Airway Obstruction in an Adolescent with Relapsing Polychondritis

Prasad, Sanjay; Grundfast, Kenneth W.; Lipnick, Robert

doi:10.1177/019459989010300117

Cited by 7 publications

(3 citation statements)

References 5 publications

(2 reference statements)

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“…Mild disease has been managed with diaminodiphenylsulphone (dapsone), which inhibits lysosome release, 16 , aspirin, and other nonsteroidal anti‐inflammatory drugs. Success has also been reported with the use of immunosuppressive agents including azathiaprin, 2 cyclophosphamide, 6‐mercaptopurine, penicillamine, and cyclosporin 15 .…”

Section: Discussionmentioning

confidence: 99%

Management of Laryngotracheobronchial Sequelae and Complications of Relapsing Polychondritis

1997

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Relapsing polychondritis is a rare multisystem disorder of unknown etiology characterized by recurrent inflammation and degeneration of cartilage and connective tissue. Laryngotracheobronchial complications are the most severe manifestations of the disease and present the most challenging management decisions. We present four cases of relapsing polychondritis with laryngotracheobronchial manifestations that illustrate the clinical features and review the treatment options.

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Section: Discussionmentioning

confidence: 99%

Management of Laryngotracheobronchial Sequelae and Complications of Relapsing Polychondritis

1997

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“…Although onset may vary from birth to the ninth decade, only a few cases have been reported in childhood [2][3][4][9][10][11][12]. The aetiology is unknown, but mounting evidence suggests that this disorder is characterized by a selected perturbation of connective tissue structures with mucopolysaccharide depletion [4,5].…”

Section: Discussionmentioning

confidence: 99%

“…As for other connective tissue diseases, anti-inflammatory and immunosuppressive agents are considered the drugs of choice in patients with relapsing polychondritis [11,18,19] and, although there is no evidence that these drugs alter the natural course of the disease, they may be effective in suppressing the acute manifestations and, possibly, in reducing the frequency and the severity of recurrences. In our patient, a combination of prednisone, azathioprine, and cyclosporin was only partially effective against the inflammatory component of the bronchial lesions observed.…”

Section: Discussionmentioning

confidence: 99%

Severe endobronchial obstruction in a girl with relapsing polychondritis: treatment with Nd YAG laser and endobronchial silicon stent

Sacco¹,

Fregonese²,

Oddone³

et al. 1997

Eur Respir J

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Relapsing polychondritis (RP) is an uncommon disorder of unknown aetiology characterized by inflammation and destruction of the cartilaginous structures of many organs, including the tracheobronchial tree. When untreated, there is a high mortality rate, usually from respiratory obstructive complications.An 8 year old white girl, with a previous diagnosis of RP, was referred to our department for evaluation of worsening dyspnoea. Bronchoscopy showed localized inflammatory and fibrotic alterations of the mucosa, leading to severe obstruction of the left mainstem bronchus at its origin. The condition was successfully treated by endoscopic neodymium yttrium aluminium garnet (Nd YAG) laser. Re-evaluation of the patient, 7 months later, demonstrated bronchial stenosis and malacia requiring mechanical dilatation and positioning of an endobronchial silicon stent, which was well-tolerated by the patient. Eur Respir J 1997; 10: 494-496 Relapsing polychondritis is an uncommon inflammatory disease of unknown cause affecting cartilages in many organs and systems [1,2]. This disorder is manifested by recurrent and, generally, progressive inflammation of the cartilaginous portion of the external ear, joints, cardiovascular system and respiratory tract [2,3]. At the airway level, the extent of the disease may vary and all cartilage-containing structures can be affected [4]. Although onset of relapsing polychondritis may vary, the majority of cases appear between the ages of 40 and 60 yrs, with an equal sex distribution and no known familial predisposition [2][3][4].The most widely-accepted hypothesis for the pathogenesis of polychondritis is that a primary or secondary immune reaction against mucopolysaccharide components of cartilage ground substance results in severe inflammatory changes leading to the destruction of cartilage [4,5]. Histologically, the characteristic abnormalities are: 1) focal or diffuse loss of basophilic staining, indicating depletion of proteoglycans from the cartilage matrix; 2) inflammatory infiltrates, consisting predominantly of mononuclear cells and plasma cells; and 3) destruction of the cartilage with loss of chondrocytes [2][3][4][5].When untreated, this disorder is associated with a high mortality rate, usually from respiratory and cardiovascular complications, such as obstructive pneumonia, aortic valve disease and aortic aneurysm [4][5][6][7]. In addition to anti-inflammatory and immunosuppressive drugs, airway lesions may require a variety of interventions, including tracheostomy and bronchial resection [8]. Case reportAn 8 year old Caucasian girl was referred to our department for evaluation of worsening dyspnoea. At another institution, a diagnosis of relapsing polychondritis had been made 18 months previously, on the basis of the presence of: 1) nasal chondritis, resulting in collapse of the nasal bridge with a saddle deformity; 2) clinical, roentgenographic and endoscopic demonstration of inflammatory alteration of cartilages of the proximal trachea; 3) histological abnormality ...

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