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Weekly Chest CasesCases by Disease Category

Case No : 1380 Date 2024-04-02

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  • Courtesy of Kyung Eun Shin / Soonchunhyang University Hospital, Bucheon
  • Age/Sex 63 / M
  • Chief Complaintoliguria, cough, sputum → After one week, the sudden onset of hemoptysis accompanied by worsening dyspnea.
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4
  • Figure 5
  • Figure 6

date 06.01

Diagnosis With Brief Discussion

Diagnosis
Microscopic polyangiitis (MPA)
Radiologic Findings
Fig 1. Chest CT scans show honeycombing cysts and consolidation in the right lower lobe.
Fig 2. Chest radiograph reveals a mild increase in peribranchial opacity in the right lung. Reticular opacities are combined in the right lower lung zone. A scant amount of right pleural and fissural effusion is observed on the chest radiograph.
Fig 3. Chest radiograph shows multifocal increased opacities in both lungs. The patients symptoms abruptly progressed with the sudden onset of hemoptysis at that time.
Fig 4. Chest CT scans display multifocal consolidation, GGO, with a relatively sparing subpleural area in both lungs. Bronchoscopy reveals bleeding.

The patient underwent renal biopsy, and the results indicated ANCA-associated crescentic glomerulonephritis. The laboratory test for p ANPA was positive. The patient was finally diagnosed with microscopic polyangiitis.
Brief Review
Antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) are a group of disorders that include granulomatosis with polyangiitis (GPA, formerly Wegeners granulomatosis), microscopic polyangiitis (MPA), renal-limited vasculitis, and eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg Strauss syndrome). All are associated with ANCA, affect predominantly small-sized arteries, and have similar features on kidney histology.
Patients with MPA typically present with symptoms and signs of small-vessel vasculitis involving the airways and lungs; kidneys; skin; eyes; and/or peripheral nervous system. While the kidneys and skin are very commonly affected, ENT involvement is less frequent in MPA than in GPA. Most patients (55 to 65 percent) are positive for MPO-ANCA. Tissue biopsy typically shows necrotizing vasculitis primarily affecting small-to-medium vessels; pauci-immune necrotizing glomerulonephritis is common. In contrast with GPA, granulomatous inflammation is generally absent.
MPA typically occurs in middle-aged adults; men are more commonly involved. Glomerulonephritis develops in 90% of patients, and other organs may be involved. In Europe, the most frequent pulmonary manifestation of MPA is diffuse alveolar hemorrhage (DAH) (20.4%) associated with dyspnea and hemoptysis. According to Suzukis study, the frequency of pulmonary involvement in MPA in Japan was 50% for interstitial pneumonia (mostly UIP pattern) and 10% for diffuse alveolar hemorrhage, respectively. Pleural effusion and pulmonary edema are seen in about 10%, related to renal disease. Treatment with cyclophosphamide and steroids often results in remission.
References
1. Jennette JC, Falk RJ et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013;65(1):1.
2. Savage CO, Winearls CG, Evans DJ et al. Microscopic polyarteritis: presentation, pathology and prognosis. GJ Med 1985;56:467-483.
3. Aika Suzuki, Susumu Sakamoto et al. Chest High-Resolution CT Findings of Microscopic Polyangiitis: A Japanese First Nationwide Prospective Cohort Study. AJR 2019 Jul;213(1):104-114.
Keywords

No. of Applicants : 77

▶ Correct Answer : 22/77,  28.6%
  • - Saitama-Sekishinkai Hosptal , Japan MIHOKO YAMAZAKI
  • - The University of Tokyo Hospital , Japan JUN KANZAWA
  • - Oita university , Japan AYUMI KAMEI
  • - Kyoto City Hospital , Japan YUSUKE UTSUNOMIYA
  • - , Korea (South) DONG-HO BANG
  • - Healthy Longevity Medical Center , Japan SHIN-ICHI CHO
  • - Vita Hospital , Brazil DIOGO LAGO PINHEIRO
  • - Other , Korea (South) SEONGSU KANG
  • - Oita University, Faculty of Medicine , Japan FUMITO OKADA
  • - Uniyersity of Yamanashi , Japan TAKAAKI HASHIMOTO
  • - The University of Tokyo Hospital , Japan TOSHIHIRO FURUTA
  • - University of Yamanashi , Japan HIROAKI WATANABE
  • - Nankai medical center , Japan NORITAKA KAMEI
  • - Osaka Metropolitan University Hospital , Japan SHU MATSUSHITA
  • - , Japan SHUNJIRO NOGUCHI
  • - Tokyo Medical and Dental University , Japan MIKIYA FUJII
  • - Yonsei University,Severance Hospital , Korea (South) SEO BUM CHO
  • - Fukuoka university , Japan KEISUKE SATO
  • - Kantou Rousai Hospital , Japan KAORU SUMIDA
  • - Nerimahikarigaoka hospital , Japan TAKANA HAYASHI
  • - , Japan YUMI MAEHARA
  • - Toyota Kosei Hospital , Japan YUKI HAYASHI
▶ Correct Answer as Differential Diagnosis : 15/77,  19.5%
  • - medical scanning , Japan HIROAKI ARAKAWA
  • - Kyoto University , Japan AKIHIKO SAKATA
  • - IRSA LA ROCHELLE , France JEAN LUC BIGOT
  • - Secomedic Hospital , Japan FUMINORI MIYOSHI
  • - Osaka University , Japan AKINORI HATA
  • - Soonchunhyang University Hospital Bucheon , Korea (South) HYEJOO PARK
  • - Nagoya University , Japan YASUSHI AOYAGI
  • - , Japan YOSHIKI ISHII
  • - , Japan KENTARO KOTANI
  • - , Korea (South) HEONSEOK LEE
  • - Osaka metropolitan university Hospital , Japan TATSUSHI OURA
  • - Matsunami General Hospital , Japan TARO TAKEDA
  • - University of Yamanashi , Japan TIHAN WUMU
  • - , Korea (South) JIN WOO YOON
  • - Jiangsu province hospital , China WANGJIAN ZHA
▶ Semi-Correct Answer : 20/77,  26.0%
  • - , Japan SUZUNE TSUKAMOTO
  • - Shiga University of Medical Science , Japan AKITOSHI INOUE
  • - McGill University Health Center , Canada ALEXANDRE SEMIONOV
  • - NIMS, HYDERABAD , India BHASKAR K
  • - , Italy PAOLO BALDASSARI
  • - Mie University , Japan HIKARI FUKUI
  • - Gifu University Hospital , Japan Yo Kaneko
  • - Jichi Medical University, School of Medicine , Japan MITSURU MATSUKI
  • - University of Tsukuba, Dept of Radiology , Japan MANABU MINAMI
  • - Avrasya Hospital/ISTANBUL , Turkey MURAT ULUSOY
  • - Mie university , Japan SHIKO OKABE
  • - GHICL , France MANUEL TOLEDANO
  • - Dokkyo Medical University , Japan HIROAKI ARAKAWA
  • - , Algeria SAMIR SALAH AIECH
  • - , Taiwan SHIHHAN PAN
  • - Kyoto University , Japan SHO KOYASU
  • - Ichinomiya Nishi Hospital , Japan Takao Kiguchi
  • - the first affiliatited hospital of nanjing medical univercity , China HAI XU
  • - University of Yamanashi , Japan KOJIRO ONOHARA
  • - , China LI GUO
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