MyMedR

Eosinophilic Granulomatosis with Polyangiitis

Yong GY, Lim AL

N Engl J Med, 2022 Jan 27;386(4):374.

PMID: 35061339 DOI: 10.1056/NEJMicm2112515

Matched MeSH terms: Granulomatosis with Polyangiitis/drug therapy; Granulomatosis with Polyangiitis/pathology*

Fulltext An unusual case of Wegener's granulomatosis

Loke YK, Tan MH

Med J Malaysia, 1998 Mar;53(1):107-9.

PMID: 10968148

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/complications*; Granulomatosis with Polyangiitis/diagnosis; Granulomatosis with Polyangiitis/drug therapy

Strawberry gingivitis as the first presenting sign of Wegener's granulomatosis: report of a case

Siar CH, Yeo KB, Nakano K, Nagatsuka H, Tsujigiwa H, Tomida M, et al.

Eur J Med Res, 2011 Jul 25;16(7):331-4.

PMID: 21813375

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/complications; Granulomatosis with Polyangiitis/diagnosis*; Granulomatosis with Polyangiitis/drug therapy; Granulomatosis with Polyangiitis/pathology

Fulltext Successful pregnancy in untreated limited Wegener's granulomatosis

Sahni V, Agarwal SK, Singh NP, Sikdar S, Yadav A, Wadhwa A, et al.

Med J Malaysia, 2005 Oct;60(4):492-4.

PMID: 16570714

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*; Granulomatosis with Polyangiitis/physiopathology

Fulltext A case of Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss, EGPA) presenting with relapsed Mononeuritis multiplex

Hilmi, B.A., Ainon, M.M.

International Medical Journal Malaysia, 2014;13(1):69-72.

MyJurnal

We report a case of eosinophilic granulomatosis with polyangiitis (EGPA), a rare multisystem disorder characterized by difficult-to-control asthma, hypereosinophilia and polyneuropathy. We also discuss the Five Factor Score (FFS) risk stratification strategy, which is used to quantitate the extent of the disease and guide treatment strategy.

Matched MeSH terms: Granulomatosis with Polyangiitis

Tissue Interleukin-17 and Interleukin-23 as Biomarkers for Orbital Granulomatosis with Polyangiitis

Isa H, Luthert P, Rose G, Verity D, Pusey C, Tomkins-Netzer O, et al.

Ophthalmology, 2015 Oct;122(10):2140-2.

PMID: 26116342 DOI: 10.1016/j.ophtha.2015.04.016

Matched MeSH terms: Granulomatosis with Polyangiitis/diagnosis*; Granulomatosis with Polyangiitis/metabolism

Clinical and Imaging Features of Lacrimal Gland Involvement in Granulomatosis with Polyangiitis

Tan LT, Davagnanam I, Isa H, Rose GE, Verity DH, Pusey CD, et al.

Ophthalmology, 2015 Oct;122(10):2125-9.

PMID: 26233627 DOI: 10.1016/j.ophtha.2015.06.026

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/complications; Granulomatosis with Polyangiitis/diagnosis*; Granulomatosis with Polyangiitis/pathology

Fulltext Cavitary lung lesions: Melioidosis and pulmonary embolism causing necrotizing pneumonia

Mohammad N, Wan Ghazali WS

IDCases, 2017;10:4-6.

PMID: 28791214 DOI: 10.1016/j.idcr.2017.07.008

Cavitary lung lesions of various etiologies may be encountered in patients with respiratory symptoms associated with fever. Non-malignant cavitary lesions may mimic malignant lung lesions on most of radiographic modalities including chest radiographs or thoracic computed tomography (CT). Primary lung malignancy can be detected in as high as one-fifths of CT thorax as cavitary lesions and the remaining aetiologies may be due to bacterial, parasitic, and invasive fungal infections, as well as Granulomatosis with polyangiitis (GPA), sarcoidosis, septic thrombo-embolism, and lung metastasis from extra-pulmonary primaries. We report an interesting case of melioidosis infection complicated with pulmonary embolism, both of which can lead to cavitary lung lesions and subsequently cause a clinical conundrum.

Matched MeSH terms: Granulomatosis with Polyangiitis

Macula retinal vasculitis and choroiditis associated with granulomatosis with polyangiitis

Lim LT, Vasudevan V, Shelton J, Vitale AT, Moorthy R

Retin Cases Brief Rep, 2017 Spring;11(2):119-122.

PMID: 27305849 DOI: 10.1097/ICB.0000000000000307

PURPOSE: To report a case series of two cases of granulomatosis with polyangiitis, previously known as Wegener granulomatosis, which developed macular necrosis, not previously associated with granulomatosis with polyangiitis, healed with fibrosis, despite aggressive immune-modulating therapy and good control of systemic disease.

METHODS: Case series of two cases with observation of treatment progress.

RESULTS: The results reported the progress of response to treatment in the two cases, which resulted in the final outcome of fibrosis in the macula region, despite being on aggressive immune-modulating therapy and good systemic control.

CONCLUSION: Granulomatosis with polyangiitis can be associated with macular necrosis leading to fibrosis, unresponsive to immune-modulating therapy.

Matched MeSH terms: Granulomatosis with Polyangiitis/complications*

Fulltext Cutaneous Vasculitis as Early Presentation of Eosinophilic Granulomatosis with Polyangiitis without Systemic Symptoms

Sulaiman W, Abdullah AC, Chung SF, Karim N, Tang JJ

Oman Med J, 2019 Jul;34(4):345-349.

PMID: 31360325 DOI: 10.5001/omj.2019.67

Eosinophilic granulomatosis with polyangiitis (formerly known as Churg-Strauss syndrome) is a rare vasculitis in children. It commonly presents with respiratory symptoms, especially asthma, allergic rhinitis, and peripheral eosinophilia. Involvement of other systems, such as renal and cardiac, may carry a poor prognosis. Anti-neutrophil cytoplasmic antibodies have been found less frequently in children with this condition. We report a case of a 15-year-old male who presented with indurated and pruritic papules on both legs and peripheral eosinophilia without other system involvement. Histopathological findings from a skin biopsy were suggestive of eosinophilic granulomatosis with polyangiitis. Anti-neutrophil cytoplasmic antibodies were negative.

Matched MeSH terms: Granulomatosis with Polyangiitis

Fulltext Upper respiratory tract symptoms, renal involvement and vasculitis: a case report and review of wegener granulomatosis

Said MS

J Clin Med Res, 2010 Aug 18;2(4):189-93.

PMID: 21629538 DOI: 10.4021/jocmr412w

Wegeners Granulomatosis is a condition associated with systemic vasculitis which can present with upper respiratory tract symptoms initially. On September 2001, a 15-year-old girl presented with symptoms of nasal block for 3 weeks. She later developed joint pains and worsening renal status requiring dialysis. A renal biopsy was performed which showed pauci-immune cresentric glomerulonephritis. Her cANCA levels were positive. She was treated with oral cyclophosphamide and steroids and later responded.

KEYWORDS: Wegener granulomatosis; Young girl; Cyclophophamide; cANCA.

Matched MeSH terms: Granulomatosis with Polyangiitis

Clinical and imaging features predictive of orbital granulomatosis with polyangiitis and the risk of systemic involvement

Tan LT, Davagnanam I, Isa H, Taylor SR, Rose GE, Verity DH, et al.

Ophthalmology, 2014 Jun;121(6):1304-9.

PMID: 24560566 DOI: 10.1016/j.ophtha.2013.12.003

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*

Fulltext Immunofluorescence (IF) pattern, autoantigens of anti-neutrophil cytoplasmic antibodies (ANCA) and their clinical associations

Masita Arip, Marlyn Mohammad, Salbiah Nawi, Shahnaz Murad

Jurnal Sains Kesihatan Malaysia, 2010;8(2):37-41.

MyJurnal

Antineutrophil cytoplasmic antibodies (ANCA) are autoantibodies directed against primary granules of neutrophils and monocytes' lysosomes. In general, c-ANCA is strongly associated with vasculitic disorders mainly in ANCA-associated systemic vasculitis (AASV). p-ANCA have been identified in several diseases such as primary (AASV) and secondary vasculitis such as collagen vascular diseases, rheumatoid arthritis and inflammatory bowel diseases given the term 'ANCA-associated disease.' The objective of this study was to determine the rate of ANCA positivity by indirect immunofluorescent (IF) and enzyme linked immunosorbent assay (ELISA) and its association with AASV and ANCA associated diseases. Serum from patients with history suspicion of systemic vasculitis were tested for ANCA by IF. Those samples positive for ANCA by IF were further tested for antibodies against myeloperoxidase (MPO) and proteinase 3 (PR3) using the ELISA. Clinical data from medical records were obtained and analyzed. Of 468 samples, a total of 110 were positive for ANCA by IF. IF results showed a p-ANCA pattern in 96 patients and c-ANCA in 14. Of 110 IF positive ANCA, 45 patients were positive by ELISA. Seventeen were positive for MPO-ANCA, 9 were PR3-ANCA positive and 19 were both MPO and PR3 positive. Only 2 patients were classified AASV ie Wegener granulomatosis and the other with microscopic polyangiitis. The remaining patients (n = 108) may be classified as ANCA associated diseases. Our study showed that pANCA (87.3%) was the more common ANCA pattern and 40.9% of IF positive samples were positive for PR3- and MPOANCA.

Matched MeSH terms: Granulomatosis with Polyangiitis

Follicular bronchiolitis in an HIV-infected individual on combination antiretroviral therapy with low CD4+ cell count but sustained viral suppression

Rasmussen LD, Pedersen C, Madsen HD, Laursen CB

BMJ Case Rep, 2017 Nov 29;2017.

PMID: 29191821 DOI: 10.1136/bcr-2017-221025

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; Granulomatosis with Polyangiitis/drug therapy

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