The Association of Relapsing Polychondritis and Myelodysplastic Syndrome: Report of Three Cases

Salahuddin, Naveed; Libman, Bonita; Lunde, John H.; Kay, Jonathan; Cooper, Sheldon M.

doi:10.1097/00124743-200006000-00006

Cited by 13 publications

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“…An increased risk of myelodysplastic syndrome (MDS) may exist in patients with RP [22,23]. In a single centre series of 200 patients from France, MDS was seen in 11% [24].…”

Section: Discussionmentioning

confidence: 99%

Relapsing polychondritis: clinical presentations, disease activity and outcomes

Sharma

Law

Bambery

et al. 2014

Orphanet J Rare Dis

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BackgroundRelapsing polychondritis is a rare disease characterised by inflammation of cartilaginous and proteoglycan rich structures. As there are only a few published single centre case series from all across the world, we describe our experience with 26 patients at a tertiary centre in north India.MethodsA retrospective study with all patients meeting Damiani and Levine’s modification of McAdam’s diagnostic criteria. Clinical details, investigations, disease activity assessment [(Relapsing Polychondritis Disease Activity Index (RPDAI)], treatment and outcomes were recorded.ResultsTen men and sixteen women (median age 45 years) met the diagnostic criteria. Auricular chondritis (96%), arthritis (54%), hearing impairment (42%), ocular (42%), dermal (26%), cardiovascular (11%) and laryngotracheal involvement (11%) characterized the clinical presentations. The median RPDAI was 31 (range 9-66). Two patients died during observation. Overall survival was 92.3% (median survival 13.5 years).ConclusionsApart from reduced laryngotracheal involvement, RP in India was clinically similar to recorded patterns elsewhere.

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“…An increased risk of myelodysplastic syndrome (MDS) may exist in patients with RP [22,23]. In a single centre series of 200 patients from France, MDS was seen in 11% [24].…”

Section: Discussionmentioning

confidence: 99%

Relapsing polychondritis: clinical presentations, disease activity and outcomes

Sharma

Law

Bambery

et al. 2014

Orphanet J Rare Dis

View full text Add to dashboard Cite

show abstract

“…This exceedingly rare manifestation has not been described previously in literature and showed a dramatic response to pulse methylprednisolone [21]. An increased risk of myelodysplastic syndrome (MDS) may exist in patients with RP [22,23]. In a single centre series of 200 patients from France, MDS was seen in 11% [24].…”

Section: Discussionmentioning

confidence: 93%

Relapsing polychondritis: Clinical presentations, disease activity and outcomes

Sharma

Law

Bambery

et al. 2014

Indian Journal of Rheumatology

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Background: Relapsing polychondritis is a rare disease characterised by inflammation of cartilaginous and proteoglycan rich structures. As there are only a few published single centre case series from all across the world, we describe our experience with 26 patients at a tertiary centre in north India. Methods: A retrospective study with all patients meeting Damiani and Levine's modification of McAdam's diagnostic criteria. Clinical details, investigations, disease activity assessment [(Relapsing Polychondritis Disease Activity Index (RPDAI)], treatment and outcomes were recorded. Results: Ten men and sixteen women (median age 45 years) met the diagnostic criteria. Auricular chondritis (96%), arthritis (54%), hearing impairment (42%), ocular (42%), dermal (26%), cardiovascular (11%) and laryngotracheal involvement (11%) characterized the clinical presentations. The median RPDAI was 31 (range 9-66). Two patients died during observation. Overall survival was 92.3% (median survival 13.5 years).

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“…The former is a rare autoimmune disease that primarily manifests as cartilaginous tissue destruction. This condition has been described across the different MDS subtypes with different severity, ranging from limited joint involvement to total hearing loss and blindness due to ear and eye localization . Rheumatoid arthritis has been less frequently described once again with a range of severity up to aggressive clinical evolution with atlantoaxial subluxation .…”

Section: Autoimmune Manifestations Involving Specific Sitesmentioning

confidence: 99%

The burden of autoimmunity in myelodysplastic syndromes

Fozza

2017

Hematological Oncology

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The clinical history of patients with myelodysplastic syndromes (MDS) is characterised by bone marrow insufficiency as well as by the possible evolution into acute leukaemia. However a number of reports highlight the frequent occurrence of autoimmune manifestations involving different sites and organs. The present review will first describe the clinical pictures most often observed in MDS patients. The actual burden of autoimmunity will be then addressed by focusing on the few available registry studies. Finally, the potential collateral impact of specific treatments for MDS on the evolution of autoimmune disorders will be considered.

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The Association of Relapsing Polychondritis and Myelodysplastic Syndrome: Report of Three Cases

Cited by 13 publications

References 0 publications

Relapsing polychondritis: clinical presentations, disease activity and outcomes

Relapsing polychondritis: clinical presentations, disease activity and outcomes

Relapsing polychondritis: Clinical presentations, disease activity and outcomes

The burden of autoimmunity in myelodysplastic syndromes

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