures and the disease course and clinical manifestations are highly variable. End organ complications due to inflammation of cartilage and proteoglycan rich structures are diverse and include deformity of the ears(2), nose(3) and chest(4), subglottic stenosis (5), obstructive lung disease, tracheomalacia(6), bronchomalacia(7), recurrent pneumonia(8), mitral and aortic regurgitation(9, 10), large and medium vessel aneurysms(11), blindness(12), deafness(13), renal insufficiency(14), chronic pain, and sudden death(15–17). The protean aspects of RP pose diagnostic challenges.…”

mentioning

confidence: 99%