A case of polyangiitis overlap syndrome]

A 25 year old man was admitted to our hospital on June 1, 1989 because of general fatigue and pleural effusion. He had noticed purpuras and nodules on his bilateral lower legs in July 1988. He was admitted to Nihon University Hospital and was diagnosed as allergic granulomatous angiitis. Methylprednisolone was administered. In March, 1989, a nodular shadow was detected in his lung CT films. From the findings of TBLB a granulomatous lesion was suspected. However, his clinical course was rather stable so he was discharged from the hospital. Two months later, he was suffered from fever and general fatigue. On his chest X ray film pleural effusion was detected in the lower part of his left lung. The nodular shadow was increasing gradually. Laboratory findings on his admission showed leukocytosis, thrombocytosis, elevated CRP and a high titer of RA factor. He was diagnosed as hypersensitivity angiitis in a broad sense based on his biopsy findings of the skin lesion. Furthermore, the diagnosis of an early stage of Wegener's granulomatosis (WG) or limited type of WG was also made from the clinical course of his lung lesion. The combination therapy with cyclophosphamide and methylprednisolone was started. It was so effective that the nodule of his left lung was almost disappeared. It seems that this case is one of the polyangiitis overlap syndrome proposed by Fauci.     

A case of Wegener's granulomatosis with necrotizing granuloma limited to intraorbital lesions]

A patient of Wegener's granulomatosis (WG) who developed intraorbital tumor without pulmonary and renal involvement was reported. A 52-year-old male has suffered from visual disturbance and right proptosis. Administration of betamethasone resulted in a temporary relief of his symptoms. However, following the reduction of steroid dosage, severe recurrence of ocular symptom occurred and his right sight was completely lost. On admission to our hospital, there were swollen eyelids and saddle nose. The CT scan demonstrated intraorbital tumors bilaterally associated with chronic sinusitis. The repeated biopsies of nasal mucosal lesions disclosed presence of a giant cell granuloma with necrotizing vasculitis. A diagnosis of WG had been made, so intermittent high-dose intravenous cyclophosphamide therapy were initiated. The size of the bilateral intraorbital tumors reduced with continuous cyclophosphamide therapy. At the last observation during 22 months after the initiation of cytotoxic therapy, there was no evidence of clinical and radiological recurrence. Three cases of WG with intracranial tumors in our hospital showed multiple cranial nerve palsy and poor response to corticosteroid therapy. The "limited form" without renal involvement has been known as a subset of WG with a relatively good prognosis. This report suggest the existence of further limited form with intracranial granuloma as a cardinal presentation of WG. Although the extent of granulomatous lesions are limited and progression is slow, the treatment by corticosteroid alone is not sufficient and as the systems form of WG, combined therapy with cyclophosphamide appeared to be indicated.     

Bronchopulmonary manifestations of Horton's disease. Apropos of a case

A 78 years old woman developed a pulmonary recurrence four years after the diagnosis of temporal arteritis. The lung biopsy showed the same giant-cells granulomatous lesions with central vessels as the initial biopsy. The recurrence in giant cells-arteritis has already been published but the pulmonary involvement had never been illustrated before by histological data. The difficulties of diagnosis in case of granulomatous angiitis on lung samples is emphasized.     

The lung in systemic vasculitis

Pulmonary vasculitis is usually caused by one of three disorders: (1) Wegener's granulomatosis (WG); (2) Churg-Strauss syndrome (CSS), or allergic angiitis and granulomatosis; or (3) a nonspecific small vessel systemic necrotizing vasculitis (SNV), or microscopic polyarteritis. WG, the most common cause of lung vasculitis, has features of a granulomatous vasculitis of the upper airway and lung and widespread small vessel vasculitis involving the kidneys and other organs. The features of pulmonary WG overlap with those of malignancy and infectious granulomatous lung disease; accurate diagnosis generally requires open lung biopsy. CSS is defined by the triad of asthma, eosinophilia, and systemic vasculitis. Easily accessible tissues should be biopsied, but the clinical features are so distinctive that tissue biopsy is not invariably required for diagnosis. CSS must be differentiated from other diseases that cause pulmonary infiltrates with eosinophilia, including infections. Nonspecific SNV causes diffuse alveolar hemorrhage due to pulmonary capillaritis. Concomitant segmental necrotizing glomerulonephritis is almost always present. Diagnosis is made by renal biopsy, compatible extrarenal features, exclusion of nonimmune causes of lung hemorrhage, and exclusion of WG to the extent possible.     

Wegener's granulomatosis: pitfalls in the management of pulmonary disease: A case of Wegener's granulomatosis with a hilar mass

Wegener's granulomatosis (WG) is a systemic, granulomatous vasculitis that typically affects the upper airways, lungs, and, in most cases, the kidneys. Lung involvement occurs in 85% of patients. A classic feature of WG is multiple pulmonary nodules, which frequently cavitate. Hilar adenopathy or mediastinal masses are rare. These atypical pulmonary findings should raise suspicion of diseases other than WG and lead to biopsy with cultures, even when the diagnosis of WG appears to be certain. These guidelines proved to be reliable in a patient with WG in whom a hilar mass was associated with tuberculosis.     

Sinonasal NK/T-cell lymphoma mimicking Wegener's granulomatosis: a case report

In Western population, sinonasal malignant lymphoma is rare and constitutes 1.5% of all non-Hodgkin lymphoma (NHL) and 2.2% of extranodal lymphomas. Wegener's granulomatosis (WG) is the necrotizing vasculitis of small arteries and veins. WG is characterized by granulomatous vasculitis and involves the upper and lower respiratory tract together with glomerulonephritis. But there are some forms of WG named limited WG that involves the upper respiratory tract only without glomerulonephritis and even seronegative without renal involvement. Herein, we present a typical WG with isolated sinonasal tract involvement with clinical, and radiological findings with the final diagnosis of NK/T-cell angiocentric lymphoma by the repeated biopsies. Since both diseases have same clinical and radiological findings differential diagnosis may be difficult.     

Wegener’s Granulomatosis Presenting as Mediastinal Soft Tissue Mass Invading the Tracheal Wall

Wegener’s granulomatosis (WG) is a clinicopathologic entity of unknown cause characterised by a necrotising granulomatous vasculitis affecting multiple organs, especially the upper and lower respiratory tracts, lung and kidney. The lung is the most frequently, and sometimes the only involved organ. Single or multiple nodules, with or without cavitation, are the most common pulmonary manifestations in WG, but mediastinal involvement is atypical. The sole tracheal involvement is rare and hilar and mediastinal involvement has been thought not to be part of the clinical feature. We experienced a rare case of WG presenting as paratracheal mediastinal lesions with tracheal wall invasion, which responded dramatically to corticosteroid treatment. We present this case with a review of the literature. Received: 22 October 1999 / Accepted: 20 April 2000     

Wegener's granulomatosis effectively treated with rituximab: a case study

Wegener's granulomatosis (WG) is a granulomatous disorder associated with systemic necrotizing vasculitis. Wegener's granulomatosis predominantly involves the upper airways, lung and kidneys. The disease is often associated with cytoplasmic antineutrophil cytoplasmic antibodies (cANCA). B lymphocytes are potential cANCA producers and there is an evident correlation between cANCA titre, severity of the disease and response to treatment. Wegener's granulomatosis usually begins with symptoms limited mostly to the upper and/or lower respiratory tracts and may transform into the generalized phase, characterized by systemic necrotizing vasculitis. If left untreated, it can turn fulminant with poor prognosis. The severe form of the disease is usually treated with a combination of cyclophosphamide and corticosteroids. In refractory cases, rituximab that binds to CD20 expressed on B-cells should be considered. We presented a case of a 38-year-old woman with severe form of WG, refractory to standard therapy. Despite the standard treatment with cyclophosphamide and corticosteroids and the addition of infliximab with methotrexate, progression of the disease was observed. Exacerbation affected mainly the lungs and caused the gradual destruction of pulmonary tissue and development of respiratory insufficiency. Rituximab (500 mg) was given intravenously every week in four infusions, causing a partial remission of WG and the arrest of lung deterioration. The following administration of 500 mg was given every two weeks, which induced the remission of WG and enabled the patient to return to her normal activity and work. Such treatment appeared to be successful and prevented severe pulmonary involvement.     

Purely granulomatous Wegener's granulomatosis: a new concept for an old disease

In summary, PGWG corresponds to an early phase of WG, presenting only with extravascular granuloma. Primarily, ENT, eye, or lung tissues are involved, but any organ may be affected. The diagnosis is made by typical palisading granuloma associated with elevated c-ANCA levels in the patients' serum. The concept of an early granulomatous lesion (PGWG) facilitates the early diagnosis of WG and leads to classification of different forms of disease according to the multistep evolution hypothesis: first PGWG, then localized or limited forms of the disease, and ultimately generalized vasculitis with renal involvement (classical WG). The concept of PGWG forms the basis for future therapeutic trials where folate antagonists are restricted to PGWG, while immunosuppressive treatment is required for the other stages of WG.     

Wegener's granulomatosis of the prostate gland

Wegener's granulomatosis (WG) is a systemic granulomatous vasculitis affecting medium and small arteries, venules, and arterioles. The upper and lower respiratory tract and kidney are primarily involved. Patients with classic WG essentially present with upper airway and pulmonary involvement. Renal disease is common. Involvement of other organ systems is also relatively frequent, most often heart, joints, muscles, eyes, skin, and central and/or peripheral nervous system. We present a patient in whom WG was diagnosed primarily because of prostate involvement. This seems to be a rare manifestation.     

Thyroid involvement in Wegener's granulomatosis: a case report

Wegener's granulomatosis is a systemic necrotizing vasculitis affecting small to medium-sized vessels, characterized by involvement of the upper airways, lungs and kidneys. Endocrine involvement, particularly the thyroid gland is extremely rare. We report a 56-year-old woman presenting a limited form of Wegener's granulomatosis with the following clinical manifestations: sinusitis, pulmonary nodules, diabetes insipidus and a cold thyroid nodule. A right hemithyroidectomy was performed and the histopathological examination revealed a necrotizing vasculitis surrounded by granulomatous lesions. She was treated by corticosteroids without immunosuppressive therapy with a favourable outcome.     

Massive intracerebral hemorrhage associated with Wegener granulomatosis

Wegener granulomatosis (WG) is a necrotizing granulomatous vasculitis that predominantly affects airways and kidneys. But central nervous system involvement (7–11%) is an uncommon. Massive ICH may occur in the course of WG, and this serious condition is related with high risk of mortality. Therefore, the new treatment strategies may be considered in addition to classical practices in serious organ involvement and recurrent attack. Here, we present an adult patient with WG whose disease was complicated by a massive intracerebral hemorrhage (ICH), which subsequently led to death.     

Orbital Wegener’s granulomatosis: a case report and review of the literature

Wegener's granulomatosis (WG) is a multisystem granulomatous, necrotizing vasculitis of presumed autoimmune origin that affects small- to medium-sized blood vessels. The respiratory tract and kidneys are typically involved (Gross and Reinhold-Keller, "Clinical features of primary ANCA-associated vasculitis" in Oxford textbook of rheumatology, third edition, 2004). The limited form usually involves the head and neck, lacks renal involvement, and may not progress to generalized disease (Cassan et al., Am. J. Med. 49:366-379, 1970). Ocular involvement, which may be the initial manifestation, is often encountered and can result in significant morbidity and possibly blindness (Pakrou et al., Semin. Arthritis Rheum. 35:284-292, 2006). We report an unusual case of WG presenting as an orbital mass. The diagnostic triad of granulomatous inflammation with multinucleated giant cells, vasculitis, and necrosis was discovered on histopathology (McDonald and Edwards, JAMA 173:1205-1209, 1960).     

Clinical value of gallium-67 scintigraphy in assessment of disease activity in Wegener's granulomatosis

BACKGROUND: Diagnosis of active pulmonary and paranasal involvement in patients with Wegener's granulomatosis (WG) can be difficult. The diagnostic value of gallium-67 scintigraphy in WG is unclear. OBJECTIVE: To evaluate the added diagnostic value of gallium-67 scintigraphy in patients with WG with suspected granulomatous inflammation in the paranasal and chest regions. METHODS: Retrospectively, the diagnostic contribution of chest and head planar gallium scans in 40 episodes of suspected vasculitis disease activity in 28 patients with WG was evaluated. Scans were grouped into normal or increased uptake for each region. Histological proof or response to treatment was the "gold standard" for the presence of WG activity. RESULTS: WG activity was confirmed in 8 (20%) episodes, with pulmonary locations in three, paranasal in four, and both in one (n=7 patients); all these gallium scans showed increased gallium uptake (sensitivity 100%). Gallium scans were negative for the pulmonary area in 23/36 scans (specificity 64%), and negative for paranasal activity in 13/16 scans (specificity 81%) in episodes without WG activity. Positive predictive value of WG activity for lungs and paranasal region was 24% and 63%, respectively, negative predictive value was 100% for both regions. False positive findings were caused by bacterial or viral infections. CONCLUSION: Gallium scans are clinically helpful as a negative scan virtually excludes active WG. Gallium scintigraphy of chest and nasal region has a high sensitivity for the detection of disease activity in WG. However, because of positive scans in cases of bacterial or viral infections, specificity was lower.     

Systemic manifestations of angiitis with asthma and hypereosinophilia. Study of 10 cases

We report 10 cases of systemic manifestations of angiitis with asthma and hypereosinophilia, some of which had the pathologic features of an allergic granulomatous angiitis of Churg and Strauss. We compare these 10 cases with 109 observations previously reported in the literature from which they differ by a high incidence of digestive tract involvement and by a near constant increase of total plasma immunoglobulin E concentration during the evolutive phases of vasculitis. Broncho-alveolar lavage had been performed in 3 patients, showing pulmonary hypereosinophilia. We discuss the clinical, biological and pathogenic features which differentiate this syndrome, particularly from periarteritis nodosa.     

Periaortitis and Aortic Dissection due to Wegener’s Granulomatosis

We describe here a patient with abdominal periaortitis and intramural dissection as early manifestations of Wegener’s granulomatosis (WG). Surgical biopsies taken from the retroperitoneal inflammatory tissue surrounding the aorta showed granulomatous vasculitis. The patient had antiproteinase-3 antibodies and suffered from nasal, pulmonary, nervous and renal WG involvement. Although being a vasculitis of medium size and small vessels, WG should be included in the systemic vasculitides which can give rise to (peri)aortic inflammation. Received: 6 July 1999 / Accepted: 24 September 1999     

A case of Wegener’s granulomatosis with intracranial involvement

We report a case of Wegener’s granulomatosis (WG) associated with intracranial involvement and brain magnetic resonance imaging (MRI) findings. A 44-year-old woman was diagnosed as having WG with involvement of an ear, lungs and kidneys. A brain computed tomography scan showed a space-occupying lesion with a ring-like enhancement in the left temporal lobe. Findings on a brain MRI, different from those characteristic of a brain abscess, supported an intracerebral granulomatous inflammation due to WG. Immunosuppression, which included corticosteroids, was an effective treatment.     

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

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).     

Nasal Wegener’s and skin sarcoid—a rare combination of two granulomatous diseases

Wegener’s granulomatosis (WG) and sarcoidosis are two distinct granulomatous diseases characterized by multisystem involvement. We report a patient who initially presented with symptoms of limited WG predominantly affecting the nose, followed by a facial rash, which was histologically proven to be due to sarcoidosis. The sequential development of these two diseases in one patient is very rare, and to our knowledge, only one such case has been reported in the last 50 years (Am J Kidney Dis 28:893–898, 1996).     

Wegener’s granulomatosis—a rare presentation

Wegener’s granulomatosis (WG) is a necrotizing granulomatous vasculitis involving the nose, paranasal sinuses, lungs, and kidneys. There are two types of WG—systemic, which is characterized by focal segmental necrotizing glomerulonephritis and limited in which the kidneys are spared. Without proper immunosuppression, WG can be aggressive and often fatal. There are very few reports on WG presenting as parotitis and lacrimal gland involvement. We report a lady who presented recurrent parotitis, focal segmental glomerulosclerosis, and orbital cellulitis, in whom the final diagnosis was revealed after an open lung biopsy.