No abstract available.
Mononeuropathies* ; Systemic Vasculitis ; Vasculitis*

Polyarteritis nodosa(PAN), a systemic vasculitis involving multiple organs including the nervous system, requires a long-term glucocorticoid therapy. Deflazacort is a synthetic glucocorticoid, which has been claimed to have less side effects, but its use for vasculitis has never been reported in the literature. We report a case of polyarteritis nodosa presenting with mononeuritis multiplex causing a left foot drop, which was successfully treated with deflazacort.
Foot ; Mononeuropathies ; Nervous System ; Polyarteritis Nodosa* ; Systemic Vasculitis ; Vasculitis

Multiple aneurysms arising from the same intracranial artery are rare. A few cases of multiple aneurysms of the anterior communicating artery(ACoA) have been reported in the literature. The authors present a 60-year-old female patient who had multiple aneurysms on the ACoA with systemic vasculitis. There were four aneurysms around the ACoA region. Two sizeable aneurysms had completely separated necks on each side of the ACoA. The ruptured larger aneurysm projected in an anteroinferior direction, whereas the unruptured smaller one projected superoposteriorly. Both aneurysms were surgically confirmed and clipped. Two bleb-like microaneurysms, located on ACoA and perforating artery each, were coagulated and reinforced.
Aneurysm* ; Arteries* ; Female ; Humans ; Middle Aged ; Neck ; Systemic Vasculitis ; Vasculitis

Henoch-Schönlein purpura (HSP) is a systemic vasculitis involving the small vessels with distinct clinical features. The etiology of HSP is diverse, and viral infection is one of the many predisposing factors. Cytomegalovirus (CMV) infection mostly affects immune-suppressed patients, but rarely patients with normal immunity can also be affected. Authors experienced a case of HSP patient, with underlying small-cell lung cancer (SCLC) with CMV duodenitis. This is a rare case of HSP diagnosed in SCLC patient with predisposing factor of CMV infection.
Causality ; Cytomegalovirus* ; Duodenitis* ; Humans ; Lung Neoplasms ; Purpura* ; Systemic Vasculitis

Churg-Strauss syndrome (CSS), known as eosinophilic granulomatosis with polyangiitis, is a rare type of systemic vasculitis characterized by the presence of asthma, peripheral eosinophilia, and necrotizing vasculitis with eosinophilic infiltration of multiple organs. Approximately 3-4% of all CSS cases are associated with alveolar hemorrhage. Untreated CSS may lead to a poor prognosis, but glucocorticoid and cytotoxic agent treatments may result in clinical remission. The careful diagnosis and understanding of CSS is important for making treatment decisions and providing effective care. Here, we report a case of CSS with diffuse alveolar hemorrhage.
Asthma ; Churg-Strauss Syndrome* ; Diagnosis ; Eosinophilia ; Eosinophils ; Hemorrhage* ; Prognosis ; Systemic Vasculitis ; Vasculitis

We report a case of necrotizing arteritis involving the gallbladder. This case was clinically diagnosed as cholelithiasis with cholecystitis, and necrotizing arteritis was found in the surgically resected specimen. Vascular changes were similar to those seen in classic polyarteritis nodosa, involving medium-sized muscular arteries and characterized by fibrinoid necrosis and panarterial and periarterial inflammation varying from active to resolving stages. Acute cholecystitis is a rare initial clinical manifestation of the systemic vasculitis. If acute cholecystitis is found in the absence of obvious cause, careful examination is essential. Since steroid therapy improves the prognosis in the systemic vasculitis, clinicians and pathologists should be aware of this unusual lesion.
Arteries ; Cholecystitis ; Cholecystitis, Acute ; Cholelithiasis ; Gallbladder* ; Inflammation ; Necrosis ; Polyarteritis Nodosa ; Prognosis ; Systemic Vasculitis ; Vasculitis*

Wegener's granulomatosis (WG) is defined as a granulomatous inflammation of the upper and lower respiratory tract due to a systemic vasculitis. Facial nerve palsy may occur during the course of any granulomatous or vasculitis disease. But very few cases have been reported in which facial nerve palsy is the presenting feature of WG. We recently experienced an interesting case of WG presenting as acute middle ear infection and subsequent bilateral facial palsy.
Ear, Middle ; Earache ; Facial Nerve ; Facial Paralysis ; Inflammation ; Paralysis ; Respiratory System ; Systemic Vasculitis ; Vasculitis ; Wegener Granulomatosis

Churg-Strauss syndrome is one of idiopathic, systemic necrotizing vasculitis characterized by a history of asthma or allergic rhinitis, eosinophilia of peripheral blood and variable symptoms of systemic vasculitis. There have been several recent case reports of Churg-Strauss syndrome in association with leukotriene modifier therapy when oral corticosteroids were withdrawn in asthmatic patients. We report a case of a 31-year-old man with steroid-dependent asthma who developed Churg-Strauss syndrome after 9 months of steroid withdrawal period, while he was receiving leukotriene modifier (Pranlukast).
Adrenal Cortex Hormones* ; Adult ; Asthma ; Churg-Strauss Syndrome* ; Eosinophilia ; Humans ; Rhinitis ; Systemic Vasculitis ; Vasculitis

The systemic vasculitides are a group of diverse diseases characterized by blood vessel inflammation. The existing classification criteria are intended to create homogeneous patient groups for research and not to diagnose individual patients. However, they have been misused as diagnostic criteria, in both practice and research. The existing classification systems for vasculitis are limited by the overlapping features of disease entities and unrecognized pathogenic mechanisms. This review discusses the benefits and limitations of the widely used American College of Rheumatology criteria and Chapel Hill Consensus Conference nomenclature, updated in 2012. Improved diagnostics, including antineutrophil cytoplasmic antibody (ANCA) testing and imaging, argue for updating the established classification criteria. International efforts are underway to build a more effective classification and diagnostic criteria that reflect a better understanding of the pathophysiology of vasculitis and recent discoveries of genetics and biomarkers.
Antibodies, Antineutrophil Cytoplasmic ; Biomarkers ; Blood Vessels ; Classification* ; Consensus ; Genetics ; Humans ; Inflammation ; Rheumatology ; Systemic Vasculitis ; Vasculitis*

Although digital gangrene is an uncommon clinical feature, it may lead to serious complications, such as amputation. Therefore, it requires prompt evaluation and treatment. Digital gangrene is often seen in systemic sclerosis, but is rare in systemic lupus erythematosus (SLE). In SLE, digital gangrene results from vasculitis, vasospasm, and thromboembolism. Here, we report a 15-year-old male SLE patient who initially presented with digital gangrene, and present a review of the relevant literature.
Amputation ; Gangrene ; Humans ; Lupus Erythematosus, Systemic ; Male ; Scleroderma, Systemic ; Thromboembolism ; Vasculitis