Relapsing polychondritis is an uncommon disease which may effect a number systems. It may be life threatening, debilitating, and difficult to diagnose.
It is characterized by recurring, inflammation of cartilage. These inflammations may be potentially severe, and many times are frightening. All types of cartilage may be involved, including but not limited to; the cartilage in the ears, nose, joints, ribs, and throat.
Relapsing polychondritis can also affect other areas such as eye, heart, blood vessels, and inner ear. Vasculitis affecting skin or internal organs may occur.
Relapsing olychondritis may start with a wide array of painful symptoms that often pose major diagnostic dilemmas. For SYMPTOMS as noted by the RP group click here.
No specific tests for it are available, therefore relapsing polychondritis must be diagnosed by observing and evaluating the various symptoms.
Most commonly, relapsing polychondritis symptoms seem to come and go. The more active periods are refered to as Flare-ups. In some cases the RP seems to always be active to some degree. The continued flare-ups eventually lead to the permanent destruction of the involved area.
For some people,, the disease is more limited, this unfortunately is the exceptions.
Reference: Annals of Internal Medicine 1998; 129:114, David E. Trentham, MD, Division of Rheumatology, Beth Israel Deaconess Medical Center, 330 Brookline Avenue, Boston, MA 02215
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TITLE: Respiratory involvement in relapsing polychondritis.
Clinical, functional, endoscopic, and radiographic evaluations.
AUTHORS: Tillie-Leblond I; Wallaert B; Leblond D; Salez F; Perez T; Remy-Jardin M; Vanhille P; Brouillard M; Marquette C; Tonnel AB
AUTHOR AFFILIATION: Clinique des Maladies Respiratoires, Hopital Calmette, CHRU, Lille, France.
SOURCE: Medicine (Baltimore). 1998 May;77(3):168-76.
ABSTRACT: Although respiratory involvement occurs in 50% of patients with relapsing polychondritis (RP) and augurs a poor prognosis, few previous studies have provided complete descriptions of respiratory tract involvement. For this reason, we investigated the respective role of clinical, functional, endoscopic, and radiographic (computed tomography [CT]) examinations in 9 consecutive patients with RP and lower respiratory tract localization. All exhibited cough, dyspnea, and wheezing. Eight had a nonreversible obstructive pattern with a marked decrease of the maximal flow ratio at 75% and 25% of vital capacity. Rotman functional criteria were evaluated to differentiate upper from lower respiratory tract involvement;they were consistent with the results of other examinations in 4/9 cases. Endoscopic examination showed moderate to severe inflammation in 8/9 patients; tracheal stenosis was present in 6/9 patients, bronchial stenosis in 4/9 patients, and tracheal collapse in 7 cases. CT showed tracheal stenosis in 8/9 patients (diffuse, 7; localized, 1) and bronchial stenosis in 6/9 patients. Tracheobronchial wall thickening and/or calcifications were observed in 7 cases. Clinical symptoms are of poor specificity for defining respiratory involvement precisely, although degree of dyspnea is correlated to the decrease in forced expiratory volume in 1 second (FEV1). Functional criteria were helpful in evaluating the obstructive ventilatory defect but did not differentiate, in most cases, the respective part of lower and upper respiratory involvement when using Rotman criteria. Compared to CT findings, endoscopic examination failed to identify tracheal and bronchial stenosis and tracheal wall alterations at an early stage of the disease. In our series CT appears to be a reliable method to identify tracheal and bronchial involvement and can be repeated safely during the course of the disease.
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TITLE: Expandable metallic stents in benign tracheobronchial obstruction.
AUTHORS: Lehman JD; Gordon RL; Kerlan RK Jr; Laberge JM; Wilson MW; Golden JA; Webb WR
AUTHOR AFFILIATION: Department of Radiology, University of California San Francisco, 94143-0628, USA.
SOURCE: J Thorac Imaging. 1998 Apr;13(2):105-15.
SECONDARY SOURCE ID: MED/98215574
ABSTRACT: Expandable metallic stents offer advantages over previously available techniques for treating benign tracheobronchial stenosis or obstruction. Endoluminal stent placement offers a rapid and effective means of opening up narrowed airways, and results in excellent relief of symptoms and improvement in pulmonary function. Because they are delivered in a nonexpanded state using flexible over-wire systems, they can be placed using a flexible bronchoscope and can be located in second-order bronchial branches. Metallic stents have been used to treat benign airway obstruction caused by anastomotic narrowing after lung transplantation, infection, congenital lesions, tracheobronchial malacia, inflammatory conditions including relapsing polychondritis, Wegener granulomatosis, and acquired immunodeficiency syndrome, and external compression from benign mediastinal masses or fibrosis. The stents become epithelialized, which prevents migration and permits ciliary activity to continue. Significant complications can occur, including airway inflammation, stent migration, airway erosion, and stent fracture and collapse, but more serious complications are uncommon. Computed tomography is essential in imaging patients being considered for stent placement, as it allows 1) accurate representation of airway anatomy in three dimensions. 2) measurement of airway diameter, 3) evaluation of airway anatomy distal to a narrowed segment and invisible to bronchoscopy, 4) demonstration of dynamic changes in airway morphologic features during forced exhalation in patients with airway malacia, and 5) demonstration of focal or diffuse air trapping in lung peripheral to the abnormal airway. In patients who have had stent placement, computed tomography is valuable in assessing airway morphologic features and dynamics distal to the stent, and can be valuable in assessing stent dysfunction.
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Anergy is the inability to mount a delayed-type hypersensitivity (DTH) immune response to one or several skin-test antigens. Common causes of anergy include the following: infection with the human immunodeficiency virus (HIV), live viral vaccines, steroids or other drugs, alcoholism, stress, extremes of age, chronic medical conditions, and overwhelming tuberculosis (TB) disease.
People with any of these conditions may have a negative tuberculin skin test (TST) result even if they are infected with Mycobacterium tuberculosis. In HIV-infected patients, the likelihood of anergy is greater when CD4+ T-lymphocyte counts are lower.
Anergy testing is done with common DTH antigens, such as Candida,mumps, or tetanus toxoid. However, anergy testing is neither standardized nor reliable. In fact, in most studies of skin testing, a significant number of healthy subjects are found to be anergic to the DTH antigens.(1)
Anergy skin testing in HIV-infected patients is unreliable. (2,3) Often, patients are anergic to a standard anergy panel at initial testing but respond on repeat testing. Many people react to tuberculin but not to the test antigens, or vice versa; inability to mount a DTH response to one antigen does not necessarily predict anergy to other antigens. Finally, TST-negative patients thought to be anergic sometimes become TST-positive after exposure to TB.
1. Centers for Disease Control and Prevention. Purified protein derivative(PPD)-tuberculin anergy and HIV infection: guidelines for anergy testing and management of anergic persons at risk of tuberculosis. MMWR1991;40(RR-5):27-33.
2. Chin DP, Osmond D, Page-Shafer K, et al. Reliability of anergy skin testing in persons with HIV infection. Am J Respir Crit Care Med1996;153:1982-4.
3. Pesanti EL. The negative tuberculin test: tuberculin, HIV, and anergy panels. Am J Respir Crit Care Med 1994;149:1699-1709.
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