Pulmonary Capillaritis in Wegener's Granulomatosis Detected Via Transbronchial Lung Biopsy

Ishiguro, Takashi; Takayanagi, Noboru; Yamaguchi, Shozaburo; Shimizu, Yoshihiko; Yanagisawa, Tsutomu; Sugita, Yutaka; Kawabata, Yoshinori

doi:10.2169/internalmedicine.51.6518

Cited by 5 publications

(2 citation statements)

References 16 publications

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“…Pulmonary involvement ranges from 60-85%, classically as capillaritis or granulomatosis masses, which could lead to diffuse alveolar hemorrhage (DAH) [2]. DAH has been reported over a wide range from 7-40%, and usually the results of a mass or untreated capillaritis [4,6]. DAH is characteristically not the presenting symptom, as seen in our Fig.…”

Section: Discussionmentioning

confidence: 63%

A Case of Pulmonary Hemorrhage and Renal Failure

Harrington

Foster

Vachharajani³

et al. 2012

TOUNJ

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Background: Alveolar hemorrhage can be seen in many vasculitic disorders. However, granulomatosis polyangiitis (formerly Wegener's granulomatosis) uncommonly presents with life threatening alveolar hemorrhage and has only been discussed in a few case reports [1]. Case Presentation: A 53 year old Caucasian male presented with hemoptysis and profound anemia. Two weeks prior, he had presented with abdominal pain with normal renal function and numerous pulmonary nodules. During the current admission, the patient was hypoxic with acute renal failure requiring hemodialysis. Urine sediment demonstrated dysmorphic red blood cells. A bronchoscopy revealed diffuse alveolar hemorrhage. The diagnosis of pulmonary-renal syndrome was made and therapeutic plasma exchange was initiated. Laboratory studies were significant for a c-ANCA titer positive at 1:640 FIU and anti-proteinase (PR)-3 antibody titer positive with 78.3 U/ml. Renal biopsy demonstrated necrotizing crescentic glomerulonephritis. A diagnosis of granulomatosis vasculitis was determined. Conclusion: Alveolar hemorrhage is rare to be the presenting symptom of granulomatosis vasculitis where the common presenting features are recurrent sinusitis, epistaxis, chronic otitis media or rhinitis. Physicians should consider granulomatosis vasculitis in the differential diagnosis of pulmonary-renal syndrome presenting with hemoptysis.

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Section: Discussionmentioning

confidence: 63%

A Case of Pulmonary Hemorrhage and Renal Failure

Harrington

Foster

Vachharajani³

et al. 2012

TOUNJ

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“…3,4,16,17 This treatment leads to remis� sion of the disease in 90% of cases. 18 There have been reports of the risks of developing interstitial pulmonary fibrosis fol� lowing a history of bleeding into the alveoli of patients with the AAV. 19 Presented patient received high doses of cyclophospha� mide and corticosteroids from the first day of hospitaliza� tion which quick improvement of DAH symptoms.…”

Section: Discussionmentioning

confidence: 99%

Granulomatosis with polyangiitis preceded with pyoderma gangrenosum in 41-year-old man

et al. 2020

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Introduction: A coexistence of pyoderma gangrenosum (PG) and granulomatosis with polyangiitis (GPA) is observed extremely rarely. Some authors believe that skin ulcers like-PG may be a cutaneous manifestation of the GPA. They highlight the difficulties in assessing the histopathological picture: lack of clear diagnostic criteria for PG and recommendation to repeat biopsy in order to determine the proper diagnosis. Aim: This article presents the case of 41-year-old man with GPA preceded with PG. Case study: We present the case of 41-year-old male with GPA (with the occurrence of bleeding into the alveoli, bleeding from the lower gastrointestinal tract and renal involvement) preceded with PG. He also had pathergy test positive. The patient was treated with cyclophosphamide and corticosteroid with improvement. Results and discussion: The presence of c-anti-neutrophil cytoplasmic antibodies (c-ANCAs) levels, despite the classic image of PG with a characteristic histology, requires special observation of patients who may develop systemic GPA. Such patients should have frequent assessments of internal organ involvement. ANCAs control is recommended since their concentrations correlate with disease activity. They may affect the re-activation and chemotaxis of neutrophils. Conclusions: The presence of c-ANCAs levels, despite the classic image of PG with a characteristic histology, requires special observation of patients who may develop systemic GPA.

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Appendicitis as first presentation of aggressive granulomatosis with polyangiitis and diffuse alveolar haemorrhage

de Noronha Moreira,

Moniz,

Mendes

et al. 2024

BMJ Case Rep

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Acute respiratory distress syndrome (ARDS) can be associated with a wide multiplicity of causes, including diffuse alveolar haemorrhage (DAH). Vasculitis associated with anti-neutrophil cytoplasmic antibody (ANCA), particularly granulomatosis with polyangiitis (GPA), is one of the most common causes of DAH, primarily affecting small and medium-sized vessels in the lungs and kidneys. Diagnosing GPA can be challenging, and it should be considered a potential cause of DAH, even in the absence of other organ involvement. Given the high mortality associated with this condition, early diagnosis and aggressive treatment are crucial for improving outcomes. We present the case of a young patient with severe ARDS who did not show significant alveolar haemorrhage on bronchoscopy or renal involvement but exhibited extensive gastrointestinal involvement and had a predominant laboratory diagnosis of positive cytoplasmic anti-neutrophil cytoplasmic antibody and proteinase 3 antibodies.

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Pulmonary Capillaritis in Wegener's Granulomatosis Detected Via Transbronchial Lung Biopsy

Cited by 5 publications

References 16 publications

A Case of Pulmonary Hemorrhage and Renal Failure

A Case of Pulmonary Hemorrhage and Renal Failure

Granulomatosis with polyangiitis preceded with pyoderma gangrenosum in 41-year-old man

Appendicitis as first presentation of aggressive granulomatosis with polyangiitis and diffuse alveolar haemorrhage

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