http://informahealthcare.com/mor ISSN 1439-7595 (print), 1439-7609 (online) Mod Rheumatol, 2014; 24(6): 1019–1022 © 2014 Japan College of Rheumatology DOI: 10.3109/14397595.2013.874746
CASE REPORT
A case of relapsing polychondritis initially presenting with bronchial chondritis Yoko Nakazato, Fumitaka Mizoguchi, Hitoshi Kohsaka, and Nobuyuki Miyasaka Department of Medicine and Rheumatology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Bunkyo-ku, Tokyo, Japan Abstract
Keywords
A 51-year-old woman presented with one-month history of fever, productive cough, dyspnea, hoarseness and polyarthritis. Computed tomography (CT) depicted diffuse bronchotracheal stenosis, which deteriorated in exhalation. Three-dimensional CT revealed airway stenosis from the trachea to the lobar bronchi. We made a diagnosis of relapsing polychondritis (RP). Administration of high-dose glucocorticoid and oral cyclophosphamide resolved the symptoms and the bronchial stenosis. It was noteworthy that all features presented were bronchial chondritis and polyarthritis. RP should be considered when patients have polyarthritis with respiratory symptoms. The dynamic expiratory CT and three-dimensional image reconstructions are useful for detecting and following up bronchotracheal involvement.
Bronchial chondritis, Computed tomography, Polyarthritis, Relapsing polychondritis, Three-dimensional reconstruction History Received 14 November 2012 Accepted 7 February 2013 Published online 7 March 2013
Abbreviations CT: Computed tomography; RP: Relapsing polychondritis; CRP: C-reactive protein; ESR: Erythrocyte sedimentation rate; FDG-PET: 18F-Fluorodeoxyglucose positron-emission tomography
Introduction Relapsing polychondritis (RP) is a progressive inflammatory disease of cartilaginous structures, including elastic cartilage of the ear and nose, hyaline cartilage of the peripheral joints, fibrocartilage at axial sites, and hyaline cartilage of the tracheobronchial tree [1]. It affects external ear, nose, eye, trachea and joints at 26–91, 13–33, 14–33, 14–38, 23–47 % of reported cases, respectively [1–4]. The diagnosis can be established when inflammation in the cartilage is confirmed. Clinically, involvement of the ears and nose is often the key to the diagnosis because the distribution of inflammation coincides with the cartilaginous tissue area. While histological chondritis confirms the diagnosis, diagnosis of the patient without ear or nose involvement is difficult. Here we report a case of RP who developed laryngotracheal inflammation and arthritis without any other involvements. Although respiratory distress progressed rapidly, immediate immunosuppressive treatments ameliorated the disease.
Case report A 51-year-old Japanese woman presented with fever and dyspnea with cough and sputum. Three weeks later, arthritis of the bilateral elbows, wrists, fingers, knees, and ankles developed and made her immobile. She was admitted to our hospital after a week. Her body temperature was 38.5 °C, heart rate was 115/min, blood pressure was 118/68 mmHg, and respiratory rate was 18/ Correspondence to: Nobuyuki Miyasaka, Department of Medicine and Rheumatology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, 1-5-45 Yushima, Bunkyo-ku, Tokyo 113-8510, Japan. Tel: +81-3-38136111. Fax: +81-3-58035998. E-mail: [email protected]
min. Physical examination revealed inspiratory stridor in the left lung and swelling with tenderness of the bilateral wrists, fingers, knees, ankles and toes. There was no abnormality of the ears, nose or eyes. A blood test revealed white blood cells 8,000/mm3, hemoglobin 9.2 g/dl, and platelets 525 × 109/l. C-reactive protein (CRP) 139.5 mg/l and erythrocyte sedimentation rate (ESR) 135 mm/h were elevated. Rheumatoid factors, anti-cyclic citrullinated peptide antibody, PR3-ANCA and MPO-ANCA were negative. Arterial blood gas revealed low O2 tension (71.6 mmHg) and high CO2 tension (49.1 mmHg), indicating alveolar hypoventilation. A chest radiogram showed normal lung field with narrowing of the trachea and main bronchi (Figure 1). A CT revealed thickening of the wall with calcification and stenosis of the trachea, which deteriorated with exhalation (Figure 2a–c). Three-dimensional CT depicted diffuse stenosis from trachea to lobar bronchi (Figure 3). An obstructive pattern was observed in spirometry: forced vital capacity was 2.19 l (89.7 % of predicted) and ratio of FEV1 to forced vital capacity (FEV1.0%) was 53.7 %. A bronchoscopy identified significant supraglottic edema and focal stenosis in the tracheobronchial tree. The airway collapsed easily with suction during the bronchoscopy (Figure 4a–d). Based on the presence of polyarthritis and clinical and laboratory findings of the pulmonary tracts, a diagnosis of RP was made without histological examination. As the patient’s dyspnea deteriorated rapidly, she was treated with high-dose oral prednisolone (1 mg/kg/day) and oral cyclophosphamide (50 mg/ day). The fever and arthritis resolved, and the CRP and ESR decreased to normal levels within several days. The cough and hoarseness gradually disappeared (Figure 5). After 2 months, a repeat chest CT revealed resolution of the tracheal thickening and stenosis (Figure 2d). Rafeq et al. [5] suggested that biopsies should be considered during bronchoscopy if there is any
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Figure 3. Three dimensional CT showing diffuse stenosis of respiratory tract from trachea to lobar bronchi (arrows) Figure 1. Chest radiogram on admission showing normal lung field with narrowing of the trachea (arrow)
process existing in the airway and its cause is otherwise unclear. Also they stated that when the biopsies were performed, they should include deep biopsies of the anterior cartilaginous tracheal wall. Our case revealed the findings typical of RP in the bronchoscopy and the CT, therefore we considered that the diagnosis was RP and started the treatment. In addition, as the patient had significant supraglottic and tracheobronchial edema and her airway collapsed easily, we decided not to perform biopsies due to high risk of an acute flare-up of disease leading to worsening of edema, bronchospasm, bleeding and respiratory failure.
Discussion The present case illustrates that RP should be an important differential diagnosis of polyarthritis even without involvement of the nose, ears or eyes. Expiratory CT and three-dimensional reconstruction of the tracheobronchial images were useful to establish the diagnosis. Using McAdam’s diagnostic criteria, it is necessary to fulfill at least three of six defined typical symptoms [2]. Histological proof is recommended to confirm the diagnosis. The present case had only two symptoms of cartilaginous structure involvement, and these were not readily accessible for histological evaluation. Damiani et al. [6] proposed modified criteria to include patients with clinical
Figure 2. a Chest CT showing stenosis of trachea in the left main bronchi (arrows). b The stenosis deteriorated with exhalation (arrows). c Chest CT showing thickening of the tracheal wall with calcification (arrow). d Repeat chest CT after two months of treatment showed resolution of the thickening and stenosis of the tracheal wall
DOI 10.3109/14397595.2013.874746
Figure 4. a–c Bronchoscopy image representing significant supraglottic edema and focal stenosis in the tracheobronchial tree. d The airway collapsed easily with suction
Figure 5. Clinial course and efficacy of high-dose oral prednisolone (PSL) and oral cyclophosphamide (CPA). The symptoms resolved, and the CRP (C-reactive protein) decreased to a normal level within several days
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chondritis at two or more separate anatomic locations as well as with responsiveness to steroids and/or dapsone. Although there is no reported case of RP who presented only arthritis and respiratory symptoms, imaging studies of our case disclosed respiratory tract involvement that suggested presence of chondritis. This involvement was improved with corticosteroids and an immunosuppressant. The diagnosis of RP was also supported by the presence of the increased anti-type-II collagen antibodies in this case (>107 EU/ ml; normal:
CASE REPORT
A case of relapsing polychondritis initially presenting with bronchial chondritis Yoko Nakazato, Fumitaka Mizoguchi, Hitoshi Kohsaka, and Nobuyuki Miyasaka Department of Medicine and Rheumatology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Bunkyo-ku, Tokyo, Japan Abstract
Keywords
A 51-year-old woman presented with one-month history of fever, productive cough, dyspnea, hoarseness and polyarthritis. Computed tomography (CT) depicted diffuse bronchotracheal stenosis, which deteriorated in exhalation. Three-dimensional CT revealed airway stenosis from the trachea to the lobar bronchi. We made a diagnosis of relapsing polychondritis (RP). Administration of high-dose glucocorticoid and oral cyclophosphamide resolved the symptoms and the bronchial stenosis. It was noteworthy that all features presented were bronchial chondritis and polyarthritis. RP should be considered when patients have polyarthritis with respiratory symptoms. The dynamic expiratory CT and three-dimensional image reconstructions are useful for detecting and following up bronchotracheal involvement.
Bronchial chondritis, Computed tomography, Polyarthritis, Relapsing polychondritis, Three-dimensional reconstruction History Received 14 November 2012 Accepted 7 February 2013 Published online 7 March 2013
Abbreviations CT: Computed tomography; RP: Relapsing polychondritis; CRP: C-reactive protein; ESR: Erythrocyte sedimentation rate; FDG-PET: 18F-Fluorodeoxyglucose positron-emission tomography
Introduction Relapsing polychondritis (RP) is a progressive inflammatory disease of cartilaginous structures, including elastic cartilage of the ear and nose, hyaline cartilage of the peripheral joints, fibrocartilage at axial sites, and hyaline cartilage of the tracheobronchial tree [1]. It affects external ear, nose, eye, trachea and joints at 26–91, 13–33, 14–33, 14–38, 23–47 % of reported cases, respectively [1–4]. The diagnosis can be established when inflammation in the cartilage is confirmed. Clinically, involvement of the ears and nose is often the key to the diagnosis because the distribution of inflammation coincides with the cartilaginous tissue area. While histological chondritis confirms the diagnosis, diagnosis of the patient without ear or nose involvement is difficult. Here we report a case of RP who developed laryngotracheal inflammation and arthritis without any other involvements. Although respiratory distress progressed rapidly, immediate immunosuppressive treatments ameliorated the disease.
Case report A 51-year-old Japanese woman presented with fever and dyspnea with cough and sputum. Three weeks later, arthritis of the bilateral elbows, wrists, fingers, knees, and ankles developed and made her immobile. She was admitted to our hospital after a week. Her body temperature was 38.5 °C, heart rate was 115/min, blood pressure was 118/68 mmHg, and respiratory rate was 18/ Correspondence to: Nobuyuki Miyasaka, Department of Medicine and Rheumatology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, 1-5-45 Yushima, Bunkyo-ku, Tokyo 113-8510, Japan. Tel: +81-3-38136111. Fax: +81-3-58035998. E-mail: [email protected]
min. Physical examination revealed inspiratory stridor in the left lung and swelling with tenderness of the bilateral wrists, fingers, knees, ankles and toes. There was no abnormality of the ears, nose or eyes. A blood test revealed white blood cells 8,000/mm3, hemoglobin 9.2 g/dl, and platelets 525 × 109/l. C-reactive protein (CRP) 139.5 mg/l and erythrocyte sedimentation rate (ESR) 135 mm/h were elevated. Rheumatoid factors, anti-cyclic citrullinated peptide antibody, PR3-ANCA and MPO-ANCA were negative. Arterial blood gas revealed low O2 tension (71.6 mmHg) and high CO2 tension (49.1 mmHg), indicating alveolar hypoventilation. A chest radiogram showed normal lung field with narrowing of the trachea and main bronchi (Figure 1). A CT revealed thickening of the wall with calcification and stenosis of the trachea, which deteriorated with exhalation (Figure 2a–c). Three-dimensional CT depicted diffuse stenosis from trachea to lobar bronchi (Figure 3). An obstructive pattern was observed in spirometry: forced vital capacity was 2.19 l (89.7 % of predicted) and ratio of FEV1 to forced vital capacity (FEV1.0%) was 53.7 %. A bronchoscopy identified significant supraglottic edema and focal stenosis in the tracheobronchial tree. The airway collapsed easily with suction during the bronchoscopy (Figure 4a–d). Based on the presence of polyarthritis and clinical and laboratory findings of the pulmonary tracts, a diagnosis of RP was made without histological examination. As the patient’s dyspnea deteriorated rapidly, she was treated with high-dose oral prednisolone (1 mg/kg/day) and oral cyclophosphamide (50 mg/ day). The fever and arthritis resolved, and the CRP and ESR decreased to normal levels within several days. The cough and hoarseness gradually disappeared (Figure 5). After 2 months, a repeat chest CT revealed resolution of the tracheal thickening and stenosis (Figure 2d). Rafeq et al. [5] suggested that biopsies should be considered during bronchoscopy if there is any
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Mod Rheumatol, 2014; 24(6): 1019–1022
Figure 3. Three dimensional CT showing diffuse stenosis of respiratory tract from trachea to lobar bronchi (arrows) Figure 1. Chest radiogram on admission showing normal lung field with narrowing of the trachea (arrow)
process existing in the airway and its cause is otherwise unclear. Also they stated that when the biopsies were performed, they should include deep biopsies of the anterior cartilaginous tracheal wall. Our case revealed the findings typical of RP in the bronchoscopy and the CT, therefore we considered that the diagnosis was RP and started the treatment. In addition, as the patient had significant supraglottic and tracheobronchial edema and her airway collapsed easily, we decided not to perform biopsies due to high risk of an acute flare-up of disease leading to worsening of edema, bronchospasm, bleeding and respiratory failure.
Discussion The present case illustrates that RP should be an important differential diagnosis of polyarthritis even without involvement of the nose, ears or eyes. Expiratory CT and three-dimensional reconstruction of the tracheobronchial images were useful to establish the diagnosis. Using McAdam’s diagnostic criteria, it is necessary to fulfill at least three of six defined typical symptoms [2]. Histological proof is recommended to confirm the diagnosis. The present case had only two symptoms of cartilaginous structure involvement, and these were not readily accessible for histological evaluation. Damiani et al. [6] proposed modified criteria to include patients with clinical
Figure 2. a Chest CT showing stenosis of trachea in the left main bronchi (arrows). b The stenosis deteriorated with exhalation (arrows). c Chest CT showing thickening of the tracheal wall with calcification (arrow). d Repeat chest CT after two months of treatment showed resolution of the thickening and stenosis of the tracheal wall
DOI 10.3109/14397595.2013.874746
Figure 4. a–c Bronchoscopy image representing significant supraglottic edema and focal stenosis in the tracheobronchial tree. d The airway collapsed easily with suction
Figure 5. Clinial course and efficacy of high-dose oral prednisolone (PSL) and oral cyclophosphamide (CPA). The symptoms resolved, and the CRP (C-reactive protein) decreased to a normal level within several days
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chondritis at two or more separate anatomic locations as well as with responsiveness to steroids and/or dapsone. Although there is no reported case of RP who presented only arthritis and respiratory symptoms, imaging studies of our case disclosed respiratory tract involvement that suggested presence of chondritis. This involvement was improved with corticosteroids and an immunosuppressant. The diagnosis of RP was also supported by the presence of the increased anti-type-II collagen antibodies in this case (>107 EU/ ml; normal:
