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

Abstract

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 ...

Refractory relapsing polychondritis in a child treated with antiCD20 monoclonal antibody (rituximab): First case report

“…So far only 14 cases have been reported in English literature (below 13 years of age), the lowest age being 2 years [7][8][9][10][11][12][13][14][15][16][17][18][19]. Most of them presented with stridor or respiratory distress.…”

“…The disease can have a co-existence with other autoimmune disease such as Sjogrens syndrome, systemic lupus erythematosis, myelodysplasia, and other inflammatory conditions in 30% of cases [6]. To the best of our knowledge, English literature review published only 14 cases of pediatric onset relapsing polychondritis below 13 yrs till date [7][8][9][10][11][12][13][14][15][16][17][18][19].…”

Respiratory insufficiency and dynamic hyperinflation after rigid bronchoscopy in a patient with relapsing polychondritis -a case report-

“…Major complications with stent placement are migration, pneumothorax, persistent cough, retained secretions, infection, hemorrhage, and formation of granulation tissues [9]. Most important thing in stent placement is choosing the location at which the stents will do the most good [10]. However, in case of diffuse involvement, as in this patient, the effect of stenting would not outweigh the risk of complications [11].…”

Relapsing polychondritis, an underestimated dermatological urgency: case report and literature review

“…Damiani and Levine proposed a modification of these criteria: for the diagnosis, (i) all patients had to have at least three or more McAdam diagnostic criteria, the histologic confirmation is not necessary; (ii) one or more of McAdam's signs with histologic confirmation; (iii) chondritis in two or more different anatomic locations with response to steroids and/or dapsone treatment. The epidemiological data show an incidence of approximately 0.71, 2.0, and 3.5 per million from population studies in the United Kingdom, Hungary, and Minnesota, USA, respectively, with a slight predominance in Caucasian women than in men (2:1 to 4:1) between the 4th to 5th decade of life, although it can also affect the pediatric population …”