Several abstracts regarding RP

[Guest guest]

One of the Ladys in my SBI (silicone breast implant) support groups

was kind enough to send the following abstracts regarding RP. It's

rather long, but at least shows there are doctors out there who are

aware of us. One study was done in Gainesville where I work.

Neurologic manifestations of connective tissue disease [in Process

Citation]

Neurol Clin 2002 Feb;20(1):151-78 (ISSN:

0733-8619)

Nadeau SE

Geriatric Research, Education and Clinical Center, Department of

Veterans Affairs Medical Center, and Department

of Neurology, University of Florida, College of

Medicine, Gainesville, Florida. The clinical features, diagnosis,

and treatment of systemic lupus erythematosus, scleroderma,

rheumatoid arthritis, Sjogren's syndrome, mixed connective tissue

disease, Behcet's disease, Cogan's syndrome, and relapsing

polychondritis are reviewed from a neurological perspective with an

emphasis on pathogenic mechanisms and their relationship to

treatment.

Relapsing polychondritis affecting the lower respiratory tract.

AJR Am J Roentgenol 2002 Jan;178(1):173-7 (ISSN:

0361-803X)

Behar JV; Choi YW; Hartman TA; NB;

Mc HP

Department of Radiology, Duke University Medical Center, Box

3808, Durham, NC 27710, USA.

OBJECTIVE: The purpose of this study was to describe the CT

findings of lower respiratory tract involvement by relapsing

polychondritis. CONCLUSION: The most common CT manifestations were

increased attenuation and smooth thickening of airway walls.

Tracheal or bronchial stenosis was less common. Airway collapse and

lobar air trapping were seen in half of patients examined with

expiratory CT.

Pulmonary fibrosis with intractable pneumothorax: new pulmonary

manifestation of relapsing polychondritis [in Process Citation]

Tohoku J Exp Med 2001 Jul;194(3):191-5 (ISSN:

0040-8727)

Wu S; Sagawa M; Suzuki S; Kumagai-Braesch M; Honda Y;

Sato M; Kondo T

Department of Thoracic Surgery, Institute of Development, Aging

and Cancer, Tohoku University, Sendai, Japan. Relapsing

Polychondritis is a rare disease which causes the repetitive

inflammation of cartilage and connective tissues. Although the

large airway is sometimes involved and the stenosis of them often

influences the prognosis of the patients, there have been few

reports concerning the manifestation of the peripheral lung. A 60-

year-old man with pulmonary fibrosis was admitted to a regional

hospital due to sudden deafness, and then he suffered from

relapsing polychondritis. During the steroid therapy, he also

suffered from bilateral pneumothoraces. His computed tomogram

revealed many bilateral bullae, emphysematous changes, and fibrotic

changes in bilateral lungs. The mechanism of generating peripheral

pulmonary manifestations is also discussed.

Relapsing polychondritis: a report of eight cases [in Process

Citation]

Auris Nasus Larynx 2001 May;28 Suppl:S107-10

(ISSN:

0385-8146)

Nakamaru Y; Fukuda S; Maguchi S; Ryu T;

Inuyama Y

Department of Otolaryngology, Hokkaido University School

of Medicine, Sapporo, Japan.

nmaru@....

OBJECTIVE: To investigate clinical manifestations of relapsing

polychondritis and to clarify the significance of type II collagen

antibody in the disease. METHODS: Clinical manifestations and

antibody titers were examined in eight cases of relapsing

polychondritis which had been treated at Otolaryngology Department,

Hokkaido University during the eight years from 1991 to 1998. Anti-

type II collagen antibody titer was measured by ELISA method.

RESULTS: The most frequent symptom was auricular chondritis; it was

seen in 88% (7/8) of the cases. Ocular symptom, nasal chondritis,

arthritis, respiratory tract chondritis, and audio-vestibular

symptom were also common in the cases. Compared with previous

reports, no difference was recognized in the manifestation

frequency. Considering none of the samples from the controls was

positive for anti-type II collagen antibody, two samples from the

disease group were positive. The antibody positive rate was 25%

(2/8). CONCLUSION: Though the measurement of type II collagen

antibody titer is not a decisive factor for detection of relapsing

polychondritis, it is useful as one of the complementary factors for

the diagnosis, since there is no specific test for this disease.

The Association of Relapsing Polychondritis and Myelodysplastic

Syndrome [Record Supplied By Aries Systems]

J Clin Rheumatol 2000 Jun;6(3):146-149 (ISSN:

1076-1608)

Salahuddin Naveed; Libman Bonita S; Lunde H; Kay

; Sheldon M

Rheumatology and Clinical Immunology Unit, Department of

Medicine, The University of Vermont College of

Medicine, Burlington, Vermont.

Myelodysplastic syndrome is a group of clonal stem cell disorders

characterized by cytopenias, including anemia. Rheumatologic

manifestations have been reported in patients with myelodysplastic

syndrome, including the rare disorder of relapsing polychondritis,

which seems to occur more frequently than would be expected by

chance. We describe three older males with relapsing

polychondritis, who developed myelodysplastic syndrome, and we

review the clinical features of the previously reported cases. Most

of these patients are epidemiologically and prognostically similar

to those with myelodysplastic syndrome alone, leading to speculation

that relapsing polychondritis may be a paraneoplastic manifestation

of myelodysplastic syndrome. These cases illustrate the need for

greater awareness by physicians of the association between these two

entities, and a thorough evaluation of hematologic abnormalities in

patients, especially older patients, with relapsing polychondritis.

[Copyright

Information: Copyright ©2000 by Lippincott & Wilkins]

Language: English

Serum level of macrophage migration inhibitory factor as a useful

parameter of clinical course in patients with Wegener's

granulomatosis and relapsing polychondritis.

Ann Otol Rhinol Laryngol 2001 Nov;110(11):1035-40

(ISSN: 0003-4894)

Ohwatari R; Fukuda S; Iwabuchi K; Inuyama Y; Onoe

K; Nishihira J

Department of Otolaryngology-Head and Neck Surgery Graduate

School of Medicine, Hokkaido University,

Sapporo, Japan.

Novel biological activities of macrophage migration inhibitory

factor

(MIF) have been rediscovered. In addition,

elevation of the serum MIF level has been reported in different

types of disorders, including various inflammatory and autoimmune

diseases. In the present study, serum MIF levels were analyzed in

patients with Wegener's granulomatosis (WG) and relapsing

polychondritis. It was shown that the serum MIF levels in these

patients were significantly higher than those of normal healthy

controls. In a WG patient, the MIF level showed a good correlation

with clinical symptoms and C-ANCA titers. Thus, serum MIF levels

will be a useful laboratory parameter for following the clinical

course of WG patients and determining medical treatment. The

immunopathologic roles of MIF in these diseases are discussed.

Active aortitis in relapsing polychondritis.

J Clin Pathol 2001 Nov;54(11):890-2 (ISSN:

0021-9746)

Selim AG; Fulford LG; Mohiaddin RH; Sheppard

MN

Department of Histopathology, Royal Brompton Hospital, Imperial

College, School of Medicine, Sydney Street,

London SW3 6NP, UK. Relapsing

polychondritis (RP) is a rare inflammatory multiorgan disorder

affecting cartilaginous structures and other connective tissues.

Serious cardiovascular complications have been reported in patients

with RP, the most frequent being aortic or mitral regurgitation and

aortic aneurysms. Aortitis is a very rare complication. An unusual

case of active aortitis in a patient with RP, despite intensive

immunosuppressive treatment, is described with a special emphasis

on the pathological findings.

The occurrence of autoantibodies to matrilin 1 reflects a tissue-

specific response to cartilage of the respiratory tract in patients

with relapsing polychondritis.

Arthritis Rheum 2001 Oct;44(10):2402-12 (ISSN:

0004-3591)

Hansson AS; Heinegard D; Piette JC; Burkhardt H;

Holmdahl R

Medical Inflammation Research, Lund University, Sweden.

Ann-Sofie.Hansson@....

OBJECTIVE: Relapsing polychondritis (RP) is an inflammatory disease

that mainly affects cartilage tissue in the auricle, nose, and

lower respiratory tract. When tracheolaryngeal cartilage is

involved, the disease is occasionally fatal. Matrilin 1 is a

cartilage-specific protein most prominently expressed in tracheal

cartilage, but not in joint cartilage. Immunization with the

protein in rats and mice induces respiratory distress and nasal

destruction, as seen in RP. We investigated the response to

matrilin 1 and other cartilage proteins in sera from patients with

RP, 4 additional groups of patients with other major connective

tissue diseases, and healthy control subjects.

METHODS: Sera were analyzed by enzyme-linked immunosorbent assay

(ELISA) for antibody responses to

matrilin 1, types II, IX, and XI collagen, and cartilage oligomeric

matrix protein (COMP). Titers above the mean + 3SD of controls were

considered positive. Specificity of matrilin 1 recognition was

further investigated by the capacity of high-titer sera to block

the binding of a matrilin 1-specific monoclonal antibody in

inhibition ELISAs. In vivo reactivity and specificity were tested

by injecting sera into neonatal mice, and antibody binding was

detected by immunohistochemical staining. RESULTS: Serum antibodies

from RP patients bound tracheolaryngeal and nasal cartilage in vivo

and inhibited the binding of anti-matrilin 1-specific monoclonal

antibodies. Thirteen of the 97 RP patients had increased titers of

matrilin 1 antibody. Positive titers correlated with respiratory

symptoms in 69% of the cases. Significant responses to type II

collagen and COMP were also detected. CONCLUSION: Antibodies to

matrilin 1 bind tracheolaryngeal cartilage in vivo and are

correlated with an inflammatory attack on tracheolaryngeal

cartilage that is often seen in RP.

Leflunomide induced fevers, thrombocytosis, and leukocytosis in a

patient with relapsing polychondritis

J Rheumatol 2002 Jan;29(1):192-4 (ISSN:

0315-162X) Koenig AS; Abruzzo JL

Department of Medicine, Jefferson University,

Philadephia, Pennsylvania 19107, USA. The most common adverse

events reported with the use of leflunomide are hypertension,

infections, alopecia, and various gastrointestinal complaints. No

fatal adverse hematologic events have been reported in humans,

although anemia and leukopenia have been described in animals

receiving 20 mg/kg/day. We describe a patient with relapsing

polychondritis, in whom treatment failure with glucocorticoids,

methotrexate, hydroxychloroquine, and azathioprine led to the

institution of therapy with leflunomide at a maintenance dose of 20

mg/day. Two months after the dose of leflunomide had been increased

to 30 mg daily, the patient developed high fevers, photophobia,

thrombocytosis, and leukocytosis that returned to normal following

treatment with cholestyramine and discontinuation of leflunomide.

Rechallenge with leflunomide was not attempted and the syndrome did

not recur during 14 month followup.

Relapsing polychondritis affecting the lower respiratory tract.

AJR Am J Roentgenol 2002 Jan;178(1):173-7 (ISSN:

0361-803X)

Behar JV; Choi YW; Hartman TA; NB;

Mc HP

Department of Radiology, Duke University Medical Center, Box

3808, Durham, NC 27710, USA.

OBJECTIVE: The purpose of this study was to describe the CT

findings of lower respiratory tract involvement by relapsing

polychondritis. CONCLUSION: The most common CT manifestations were

increased attenuation and smooth thickening of airway walls.

Tracheal or bronchial stenosis was less common. Airway collapse and

lobar air trapping were seen in half of patients examined with

expiratory CT.

Identification of type II collagen peptide 261-273-specific T cell

clones in a patient with relapsing polychondritis.

Arthritis Rheum 2002 Jan;46(1):238-44 (ISSN:

0004-3591)

Buckner JH; Van LM; Kwok WW; Tsarknaridis L

Virginia Mason Research Center, Seattle, WA 98101, USA.

Jbuckner@....

OBJECTIVE: To characterize and clone T cells specific for type II

collagen (CII) in a patient with relapsing polychondritis (RP) and

to establish whether the immunodominant epitope of CII determined in

HLA transgenic mice is used in the human autoimmune response to

CII. METHODS: T cell responses to CII were examined in a patient

with RP, who was heterozygous for the HLA-DR allele

DRB1*0101/DRB1*0401. T cell clones were established from this

patient and characterized for peptide specificity, class II

restriction, cytokine production, and staining with HLA-DRB1*0401

class II tetramers. RESULTS: A response to CII and the peptide 255-

273 was present in this patient. T cells specific for the CII

epitope 261-273 were cloned. Evaluation of these clones

demonstrated a response to CII 261-273 in the context of both DR

alleles. HLA-DR4 CII tetramer did not demonstrate staining of

either CII-specific DRB1*0401-restricted T cell clones or a

polyclonal population of CII-reactive T cells from this individual.

CONCLUSION: T cells directed against CII were present in this

patient with RP. Also, T cell clones isolated from this individual

were found to be specific for the CII peptide 261-273 and were

restricted to either the DRB1*0101 or the DRB1*0401 allele. These

findings establish that a T cell response directed against CII is

present in this patient with RP and that the CII peptide 261-273

plays a role in the human immune response to CII.

Colchicine and indomethacin for the treatment of relapsing

polychondritis.

J Am Acad Dermatol 2002 Feb;46(2 Suppl Case Reports):S22-4

(ISSN: 0190-9622) Mark KA; s AG

Connective Tissue Section of the C. Skin and

Cancer Pavilion, The O. Perelman

Department of Dermatology, New York University School of Medicine,

New York, NY, USA.

This report describes the effectiveness of oral colchicine and

indomethacin in the maintenance phase of treatment of relapsing

polychondritis. Systemic corticosteroids are commonly used in the

induction phase of managing cases of relapsing polychondritis.

Clinicians are then faced with the challenge of controlling the

patient's symptoms while trying to decrease the dose of steroid.