A thirty four year old female presented with upper and lower respiratory symptoms in the third trimester of pregnancy. After the delivery of a healthy baby, the symptoms progressed to involve multiple organ systems and eventually a diagnosis of limited Wegener's Granulomatosis (Carrington-Liebow syndrome) was made. The extremely rare combination of WG and pregnancy, especially the onset of disease in late pregnancy is discussed. The successful outcome of pregnancy even without treatment of WG is the highlight of the case.
Matched MeSH terms: Granulomatosis with Polyangiitis/diagnosis*
A 35-year-old Malay man underwent treatment for uveitis of the right eye in 1992 but developed marked visual impairment in the affected eye after he failed to attend follow-up. Two years later, he complained of difficulty swallowing and was found to have left sided X and XI cranial nerve palsies. Chest radiograph showed a cavitating lesion in the lower zone of the right lung field. Inflammation and perforation of the nasal septum was found on examination of his upper respiratory tract. Punch biopsies taken from that area showed chronic inflammatory change and necrotizing vasculities. The patient was diagnosed as having Wegener's granulomatosis and made a very good recovery with immunosuppressive therapy.
Matched MeSH terms: Granulomatosis with Polyangiitis/diagnosis
Wegener's granulomatosis is a rare multi-system disease characterized by the classic triad of necrotizing granulomas affecting the upper and lower respiratory tracts, disseminated vasculitis and glomerulonephritis. Oral lesions as a presenting feature are only encountered in 2% of these cases. Hyperplastic gingival lesions or strawberry gingivitis, is a characteristic sign of Wegener's granulomatosis. The latter consists of reddish-purple exophytic gingival swellings with petechial haemorrhages thus resembling strawberries. Recognition of this feature is of utmost importance for timely diagnosis and definitive management of this potentially fatal disease. A case of strawberry gingivitis as the first presenting sign of Wegener's granulomatosis affecting a 50-year-old Malay male is reported here. The differential diagnosis of red lesions that may present in the gingiva is discussed.
Matched MeSH terms: Granulomatosis with Polyangiitis/diagnosis*
Granulomatosis with polyangiitis (GPA), previously Wegener's granulomatosis, requires prompt diagnosis and systemic review to exclude life-threatening disease. However, early diagnosis of orbital GPA may be difficult because anti-neutrophil cytoplasmic antibody (ANCA) and anti-PR3 antibody screening can be negative at presentation and orbital biopsies taken for diagnosis may not show the classic features of GPA. This study was designed to compare GPA with other causes of orbital inflammation and to identify the presenting clinical and imaging features most likely to predict GPA and its systemic spread.
Matched MeSH terms: Granulomatosis with Polyangiitis/diagnosis*
Lacrimal gland involvement in granulomatosis with polyangiitis (GPA) commonly accompanies orbital disease, but occasionally may be the sole presentation preceding any other organ manifestation or systemic disease. Diagnosis of orbital GPA, especially in patients with lacrimal involvement as the initial presentation, can be difficult because of nonspecific clinical features and lack of diagnostic specificity on histologic and antineutrophilic cytoplasmic antibody (ANCA) testing. Orbital GPA can be associated with a high morbidity from potential visual loss or rapid progression of latent systemic disease, making early diagnosis important. The purpose of this study was to describe the clinical and imaging features of patients with lacrimal gland involvement secondary to GPA and to compare them with those of other orbital inflammatory conditions in the lacrimal gland fossa.
Matched MeSH terms: Granulomatosis with Polyangiitis/diagnosis*
A 36-year-old Danish man, living in Asia, was diagnosed with Pneumocystis pneumonia (PCP) and HIV in 2013 (CD4+ count: 6 cells/µL; viral load: 518 000 copies/mL). He initiated combination antiretroviral therapy. Later that year, he was also diagnosed with granulomatosis with polyangiitis and was treated with prednisolone. Despite complete viral suppression and increasing CD4+ count (162 cells/µL), he was readmitted with PCP in April 2015. Subsequently, he returned to Denmark (CD4+ count: 80 cells/µL, viral suppression). Over the following months, he developed progressive dyspnoea. Lung function tests demonstrated severely reduced lung capacity with an obstructive pattern and a moderately reduced diffusion capacity. High resolution computer tomography revealed minor areas with tree-in-bud pattern and no signs of air trapping on expiratory views. Lung biopsy showed lymphocytic infiltration surrounding the bronchioles with sparing of the alveolar septa. He was diagnosed with follicular bronchiolitis. The patient spontaneously recovered along with an improvement of the immune system.
Matched MeSH terms: Granulomatosis with Polyangiitis/diagnosis