granulomatosis with polyangiitis

Teenager with

cough, fever and shortness of breath. CXR AP and lateral (above) show bilateral thick walled cavitary nodules in the lungs. Axial CT with contrast of the chest (below) shows just how thick walled the cavitary nodules are in the left lung. Multiple smaller non-cavitary nodules are seen scattered throughout both lungs.The diagnosis was granulomatosis with polyangiitis.

pediatricimaging.org • CC-by-nc-sa 4.0

An adolescent

with both Wegener"s Granulomatosis and chronic blastomycosis. Chest CT showed a 3 cm cavitary lesion in the left upper lobe with surrounding consolidation.

ped-rheum.biomedcentral.com • CC-by-2.0

Imaging of

intestinal vasculitis focusing on MR and CT enterography: a two-way street between radiologic findings and clinical data. Intestinal vasculitis and pulmonary alveolar hemorrhage in a 41-year-old male with ANCA-positive granulomatosis with polyangiitis (GPA) presenting with abdominal pain and hemoptysis. Axial thoracic CT images (A, B) at two different levels show diffuse mosaic attenuation secondary to geographic areas of ground-glass opacity, suggestive for alveolar hemorrhage. Follow-up CT images (C, D) three days after corticosteroid therapy demonstrate a notable response to treatment. CTE was also done with suboptimal small bowel distention (E, F) at admission to assess the cause of abdominal pain that revealed circumferential mural thickening at ascending colon and distal ileum (thin white arrows) associated with adjacent mesenteric fat haziness. Follow-up CT images (G, H) show complete response to corticosteroid therapy with normal bowel wall thickness (thick white arrows) and a decrease in attenuation of the ileocolic mesenteric root

insightsimaging.springeropen.com • CC-by-4.0

Imaging of

intestinal vasculitis focusing on MR and CT enterography: a two-way street between radiologic findings and clinical data. Intestinal vasculitis and perforation in a 56-year-old male with known ANCA-positive granulomatosis with polyangiitis and long history of immunosuppressive therapy presenting with acute abdominal pain and leukocytosis. Chest X-ray (A) shows two pulmonary nodules projecting over the lower zone of the left hemithorax (black dotted ovals). MRE was obtained. Axial T2-W image (B) reveals multi-focal segmental mural thickening in jejunal loops (thin black arrows). Axial post-contrast T1-W image (C) displays intense mural hyperenhancement (thin white arrows) in the involved segments. Focal intestinal pneumatosis (white dotted ovals) is also evident. Axial T2-W image (D) shows pneumoperitoneum (white arrowheads) at the non-dependent portion of the abdominal cavity, indicating bowel perforation. Coronal T2-W image (E) demonstrates small amount of free fluid (thick white arrows) at the abdominal cavity. Due to acute nature of presentation, the patient subsequently underwent laparotomy. Intraoperative photograph (F) reveals focal bowel necrosis and perforation. No signs of peritonitis were found in the initial examination, more likely due to the immunocompromised status of the patient

insightsimaging.springeropen.com • CC-by-4.0

Granulomatosis

with polyangiitis (thoracic manifestations) • Wegener granulomatosis - Ganzer Fall bei Radiopaedia

Rosen, Y. Wegener granulomatosis. Case study, Radiopaedia.org. (accessed on 20 Oct 2022) https://doi.org/10.53347/rID-16217 • CC by-nc-sa 3.0 (modified)

Granulomatosis

with polyangiitis (thoracic manifestations) • Granulomatosis with polyangiitis - Ganzer Fall bei Radiopaedia

Radswiki, T. Granulomatosis with polyangiitis. Case study, Radiopaedia.org. (accessed on 20 Oct 2022) https://doi.org/10.53347/rID-12079 • CC by-nc-sa 3.0 (modified)

Granulomatosis

with polyangiitis (thoracic manifestations) • Wegener granulomatosis - Ganzer Fall bei Radiopaedia

Byrne, A. Wegener granulomatosis. Case study, Radiopaedia.org. (accessed on 20 Oct 2022) https://doi.org/10.53347/rID-8123 • CC by-nc-sa 3.0 (modified)

A rare case

report of polyangiitis overlap syndrome: granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis. CT imaging revealing left upper lobe consolidation with central cavitary lesion, adjacent scattered consolidation, ground-glass opacities and tree-in-bud markings

bmcpulmmed.biomedcentral.com • CC-by-4.0

Breast

involvement in granulomatosis with polyangiitis: a case report. (a–c) Axial and coronal sections of PET/CT a–c show multilobulated soft tissue density lesion with increased metabolic activity in the right breast (thick arrow) with hypermetabolic soft tissue density lesions in bilateral lungs, largest lesion showing central cavitation in the right lower lobe (thin arrow). There was splenomegaly with diffuse ill-defined hypermetabolic nodular lesions (curved arrow)

ejrnm.springeropen.com • CC-by-4.0

Breast

involvement in granulomatosis with polyangiitis: a case report. (a–d) Axial and coronal sections of contrast-enhanced CT thorax of 48-year female in arterial (a, b) and venous phase (c, d) show well-defined, heterogeneously enhancing lung parenchymal lesions of varying sizes (thin arrow, star) and the largest of these lesions with central cavitation seen in the right lower lobe (star). A heterogeneously enhancing lesion noted in the right breast (open arrow in a, c)

ejrnm.springeropen.com • CC-by-4.0

Sigmoid colon

perforation in the patient with granulomatosis with polyangiitis. Abdominal contrast-enhanced computed tomography showing a colonic perforation. Mesenteric emphysema (a, arrow) and inflammation (b, arrowhead) of the sigmoid colon are visible

surgicalcasereports.springeropen.com • CC-by-4.0

Granulomatosis

with polyangiitis • Granulomatosis with polyangiitis - orbital involvment - Ganzer Fall bei Radiopaedia

Ibrahim, D. Granulomatosis with polyangiitis - orbital involvment. Case study, Radiopaedia.org. (accessed on 20 Oct 2022) https://doi.org/10.53347/rID-51884 • CC by-nc-sa 3.0 (modified)

Granulomatosis

with polyangiitis • Granulomatosis with polyangitis - Ganzer Fall bei Radiopaedia

Greif, E. Granulomatosis with polyangitis. Case study, Radiopaedia.org. (accessed on 20 Oct 2022) https://doi.org/10.53347/rID-35506 • CC by-nc-sa 3.0 (modified)

Granulomatosis

with polyangiitis • Wegener granulomatosis - Ganzer Fall bei Radiopaedia

Rosen, Y. Wegener granulomatosis. Case study, Radiopaedia.org. (accessed on 20 Oct 2022) https://doi.org/10.53347/rID-16217 • CC by-nc-sa 3.0 (modified)

Granulomatosis

with polyangiitis • Nasal septum perforation secondary to granulomatosis with polyangiitis - Ganzer Fall bei Radiopaedia

Gaillard, F. Nasal septum perforation secondary to granulomatosis with polyangiitis. Case study, Radiopaedia.org. (accessed on 20 Oct 2022) https://doi.org/10.53347/rID-6021 • CC by-nc-sa 3.0 (modified)

Differentiating

tracheobronchial involvement in granulomatosis with polyangiitis and relapsing polychondritis on chest CT: a cohort study. Granulomatosis with polyangiitis. A Coronal reformation of the unenhanced chest computed tomography shows focal tracheal wall thickening (arrow). B On the axial image, the tracheal wall thickening (arrow) is circumferential and responsible for lumen stenosis. C Below, the thoracic trachea is normal

arthritis-research.biomedcentral.com • CC-by-4.0

Granulomatosis

with polyangiitis (thoracic manifestations) • Wegener granulomatosis (gross pathology) - Ganzer Fall bei Radiopaedia

Rosen, Y. Wegener granulomatosis (gross pathology). Case study, Radiopaedia.org. (accessed on 20 Oct 2022) https://doi.org/10.53347/rID-8950 • CC by-nc-sa 3.0 (modified)

Granulomatosis

with polyangiitis (thoracic manifestations) • Wegener granulomatosis - H&E - Ganzer Fall bei Radiopaedia

Rosen, Y. Wegener granulomatosis - H&E. Case study, Radiopaedia.org. (accessed on 20 Oct 2022) https://doi.org/10.53347/rID-8951 • CC by-nc-sa 3.0 (modified)

Radiopaedia • CC-by-nc-sa 3.0 • de

Granulomatosis with polyangiitis (GPA), previously known as Wegener granulomatosis, is a multisystem necrotizing non-caseating granulomatous c-ANCA positive vasculitis affecting small to medium-sized arteries, capillaries, and veins, with a predilection for the respiratory system and kidneys.

This article discusses GPA in general. For organ-specific radiographic features, please refer to individual articles:

Epidemiology

There is a slight male predilection and onset is typically at approximately 50 years of age .

Clinical presentation

Presentation depends on which organ systems are involved:

cough and hemoptysis
subacute to chronic history of nasal obstruction, rhinitis, and epistaxis
proteinuria and hematuria

Symptoms related to other organ systems are less frequent, due to a corresponding infrequency of involvement (musculoskeletal symptoms, ocular symptoms, skin changes) .

Systemic symptoms such as anorexia, malaise and fever are also common .

Diagnostic criteria

The 1990 American College of Rheumatology criteria requires at least two of the four listed below (sensitivity 88.2% and specificity 92%)

positive biopsy for granulomatous vasculitis
urinary sediment with red blood cells
abnormal chest radiograph
oral or nasal inflammation

Pathology

It results from an immune-mediated vascular injury.

Histology

Histologically necrotizing granulomas with an associated vasculitis are the dominant feature.

Markers

In 90% of cases, cANCA (PR3) is positive and the levels correlate with disease activity .

Classification

The classic triad of organ involvement consists of:

lungs: involved in 95% of cases
upper respiratory tract / sinuses: 75-90%
kidneys: 80%

In terms of extent, granulomatosis with polyangiitis can be classified as:

classical: full triad
limited: not having the full triad
- usually respiratory tract involvement only
- renal only involvement has been described but is uncommon
widespread: additional organ involvement
- skin (50%)
- eyes (45%)
- peripheral nervous system (35%)
- occasionally also other organs, such as the heart and gastrointestinal tract

Treatment and prognosis

Treatment is typically with cyclophosphamide, methotrexate and/or steroids. More recently agents such as Rituximab are also used.

Without treatment, granulomatosis with polyangiitis is rapidly progressive with death usually occurring within a year of diagnosis, with only 10% of patients surviving 2 years . Appropriate medical therapy has dramatically increased long term survival .

History and etymology

The former name "Wegener granulomatosis" comes from the German pathologist Friedrich Wegener who first described it in 1936 . Wegener was a member of the Nazi party and it is suspected that he took part in experiments on concentration camp inmates. Following the discovery of his past history, the current name "granulomatosis with polyangiitis" has been proposed .