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Case ReportImages in Rheumatology

Severe Acute Atelectasis Caused by Complete Obstruction of Left Main Stem Bronchus Associated with Granulomatosis with Polyangiitis

RYO KUWATA, YUKO SHIROTA and TOMONORI ISHII
The Journal of Rheumatology August 2020, 47 (8) 1293-1294; DOI: https://doi.org/10.3899/jrheum.190971
RYO KUWATA
Department of Hematology and Rheumatology, Tohoku University Hospital, Sendai, Miyagi, and National Center for Global Health and Medicine, Tokyo;
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  • ORCID record for RYO KUWATA
YUKO SHIROTA
Department of Hematology and Rheumatology, Tohoku University Hospital, and Department of Hematology and Rheumatology, Tohoku Medical and Pharmaceutical University, Sendai, Miyagi;
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  • For correspondence: shirotay@med.tohoku.ac.jp
TOMONORI ISHII
Department of Hematology and Rheumatology, Tohoku University Hospital, Sendai, Miyagi, Japan.
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Although tracheobronchial stenosis (TBS) is a relatively uncommon clinical manifestation of granulomatosis with polyangiitis (GPA)1,2, it can cause severe airway obstruction, leading to fatal consequences1,2,3,4,5.

A 43-year-old woman with biopsy-proven GPA presented with sudden severe chest pain and dyspnea without any other symptoms such as cough or fever. She had a 7-year history of recurrent orbital lesions, sinusitis, and septal perforation, controlled with 10 mg/day of prednisolone (PSL), tacrolimus, azathioprine, and rituximab. Her breathing sound was diminished in the left lung field. C-reactive protein and proteinase 3–antineutrophil cytoplasmic antibodies were negative. Chest radiography showed significant mediastinal shift due to atelectasis (Figure 1–A1). Computed tomography demonstrated complete obstruction of the left main stem bronchus (MSB; Figure 1–A2 to 1–A4). Bronchoscopy confirmed that the bronchial lumen was blocked with inflamed and necrotic tissue and bleeding (Figure 2). Cytology and culture of bronchoalveolar lavage fluid were negative. She was treated with pulsed methylprednisolone followed by 50 mg/day of PSL. On Day 5, the MSB obstruction was relieved without any mechanical therapies. Followup chest imaging in 2 months showed reopening of the obstructed bronchial lumen (Figure 1–B1 to 1–B4).

Figure 1.
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Figure 1.

Chest radiographs (A1 and B1) and corresponding computed tomographic scans in axial (A2 and B2) and coronal (A3 and B3) planes, and with 3-D reconstruction (A4 and B4). A. Significant mediastinal shift due to total atelectasis of the left lung (A1) resulted from complete obstruction of the left main stem bronchus on Day 0 (A2–4: arrowhead). B. The atelectasis was drastically improved (B1) by reopening of the obstructed bronchial lumen after high-dose steroid therapy for 2 months (B2–4: arrowhead).

Figure 2.
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Figure 2.

A bronchoscopic image of the tracheal bifurcation. The lumen of the left main stem bronchus was blocked with inflamed and necrotic tissue and bleeding (arrowheads).

There are relatively few reports focusing on treatments for TBS with GPA so far2,4,5. This is a highly notable case of unpredictable complete MSB obstruction without any other active symptoms, showing drastic improvement following high-dose steroid therapy. As previously reported, ENT manifestations are frequently associated with TBS1. Practitioners need to be aware of the importance of surveillance chest imaging for patients with GPA having ENT involvement, to detect TBS1.

Footnotes

  • The ethics review board at Tohoku University Hospital approved this report (#16840). The patient gave written informed consent to publish the material.

REFERENCES

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    Subglottic stenosis and endobronchial disease in granulomatosis with polyangiitis. Rheumatology 2019;58:2203–11.
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    1. Girard C,
    2. Charles P,
    3. Terrier B,
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    Tracheobronchial stenoses in granulomatosis with polyangiitis (Wegener’s): a report on 26 cases. Medicine 2015;94:e1088.
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    Multilevel airway stenosis in patients with granulomatosis with polyangiitis (Wegener’s). Am J Otolaryngol 2015;36:361–3.
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    1. Terrier B,
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    5. Kahn JE,
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    Granulomatosis with polyangiitis: endoscopic management of tracheobronchial stenosis: results from a multicentre experience. Rheumatology 2015;54:1852–7.
    OpenUrlCrossRefPubMed
  5. 5.↵
    1. Marroquin-Fabian E,
    2. Ruiz N,
    3. Mena-Zuniga J,
    4. Flores-Suarez LF
    . Frequency, treatment, evolution, and factors associated with the presence of tracheobronchial stenoses in granulomatosis with polyangiitis. Retrospective analysis of a case series from a single respiratory referral center. Semin Arthritis Rheum 2019;48:714–9.
    OpenUrl
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Severe Acute Atelectasis Caused by Complete Obstruction of Left Main Stem Bronchus Associated with Granulomatosis with Polyangiitis
RYO KUWATA, YUKO SHIROTA, TOMONORI ISHII
The Journal of Rheumatology Aug 2020, 47 (8) 1293-1294; DOI: 10.3899/jrheum.190971

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Severe Acute Atelectasis Caused by Complete Obstruction of Left Main Stem Bronchus Associated with Granulomatosis with Polyangiitis
RYO KUWATA, YUKO SHIROTA, TOMONORI ISHII
The Journal of Rheumatology Aug 2020, 47 (8) 1293-1294; DOI: 10.3899/jrheum.190971
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