1/54. Cutaneous extravascular necrotizing granuloma (Churg Strauss granuloma).Churg Strauss granuloma (cutaneous extravascular necrotizing granuloma) is a distinct entity which is associated with systemic immunoreactive or autoimmune diseases in a majority of cases. Typically, Churg Strauss granuloma presents as symmetrical papules or nodules on the extremities. There are two histological patterns: the classic pattern reveals palisading granuloma with central degenerated collagen, interspersed polymorphonuclear leukocytes, and leukocytoclastic debris; the focal basophilic necrosis pattern does not show palisading granuloma. We report two cases of Churg Strauss granuloma with different histopathological patterns.- - - - - - - - - - ranking = 1keywords = necrotizing (Clic here for more details about this article) |
2/54. Childhood churg-strauss syndrome.churg-strauss syndrome or allergic granulomatosis and angiitis is a vasculitis that is found in adults, but is extremely rare in children. We describe a 14-year-old boy who presented with prolonged fever, weight loss, sinusitis, myalgia and arthralgia, testicular pain, pulmonary infiltrations, pericardial effusion, peripheral neuropathy, and eosinophilia. Muscle biopsy showed necrotizing arteritis with eosinophil infiltration. His clinical course was complicated by several seizures secondary to cerebral vasculitis and severe asthma, resulting in death. The clinical features and outcomes of childhood churg-strauss syndrome are reviewed.- - - - - - - - - - ranking = 0.31709594152871keywords = necrotizing, arteritis (Clic here for more details about this article) |
3/54. IgA glomerulonephritis associated with microscopic polyangiitis or Churg-Strauss syndrome.When renal insufficiency occurs in classical antineutrophil cytoplasm antibody- (ANCA) associated vasculitides, histological examination usually finds pauci-immune focal segmental glomerulonephritis. We report on 2 cases of histologically proven necrotizing vasculitis associated with IgA nephropathy. Concomitant vasculitis and IgA nephropathy has only rarely been reported but this joint occurrence may not be coincidental as its pathophysiology is not known. Among vasculitides, IgA nephropathy has more frequently been associated with Henoch-Schoenlein purpura: one microscopic polyangiitis unusual because the patient simultaneously presented ANCA and microaneurysms, and the other churg-strauss syndrome associated with mild renal insufficiency. This uncommon association might represent a possible overlap syndrome between these ANCA-associated vasculitides and IgA nephropathy or simply a new type of glomerulonephritis that must be taken into account in these vasculitides.- - - - - - - - - - ranking = 0.2keywords = necrotizing (Clic here for more details about this article) |
4/54. Simultaneous presence of C-ANCA and P-ANCA in a patient with concurrent churg-strauss syndrome and giant cell temporal arteritis.We herein describe the case of a 77-year-old woman, who presented clinical and histopathological evidence of giant cell arteritis (GCA) involving the temporal artery, together with a churg-strauss syndrome (CSS). Our patient presented positive anti-neutrophil cytoplasmic antibodies (ANCA), with cytoplasmic staining pattern (C-ANCA) that was specific against proteinase 3 (PR3), and also a perinuclear pattern (P-ANCA) with specificity against myeloperoxidase (MPO). To our knowledge, the simultaneous presence in the same patient of both types of antibodies has not been previously reported.- - - - - - - - - - ranking = 0.58547970764354keywords = arteritis (Clic here for more details about this article) |
5/54. hepatitis c virus infection with and without cryoglobulinemia as a case of churg-strauss syndrome.Chronic hepatitis c virus (HCV) infection may be associated with numerous immune disorders, with vasculitis including polyarteritis nodosa, or with both. cryoglobulinemia, which is often present, can also be expressed by vasculitis. We describe 2 cases of churg-strauss syndrome (CSS) in patients with HCV infection. We found no previous case of CSS accompanying HCV infection in the literature. The current patients were women aged 40 and 66 years. In both cases, a clinical and laboratory pattern suggesting CSS was found before the HCV infection was discovered. One patient had cryoglobulinemia. One patient was successfully treated with interferon (IFN). The other was treated for 18 months with IFN and corticosteroids. Second-line therapy consisting of IFN with ribavirin was successful. The emergence of HCV infection may have led to an induced form of CSS. The relationship among HCV, cryoglobulinemia, and CSS is not clear, but may be similar to that existing between polyarteritis nodosa and hepatitis b virus. These observations suggest that IFN-alpha therapy may be effective against CSS in HCV infected patients with or without cryoglobulinemia.- - - - - - - - - - ranking = 5.8889951103962keywords = polyarteritis, polyarteritis nodosa, nodosa, arteritis (Clic here for more details about this article) |
6/54. Severe polyneuropathy in a patient with churg-strauss syndrome.We describe the clinicopathologic features of a 56-year-old woman affected with churg-strauss syndrome with major peripheral nerve involvement. The patient presented with a 1-month history of mainly distal upper-limb symmetrical paresthesias and hypostenia (bilateral "wrist drop"), palpable purpura and eosinophilia. Multiple pulmonary infiltrates and asthma had been present since the age of 52. skin biopsy demonstrated an eosinophilic necrotizing vasculitis. During the hospitalization she was submitted to cardiac, bronchopulmonary, renal, and gastrointestinal evaluation and EMG. Peripheral nerve and skeletal muscle biopsies were performed. sural nerve biopsy showed a marked degree of demyelination. A perivascular cellular infiltrate within the epineurium was immunoreactive for T lymphocytes and macrophages. Strong HLA-DR immunostaining was present in the endoneurium. IgM, IgE and fibrinogen deposition was found in some epi- and endoneurial vessels. Muscle biopsy showed neurogenic changes and 1 thrombosed vessel surrounded by mononuclear cells. Membrane attack complex (MAC) deposition was present in a few capillaries and major histocompatibility complex products I (MHCP I) was expressed at the subsarcolemmal level in a few isolated perivascular muscle fibers. After immunosuppressive therapy, the patient showed progressive improvement of both clinical symptoms and neurophysiological parameters.- - - - - - - - - - ranking = 0.2keywords = necrotizing (Clic here for more details about this article) |
7/54. A variant form of churg-strauss syndrome: initial temporal non-giant cell arteritis followed by asthma--is this a distinct clinicopathologic entity?The clinical manifestations of the classical vasculitis syndromes are extraordinarily heterogenous with considerable overlap among them. Recently, several cases of unusual presentation of the vasculitis syndromes have been reported. We describe a patient who initially manifested with temporal arteritis and Raynaud's phenomenon and subsequently developed bronchial asthma, ie, a case of an atypical form of churg-strauss syndrome (allergic angiitis and granulomatosis) and discuss whether this case is a distinct clinicopathological entity.- - - - - - - - - - ranking = 0.58547970764354keywords = arteritis (Clic here for more details about this article) |
8/54. churg-strauss syndrome with pleural involvement.A 51-year-old Japanese man with churg-strauss syndrome (CSS) diagnosed by pleural biopsy is described. He was hospitalized because of high fever and bilateral knee, elbow and shoulder joint pain. Chest roentgenogram and chest computed tomography (CT) scan revealed bilateral massive pleural effusion. Pleural biopsy revealed eosinophilic infiltration and necrotizing granulomas. He was treated with oral prednisolone and his symptoms improved. This is the first report of CSS diagnosed by pleural biopsy.- - - - - - - - - - ranking = 0.2keywords = necrotizing (Clic here for more details about this article) |
9/54. Acral purpuric plaques in a woman with asthma: a case of allergic granulomatosis angiitis.Allergic granulomatosis angiitis (AGA) is a rare systemic vasculitis of unknown etiology. Most patients are adults in their third to fourth decade of life. The combination of asthma, eosinophilia, and necrotizing vasculitis is almost invariably present. Cutaneous lesions are found in up to 70% of the patients and include nodules, hemorrhagic lesions, and erythema multiformelike lesions. We provide a case report of a 30-year-old woman with asthma who presented with acral purpuric plaques and was diagnosed with AGA.- - - - - - - - - - ranking = 0.2keywords = necrotizing (Clic here for more details about this article) |
10/54. Childhood churg-strauss syndrome: report of a case.churg-strauss syndrome (CSS) (allergic granulomatosis and angitis) is an uncommon form of systemic vasculitis, which is rare in children. It is characterized by peripheral blood hypereosinophilia, systemic necrotizing vasculitis, and a preceding history of bronchial asthma. We described a boy with initial presentation of poorly controlled bronchial asthma, allergic rhinitis, recurrent sinusitis and several episodes of hemoptysis since the age of 9. He then developed purpuric skin lesions, generalized soreness, and symptoms of mononeuritis multiplex at age 11. On admission to our hospital at the age of 12, he developed marked pericardial effusion. After a series of studies including chest computed tomography (CT), skin biopsy, nerve conduction study, and serological tests for autoantibodies, CSS was diagnosed. Thereafter, he received regular corticosteroid therapy, and his symptoms were generally well-controlled with occasional acute exacerbation. The clinical characteristics, diagnosis and management of CSS in children are also reviewed.- - - - - - - - - - ranking = 0.2keywords = necrotizing (Clic here for more details about this article) |
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