Concurrent occurrence of allergic granulomatous angiitis and temporal arteritis: a case report and review of the literature

Abstract

Allergic granulomatous angiitis (AGA) is a disease entity that was first distinguished from classical polyarteritis nodosa by Churg and Strauss in 1951, and is characterized by the clinical features of allergic rhinitis or bronchial asthma, eosinophilia, and vasculitis. Allergic granulomatous angiitis has been described to mainly involve small vessels. We herein describe a case of Churg–Strauss syndrome which demonstrated the clinical and laboratory findings supporting a diagnosis of AGA and was also associated with the clinical and pathological findings for temporal arteritis, which was characterized by eosinophil infiltration and granuloma formation of the temporal artery (middle-sized vessel).     

Churg-Strauss syndrome revealed by granulomatous acute pericarditis: two case reports and a review of the literature

BACKGROUND: Churg-Strauss syndrome (CSS) is a necrotizing systemic vasculitis with extravascular granulomas and eosinophilic infiltrates of small vessels. CSS is usually revealed by nonspecific signs of necrotizing vasculitis in a context of late-onset asthma and blood eosinophilia. It is considered a systemic vasculitis with the highest prevalence of cardiac involvement and can lead to rapid-onset heart failure due to specific cardiomyopathy. Pericardial effusion may also occur during CSS and is usually well tolerated. OBJECTIVE: The objective of these case reports was to indicate that CSS may present as tamponade, with or without other visceral involvement. METHODS: Among CSS patients treated during the past 10 years at 2 French university hospitals, we have identified and described 2 cases revealed by tamponade with pericardial biopsy-proven granulomatous vasculitis. We have also reviewed the international medical literature in PubMed on cardiac involvement in CSS. RESULTS: The first case report describes a 66-year-old man who had an isolated cardiac tamponade with both inflammatory syndrome and eosinophilia. Long-term remission was obtained with corticosteroids. The second case report describes a 46-year-old woman whose CSS presented with tamponade and associated central nervous system and myocardial involvement. Remission was obtained with corticosteroids and cyclophosphamide. In both cases, CSS was assessed by histological analysis of a pericardial sample. CONCLUSIONS: CSS may present as isolated cardiac tamponade. Whereas pericarditis with myocardial injury warrants immunosuppressive therapy, isolated pericarditis without other visceral involvement of poor prognosis only requires corticosteroid therapy.     

Combined Churg–Strauss syndrome and allergic bronchopulmonary aspergillosis – case report and review of the literature

A rare case of combined Churg–Strauss syndrome (CSS) and allergic bronchopulmonary aspergillosis (ABPA) was presented. A 41‐year‐old woman was diagnosed with CSS based upon asthma, eosinophilia (23%), chest radiographic findings, paranasal sinusitis, peripheral neuropathy and positive p‐ anti‐neutrophil cytoplasmic antibodies (pANCA). The diagnosis of ABPA was established on the pathological findings of allegic mucin impaction and fungal hyphae on lung biopsy. It was further proved by positive serum IgE and IgG antibodies specific to afumigatus. The clinical investigation features were reviewed in the patients with combined CSS and ABPA. All patients had the time sequence of the development of CSS after ABPA uniformly, suggesting immunopathogenesis involving the emergence of CSS. The role of lung biopsy in the diagnosis of the condition was emphasized. Please cite this paper as: Ren S. Combined Churg–Strauss syndrome and allergic bronchopulmonary aspergillosis – case report and review of the literature. Clin Respir J 2013; 7: e6–e10.     

Methotrexate in refractory bilateral juvenile temporal arteritis: Report of a case

Juvenile temporal arteritis is a rare inflammatory disease of the temporal arteries that affects young adults. The clinical course is benign and the surgical excision of the affected artery is usually curative. Here we report a case of bilateral juvenile temporal arteritis with significant peripheral eosinophilia and elevated IgE, refractory to surgical excision and even to a short course of corticosteroids. Methotrexate, added as a steroid-sparing agent, resulted in a good disease control.     

Churg–strauss syndrome: importance of colonoscopy and endoscopic biopsy in establishing diagnosis

A 31‐year‐old Japanese man was admitted with complaints of left lower abdominal pain and diarrhea. He had a history of bronchial asthma since he was 26 years old. On admission, his eosinophil count was 4100/mm ³ and increased as high as 12 000/mm ³ . A neurological examination disclosed mononeuritis multiplex with paresthesia in both lower and upper extremities. Because of hematochezia, colonoscopy was performed and disclosed multiple colonic ulcers and erosions from the rectum to the ascending colon. Endoscopic biopsy showed fibrinoid necrosis of small‐sized vessels with moderate infiltration of eosinophils in the mucosa and submucosa. He was suspected of being a case with Churg–Strauss syndrome (CSS; allergic granulomatous angiitis), and prednisolone of 30 mg/day was administered. After the administration of prednisolone, his eosinophil count decreased dramatically. His symptoms also improved gradually, except for numbness in the extremities. Three weeks later, colonoscopy revealed improvement of the mucosal appearance, but the colonic ulcers and erosions were still present. Endoscopic biopsy showed exravascular granuloma with moderate infiltration of eosinophils in the submucosa. Colonoscopy and endoscopic biopsy may be important in establishing the diagnosis of CSS with intestinal symptoms.     

Churg-Strauss综合征肺损害1例并文献复习

目的 加深对Churg-Strauss综合征又名变应性肉芽肿血管炎(CSS)的认识.方法 通过对1例CSS病例的诊断、治疗及相关文献进行研究学习,分析Churg-Strauss综合征的病因、临床症状、实验室检查、影像学表现、诊断、鉴别诊断及治疗.结果 CSS病因不清,临床表现无特异性,可累及多系统器官,最常见的临床表现是哮喘及血管炎引起的肺部表现,特征性的病理表现为外周血嗜酸粒细胞增多,目前治疗首选激素,预后较好.结论 Churg-Strauss综合征是一种少见的系统性血管炎,临床缺乏特异性表现,易被误诊,当患者表现为哮喘、外周血嗜酸性粒细胞增多及肉芽肿性血管炎时,应高度怀疑此病.     

Oral and ocular/orbital manifestations of temporal arteritis: a disease with deceptive clinical symptoms and devastating consequences

Temporal arteritis (TA) is a chronic, systemic vasculitis most often presenting with severe headaches localized in the temporal region, low-grade fever, anorexia, weight loss and generalized malaise. Besides these typical characteristics, a number of vague and non-specific oral and/or ocular symptoms may also be present. A search using Medline (1955–2006) was performed for unusual oral and ocular/orbital presentations of TA. A variety of oral and ocular/orbital manifestations associated with TA have been reported. These can mislead physicians, causing a delay in establishing a diagnosis and initiating treatment. Increased awareness is necessary for the prompt recognition of this potentially devastating disease. Particularly, dentists and ophthalmologists should include TA in their differential diagnosis, as they may be the first to deal with these patients.     

误诊为大动脉炎的纤维肌性发育不良一例分析及文献复习

目的提高风湿科医生对纤维肌性发育不良的认识。方法报告一例被误诊为大动脉炎的纤维肌性发育不良病例并复习相关文献。结果患者为16岁女性,急性起病,以高血压脑病为主要临床表现,所有免疫指标均阴性,多次查血沉、C反应蛋白等炎性指标均正常,血管造影显示右肾动脉主干远段狭窄,狭窄段呈串珠样改变,证实纤维肌性发育不良的诊断。球囊扩张术后临床症状消失,血压恢复正常,随诊半年病情稳定。结论纤维肌性发育不良是一种动脉病,因其发病的性别年龄特点及病变分布与大动脉炎相似,故临床上易与大动脉炎相混淆,但纤维肌性发育不良无特异性的炎性表现,血管造影呈典型的串珠样改变,累及肾动脉时位于肾动脉主干的远段而区别于大动脉炎,风湿科医生应予重视。     

Candida arteritis in kidney transplant recipients: case report and review of the literature

Multi‐organ procurement is a risk factor for contamination of preservation fluid with intestinal flora including fungi (e.g., Candida). Transmission of fungal species to the graft vessel can cause mycotic arteritis. This is a very rare but life‐threatening complication of renal transplantation. We present 2 cases of renal transplant recipients from the same multi‐organ donor. Both recipients suffered from severe hemorrhages from renal graft anastomosis and renal artery pseudoaneurysm due to Candida albicans arteritis (CAA). The culture of the preservation fluid revealed growth of Escherichia coli, but neither preservation fluid nor multiple routine blood cultures performed before hemorrhagic complications revealed fungal growth (media non‐selective for fungal growth were applied). The first recipient suffered from sudden severe hemorrhage in the area of graft anastomosis on day 10 post surgery (without any preceding clinical or radiological symptoms). This led to urgent surgery and graftectomy, which was complicated by cardio‐respiratory arrest with resuscitation in the operating room; despite resuscitation, irreversible brain damage, and subsequent death occurred in the intensive care unit (ICU) 2 weeks later (on day 24 after transplantation). The second patient underwent urgent vascular surgery on day 22 (after transplantation), because of hemorrhage from a pseudoaneurysm of the graft artery. She required repeated vascular operations, extended antimicrobial and antifungal therapy, and ICU monitoring and, despite these interventions, she died on day 80 after transplantation as a result of Pseudomonas aeruginosa sepsis. Arteritis of the renal artery in both patients was caused by C. albicans. This was confirmed by histopathology: infiltration of renal artery with budding yeast forming pseudohyphae (Case 1), and the presence of C. albicans in the culture of the renal artery and surrounding tissue (Case 2). We conclude that organ preservation solution should be cultured with use of media selective for fungal growth. As soon as the positive culture is detected, appropriate measures protecting patients against CAA should be undertaken.     

Primary CNS angiitis presenting as short‐term memory loss: a case report and literature review

Primary angiitis of the central nervous system (PACNS) is an idiopathic vasculitis of the small to medium vessels that often eludes diagnosis because of its variable signs and symptoms. A 36‐year‐old woman presented with a 4‐week history of progressive memory loss. Neurological examination revealed only severe cognitive deficits including anterograde amnesia. Magnetic resonance imaging demonstrated multiple ring‐enhancing lesions involving the left frontal lobe and uncus and bilateral thalami. Stereotactic biopsy showed findings consistent with CNS angiitis. Further workup revealed no evidence of systemic disease. We report the first case of biopsy‐proven PACNS presenting as profound isolated anterograde amnesia.     

Human pythiosis, a rare cause of arteritis: case report and literature review

Objective: To present a case of Pythium insidiosum arteritis and review reported cases of human pythiosis.Methods: Search of MEDLINE (PubMed) and the Thai Index Medicus was performed using the words “pythiosis” and “pythium.” Search of abstracts from a scientific meeting also was performed. Only reported cases in the English or Thai language, with enough clinical information for analysis, were included.Results: There were a total of 32 cases of human pythiosis, with 31 identified in the literature. Twenty-five cases were Thai. Twenty of 21 patients with known occupations were farmers. There was ocular infection in 9 cases, subcutaneous infection in 5 cases, arterial infection in 17 cases, and cardiopulmonary infection in 1 case. Thalassemia was a common associated finding. In addition to systemic antifungal therapy, surgery was required in most cases. The highest mortality rate was seen in the arteritic form, particularly in patients with arterial lesions proximal to the superficial femoral artery.Conclusions: Human pythiosis is rare. Physicians should be suspicious of pythiosis in individuals, particularly farmers in Thailand, who develop nonhealing cutaneous ulcers or keratitis, which do not respond to conventional treatment. P insidiosum infections should be listed as a rare cause of infectious arteritis.     

Takayasu's arteritis: a review of the literature

Takayasu's arteritis is a rare, idiopathic, chronic inflammatory disease with cell-mediated inflammation, involving mainly the aorta and its major branches. It leads to stenosis, occlusion or aneurysmal degeneration of large arteries. The clinical presentation is characterised by an acute phase with constitutional symptoms, followed, months or years later, by a chronic phase in which symptoms relate to fibrosis or occlusion of vessels. Angiography is the gold standard for diagnosis and for topographical classification and it correlates with symptoms and prognosis. Here we focus on the pathophysiology, clinical and angiographical classification, diagnostic assessment and therapeutic approach of Takayasu's arteritis.     

A case of Takayasu arteritis complicated with glomerulonephropathy mimicking membranoproliferative glomerulonephritis: a case report and review of the literature

In this report, we describe the case of a 50-year-old Japanese woman with Takayasu arteritis who developed severe proteinuria and renal dysfunction. Abdominal computed tomography did not show narrowing of both renal arteries. Although her levels of C-reactive protein were negative, plasma vascular endothelial growth factor (VEGF) and serum interleukin (IL)-6 levels were elevated. Renal biopsy showed glomerulonephropathy mimicking membranoproliferative glomerulonephritis (MPGN) with glomerular capillary wall thickening (double contour). This was accompanied by mesangial cell proliferation and moderate increase of mesangial matrix without deposits of C3. These findings are quite different from MPGN as electron microscopy did not show subendothelial deposit and circumferential mesangial interposition. Here, we present the case of Takayasu arteritis associated with MPGN-like renal manifestation and elevated VEGF and IL-6. The presence of elevated VEGF and IL-6 could be factors that might contribute to MPGN-like appearance.     

Progressive bilateral lipoma arborescens of the knee complicated by juvenile spondyloarthropathy: A case report and review of the literature

Objectives

To report an unusual case of lipoma aborescens (LA) presented in a patient with treatment-responsive juvenile spondyloarthropathy (JSPA) and to summarize the clinical manifestations, therapy and prognosis of LA by literature review.

Methods

We report an atypical case of a 17-year-old patient with an initial presentation of juvenile spondyloarthropathy, whose inflammatory condition was improved successfully by traditional anti-rheumatic drugs and an anti-TNF alpha agent but developed progressive swelling of bilateral knees. Lipoma arborescens were diagnosed in each knee by synovial biopsy obtained by arthroscopic surgery. Fifty-one cases of LA have been reported and are reviewed in detail.

Results

Clinically, LA could present as monoarthritis or oligoarthritis. The lateral compartment of the knee is the most common site of involvement. Several cases were reported as a comorbidity of inflammatory diseases, but were not improved by anti-inflammatory therapy. Most patients were diagnosed by classic MRI and biopsy findings. The lesions can be managed by open or arthroscopic surgery, but a minority of the cases may have reoccurrence in the same or opposite joint.

Conclusions

LA is a very rare lesion of the synovial and bursal tissue with an unknown etiology. It is considered to be a benign proliferation of the synovial fat associated with trauma, degenerative or inflammatory conditions. LA should be considered as a secondary or comorbid condition in inflammatory arthropathies if other joints respond well to intensive therapy and one or more do not.     

Churg–Strauss syndrome: a retrospective study of 11 cases from a single center in Japan

Objective: To investigate the clinical characteristics of patients with Churg–Strauss syndrome (CSS), including symptoms, blood chemistry and immunological findings. Patients and methods: We retrospectively investigated the records of 11 patients (six female and five male) with CSS admitted to our hospital from September 2003 to October 2009. Results: Eight patients had preceding symptoms including bronchial asthma and allergic rhinitis. Seven patients showed eosinophilia. Nine patients had mononeuritis multiplex. Positive findings of myeloperoxidase‐antineutrophil cytoplasmic antibody (MPO‐ANCA) were found in five patients. Neither clinical manifestations nor laboratory findings were correlated with positivity for MPO‐ANCA. However, the MPO‐ANCA‐positive group showed a higher level of blood urea nitrogen and proteinuria than those negative for MPO‐ANCA. Ten patients recovered after starting steroid or immunosuppressive therapy, although one patient died of unknown etiology. Conclusion: Although general assessments based on various factors such as medical history, clinical manifestation and laboratory studies are indispensable in CSS, MPO‐ANCA might be useful as a predictor of renal dysfunction in patients with CSS.