IntroductionRP is a rare multisystem disorder characterized by inflammation of the cartilaginous structures of the body. Airway involvement is 50% and, is the major cause of death related to RP. 50% of deaths due to RP are due to respiratory involvement( 3,4).Male: Female -1:1. Average age at presentation: 47 years.

History and ExaminationA 40 y old male farmer, Cough and pleuritic chest pain for 02 weeks,Fever for 04 days ,No haemoptysis, wheeze, loss of appetite or loss of weightClinical examination : unremarkable

InvestigationsFBC: leukocytosis -15000mm-3

ESR: 123/1st hr 145/1st hrCXR : normal Sputum for acid fast bacilli : negativeMantoux : non-reactive Renal & liver functions : normal Blood picture : unremarkableANA & rheumatoid factor : negativeRx started as lower respiratory tact infection

02 weeks laterDeveloped pain in throat, nose, ears and, chest .Examination : tenderness of ears, nose, anterior neck and costochondral junctions Lung function tests : obstructive lung defect

Histology*Auricular cartilage biopsy : Mixed inflammatory infiltrates,loss of normal basophilia, chondrocytes and destructionof lacunar architecture.Features are consistent with RP.

DiscussionMD-CT including HRCT of the chest is capable of demonstrating airway involvement in RP (1).

Diffuse and smooth thickening of antero-lateral wall of the trachea with relative sparing of the posterior membranous wall is virtually pathognomonic of pulmonary involvement in RP (2).Tracheo-bronchial involvement is ideally demonstrated in mediastinal window.Lung algorithm is mandatory to visualize peribronchial thickening, focal air trapping and patchy atalectasis (2).These features were well appreciated in this case too.The most common CT finding of lower respiratorytract involvement in RP is increased airway wall attenuation (100%).Progressive cartilage calcification is a frequently reported feature of RP(6) and the degree of attenuation ranges from subtle to frank calcification. In our case no cartilage calcification was found but higher attenuation of airway wall was noted.As in our case, air trapping on expiration is well described key feature of RP and expiratory HRCT of chest is considered to be mandatory.

Respiratory Tract Involvement in Relapsing Polychondritis(RP) Muthunayake M.T., Dassanayake D.L.B., Gamage L.N.

Teaching Hospital, Kandy, Sri Lanka

1. McAdam LP, O’Hanlan MA, Bluestone R, Pearson CM. Relapsing polychondritis: prospective study of 23 patients and a review of the literature. Medicine 1976;55:193–2152. Trentham DE, Le CH. Relapsing polychondritis. Ann Intern Med 1998;129:114–122Keren S. Lee, MD, Armin Ernst, MD, David E. Trentham, MD, Relapsing Polychondritis: Prevalence of Expiratory CT Airway Involvement, Radiology RSNA 2006, Aug; 240; 565-571.3. Gergely P., Poor G. , Relapsing Polychodritis, Pract Best Clin 2004;18:723-738.4. Armin Ernst, Samaan Rafique, Phillip Boiselle., Relapsing Polychondritis ,CHEST 2009; 135: 1024-1030.5. Tillie-Leblond I, Wallaert B, Leblond D, et al. Respiratory involvement in relapsing olpychondritis: clinical, functional, endoscopic, and radiographic evaluations. Medicine 1998;77:168–1766. Jeffrey V. Behar ,Yo-Won Choi, et al. Relapsing Polychondritis Affecting the Lower Respiratory Tract, AJR:, January 2002;178:173-177.

References

Figure 1. Photograph of the of the right ear, inflamed (reddened) pinnaFigure 2. Histology of auricular cartilage*Figure 3. Expiratory HRCT of the chest, diffuse peribronchial inflammatory infiltrates and air trapping (black arrow).Figure 4. CT thorax; diffuse, smooth thickening of antero- lateral tracheal wall.

(1) (2)

(4)(3)

Respiratory Tract Involvement in Relapsing Polychondritis(RP)

MUTHUNAYAKE M.T.1, DASSANAYAKE D.L.B.2, GAMAGE L.N.3

1Senior Registrar in Radiology, 2Senior Registrar in Chest Medicine, 3Consultant Radiologist.

Teaching Hospital, Kandy, Sri Lanka

IntroductionRP is a rare multisystemic disorder characterized by inflammation of the cartilaginous structures of the body. In RP airway involvement is seen 50% of cases and found to be the major cause of deaths related to RP. 50% of deaths due to RP are due to respiratory involvement( 3,4).

Men and women are affected equally. The average age at presentation is 47 years.

The diagnosis is established when any three of the following clinical features are present: bilateral auricular chondritis, nonerosive seronegative inflammatory arthritis, nasal chondritis, ocular inflammation, respiratory tract chondritis, or audiovestibular damage (1). No specific histopathologic finding is considered pathognomonic for relapsing RP.

When the clinical picture is clear, biopsy is unnecessary (2). In less straightforward cases, however, biopsy may be required to exclude other diseases, including infection.

History and Examination

• A 40 y old male farmer • Cough and pleuritic chest pain for 02 weeks• Fever for 04 days .• No haemoptysis, wheeze, loss of appetite or

loss of weight• Clinical examination was unremarkable

Investigations

• FBC- leukocytosis -15000mm-3

• ESR - 123/1st hr.,145/1st hr• CXR - normal. • Sputum for acid fast bacilli – negative• Mantoux - non-reactive. • Renal & liver functions - normal • Blood picture – unremarkable• ANA & rheumatoid factor - negative.

02 weeks laterDeveloped pain in throat, nose and both ears chest wall pain. Examination - tenderness of ears, nose, anterior neck and costochondral junctions Lung function tests-obstructive lung defect

Figure 1; Photograph of the inflamed(reddened)pina of the right ear.

HRCT - Chest

Figure 1; Axial CT scan of chest reviles diffuse, smooth thickening of antero- lateral tracheal wall.

Figure 4; Axial CT scan of the chest reveals diffuse inflammatory infiltrates and expiratory HRCT shows focal air trapping(black arrow). peribronchial soft tissue thickening, consistent with RP.

Imaging Findings

CT scan of the chest diffuse, smooth thickening of antero- lateral tracheal wall.

HRCT scan of the chest diffuse peribronchial inflammatory infiltrates and expiratory HRCT shows focal air trapping.

These findings are consistent with RP

Histology

Nasal septal biopsy ; there is a mixed inflammatory infiltrate (lymphocytes, plasma cells, neutrophils, occasional eosinophils) extending into cartilage. Interface between cartilage and adjacent soft tissue is blurred. Cartilage demontrates loss of normal basophilia, loss of chondrocytes and destruction of lacunar architecture.

Features are consistent with RP.

Discussion

• MD-CT including HRCT of the chest is capable of demonstrating air way involvement in RP (1). Diffuse and smooth thickening of antero-lateral wall of the tracheal wall with relative sparing of the posterior membranous wall is virtually thought to be pathognomonic of pulmonary involvement in RP. Demonstration of traceo-bronchial involvement with mediastinal windowing and peribronchial thickening, focal air trapping and patchy atalectasis with lung algorhythm is very much important in making diagnosis (2).These features were well appreciated in this case too.

The most common CT finding of lower respiratorytract involvement by relapsing polychondritisis increased airway wall attenuation seen in (100%).The degree of increased attenuation rangedfrom subtle to frankly calcified. Progressive cartilagecalcification is a frequently reported featureof relapsing polychondritis (6).In our case no cartilage calcification was found buthigher attenuation of airway wall was noted.

Air way luminal narrowing was noted 80% of some case series but significant narrowing (25%of normal caliber) was not seen our patient(6).As in our case air trapping on expiration is well described as a key feature of RP and expiratory CT is considered to be mandatory as part of imaging.

CT is an important tool in the evaluation of patients with known or suspected RP. In patients with known disease, CT is useful for confirming intrathoracic involvement and for routine follow-up to assess progression of disease. Tillie-Leblond et al. (5) reported that CT was better than fiberoptic bronchoscopy in identifying airway stenoses and tracheal wall alterations in the early stages of disease. In patients without a known diagnosis of RP, the finding of diffuse increased airway wall attenuation and thickening at CT, with or without stenosis, suggests the diagnosis and should prompt a careful rheumatologic assessment (1, 2).

References

1. McAdam LP, O’Hanlan MA, Bluestone R, Pearson CM. Relapsing polychondritis: prospective study of 23 patients and a review of the literature. Medicine 1976;55:193–2152.Trentham DE, Le CH. Relapsing polychondritis. Ann Intern Med 1998;129:114–122Keren S. Lee, MD, Armin Ernst, MD, David E. Trentham, MD, Relapsing Polychondritis: Prevalence of Expiratory CT Airway

Involvement, Radiology RSNA 2006, Aug; 240; 565-571.3.Gergely P., Poor G. , Relapsing Polychodritis, Pract Best Clin 2004;18:723-738.4.Armin Ernst, Samaan Rafique, Phillip Boiselle., Relapsing Polychondritis ,CHEST 2009; 135: 1024-1030.5.Tillie-Leblond I, Wallaert B, Leblond D, et al. Respiratory involvement in relapsing polychondritis: clinical, functional,

endoscopic, and radiographic evaluations. Medicine 1998;77:168–1766. Jeffrey V. Behar ,Yo-Won Choi, et al. Relapsing Polychondritis Affecting the Lower Respiratory Tract, AJR:, January

2002;178:173-177.

Respiratory Tract Involvement in Relapsing Polychondritis(RP) MUTHUNAYAKE M.T.1, DASSANAYAKE D.L.B.2, GAMAGE L.N.3 1Senior Registrar in Radiology, 2Senior Registrar in Chest Medicine, 3Consultant Radiologist. Teaching Hospital, Kandy, Sri Lanka

IntroductionRP is a rare multisystemic disorder characterized by inflammation of the cartilaginous structures of the body. Airway involvement is seen 50% of cases and found to be the major cause of deaths related to RP. 50% of deaths due to RP are due to respiratory involvement( 3,4).Men and women are affected equally. The average age at presentation is 47 years.

History and ExaminationA 40 y old male farmer Cough and pleuritic chest pain for 02 weeksFever for 04 days .No haemoptysis, wheeze, loss of appetite or loss of weightClinical examination was unremarkable

InvestigationsFBC- leukocytosis -15000mm-3

ESR - 123/1st hr.,145/1st hrCXR - normal. Sputum for acid fast bacilli – negativeMantoux - non-reactive. Renal & liver functions - normal Blood picture – unremarkableANA & rheumatoid factor - negative.

Rx started as lower respiratory tact infection

02 weeks laterDeveloped pain in throat, nose and both ears chest wall pain.

Examination - tenderness of ears, nose, anterior neck and costochondral junctions Lung function tests-obstructive lung defect

Figure 1; Photograph of the inflamed(reddened)pina of the right ear.

Figure 2; a; CT thorax; diffuse, smooth thickening of antero- lateral tracheal wall.

b; HRCT scan of the chest diffuse peribronchial inflammatory infiltrates and expiratory HRCT shows focal air trapping.

HistologyNasal septal biopsy ;

there is a mixed inflammatory infiltrateCartilage demontrates loss of normal basophilia, loss of

chondrocytes and destruction of lacunar architecture.Features are consistent with RP.

DiscussionMD-CT including HRCT of the chest is capable of

demonstrating air way involvement in RP (1). Diffuse and smooth thickening of antero-lateral wall of the tracheal wall with relative sparing of the posterior membranous wall is virtually thought to be pathognomonic of pulmonary involvement in RP.Demonstration of traceo-bronchial involvement with mediastinal windowing and peribronchial thickening, focal air trapping and patchy atalectasis with lung algorhythm is very much important in making diagnosis (2).These features were well appreciated in this case too.The most common CT finding of lower respiratorytract involvement by RP is increased airway wall attenuation seen in (100%).The degree of increased attenuation rangedfrom subtle to frankly calcified. Progressive cartilagecalcification is a frequently reported featureof RP(6).In our case no cartilage calcification was found buthigher attenuation of airway wall was noted.As in our case air trapping on expiration is well described as a key feature of RP and expiratory CT is considered to be mandatory as part of imaging.

1. McAdam LP, O’Hanlan MA, Bluestone R, Pearson CM. Relapsing polychondritis: prospective study of 23 patients and a review of the literature. Medicine 1976;55:193–2152. Trentham DE, Le CH. Relapsing polychondritis. Ann Intern Med 1998;129:114–122Keren S. Lee, MD, Armin Ernst, MD, David E. Trentham, MD, Relapsing Polychondritis: Prevalence of Expiratory

CT Airway Involvement, Radiology RSNA 2006, Aug; 240; 565-571.3. Gergely P., Poor G. , Relapsing Polychodritis, Pract Best Clin 2004;18:723-738.4. Armin Ernst, Samaan Rafique, Phillip Boiselle., Relapsing Polychondritis ,CHEST 2009; 135: 1024-

1030.5. Tillie-Leblond I, Wallaert B, Leblond D, et al. Respiratory involvement in relapsing olpychondritis: clinical, functional, endoscopic, and radiographic evaluations. Medicine 1998;77:168–1766. Jeffrey V. Behar ,Yo-Won Choi, et al. Relapsing Polychondritis Affecting the Lower Respiratory Tract, AJR:, January 2002;178:173-177.

References