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Weekly Chest CasesArchive of Old Cases

Case No : 723 Date 2011-09-05

  • Courtesy of Nyoung Keun Lee, Chang Min Park, Jin Mo Goo / Seoul National University Hospital
  • Age/Sex 65 / M
  • Chief ComplaintFormy urine and odd smells in the urine
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4
  • Figure 5

Figure 1.

Diagnosis With Brief Discussion

Diagnosis
IgG4-related sclerosing disease
Radiologic Findings
Fig 1-2. Abdomen CT scan shows soft tissue masses in both renal pelvis and multifocal small nodular lesions in right kidney. And diffuse swelling of pancreas is also seen.
Fig 3. Neck CT scan shows low attenuating lesions in the right mandibular gland.
Fig 4. Chest CT scan shows soft tissue lesion surrounding the descending aorta.
Fig 5. Bronchovascular bundle thickenings are demonstrated in the central lung area.

Cutting needle biopsy was performed in of the right mandibular gland lesion, which confirmed that this disease was IgG4-related sclerosing disease. In FU images after the prednisolone medications, soft tissue lesions in the bilateral kidneys and around the descending aorta decreased, and the pancreas came back to normal. And, bronchovascular bundle thickenings were also resolved.
Brief Review
IgG4-related sclerosing disease is a systemic disease characterized by extensive IgG4-positive plasma cells and T-lymphocyte infiltration of various organs. Serum IgG4 levels and immunostaining with anti-IgG4 antibody are useful in making the diagnosis.
Clinical manifestations are apparent in the pancreas, bile duct, gallbladder, salivary gland, retroperitoneum, kidney, lung and prostate, in which tissue fibrosis with obliterative phlebitis is pathologically induced. Pancreatic disease that is indicative of IgG4-related sclerosing disease.
The disease occurs predominantly in older men, is frequently associated with lymphadenopathy, and responds well to steroid therapy. The precise pathogenesis and pathophysiology of IgG4-related sclerosing disease remain unclear.
Four major types of IgG4-related lung disease could be defined [2]: solid nodules, round-shaped GGOs, bronchovascular, and alveolar interstitial types. Solid nodular and round-shaped GGO-type lesions can mimic primary lung cancer including adenocarcinoma in situ. The differential diagnosis of bronchovascular-type lesions includes lymphoproliferative disorders such as multicentric Castleman disease, sarcoidosis, and lymphangitis carcinomatosa. Alveolar interstitial-type lesions radiologically resemble nonspecific interstitial pneumonia. IgG4-related disease seen in pancreas (autoimmune pancreatitis) or kidney shows a variety of imaging features despite the same pathologic change.
References
1. Kamisawa T, Okamoto A. IgG4-related sclerosing disease. World J Gastroenterol. 2008 Jul 7;14(25):3948-55.
2. Inoue D, Zen Y. Immunoglobulin G4-related lung disease: CT findings with pathologic correlations. Radiology. 2009 Apr;251(1):260-70.
Keywords
multiple, metabolic ans storage,

No. of Applicants : 74

▶ Correct Answer : 28/74,  37.8%
  • - Okayama University , Japan Akihiro Tada
  • - SAISEIKAI KURIHASHI HOSPITAL , Japan YASUO OOKUBO
  • - Oita University, Faculty of Medicine , Japan Fumito Okada
  • - Yokohama-asahi-chuo-general hospital , Japan Kyoko Nagai
  • - Onomichi municipal hospital , Japan Hirofumi Mifune
  • - Onomichi municipal hospital , Japan Ryotaro Kishi
  • - Seoul national university hospital , Korea (South) Sun Ah Kim
  • - Severance hospital , Korea (South) Saerom Hong
  • - Toyama University Hospital, Laboratory of Pathology , Japan TOMONORI TANAKA
  • - UWO , Canada S Lee
  • - Kohka Public Hospital , Japan Akitoshi Inoue
  • - Fukuyama daiichi Hospital , Japan Mototsugu Saeki
  • - China Medical University ,Taiwan,R.O.C. , Taiwan Jun Jun Yeh
  • - CH de la C�te Basque , France Paul ARDILOUZE
  • - snuh , Korea (South) Choo jiyung
  • - Hanyang university medical center , Korea (South) YOONAH SONG
  • - All India Institute of medical sciences , India Justin Moses
  • - Ishikawa Matto Central Hospital , Japan Manabu Akimoto
  • - Asan Medical Center , Korea (South) Sanghyun Choi
  • - Kizawa Memorial Hospital , Japan Yo Kaneko
  • - IRSA La Rochelle France , France Denis Chabassiere
  • - Okayama university, Department of Pathology , Japan Junya Itakura
  • - Seoul St Mary Hospital , Korea (South) Chae Lim
  • - Yonsei University College of Medicine, Shinchon Severance hospital , Korea (South) Young Joo Suh
  • - Kyungpook National university hospital , Korea (South) MyoungHun Ham
  • - IRCCS Istituto Oncologico - Bari , Italy Carlo Florio
  • - Hotel-Dieu Montreal , Canada nicolas gautier
  • - Okayama university , Japan Mayu Uka
▶ Correct Answer as Differential Diagnosis : 1/74,  1.4%
  • - National Jewish Health , United States Jonathan Chung
▶ Semi-Correct Answer : 2/74,  2.7%
  • - Mallinckrodt Institute of Radiology , United States Naganathan Mani
  • - NASA SCANS , India RAKESH BHATIA
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