Microscopic Polyangiitis Presenting Research Articles

Microscopic Polyangiitis Presenting with Acute Abdominal Pain Due to Rare Small Intestine Involvement

Microscopic Polyangiitis Presenting with Acute Abdominal Pain Due to Rare Small Intestine Involvement

MICROSCOPIC POLYANGIITIS PRESENTING WITH CARDIAC TAMPONADE

MICROSCOPIC POLYANGIITIS PRESENTING WITH CARDIAC TAMPONADE

Microscopic Polyangiitis Presenting as Pyrexia of Unknown Origin with Neurological Symptoms and a Management Dilemma

Microscopic Polyangiitis Presenting as Pyrexia of Unknown Origin with Neurological Symptoms and a Management Dilemma

Microscopic polyangiitis presenting as acute flaccid paralysis mimicking Guillain–Barre syndrome

Microscopic polyangiitis presenting as acute flaccid paralysis mimicking Guillain–Barre syndrome

Microscopic polyangiitis presenting with persistent cough and hemoptysis in pediatrics: a case report and review of the literature

Microscopic polyangiitis presenting with persistent cough and hemoptysis in pediatrics: a case report and review of the literature

212 Microscopic Polyangiitis Presenting as Nephrotic Syndrome with Bilateral Pulmonary Emboli

212 Microscopic Polyangiitis Presenting as Nephrotic Syndrome with Bilateral Pulmonary Emboli

A case of microscopic polyangiitis presenting with acute spinal subdural hemorrhage.

This report describes a case of a 62-year-old woman with microscopic polyangiitis (MPA) who developed acute spinal subdural hemorrhage. MPA was confirmed by positive autoantibodies to myeloperoxidase and focal segmental necrotizing and pauci-immune crescentic glomerulonephritis on renal biopsy. She did not recover from paraplegia due to acute spinal subdural hemorrhage, despite decompression operation and aggressive immunosuppression. Although spontaneous spinal hemorrhage in MPA patients is very rare, the prognosis for such patients is poor. Considering the possibility of ongoing vasculitis activity in extra-renal organs, clinicians should be very cautious to attenuate the strength of immunosuppressant drugs, even in patients with chronic or irreversible renal pathology.

Renal Involvement With Microscopic Polyangiitis Presenting as a Febrile Illness With a Persistently Normal Urine Analysis

Renal Involvement With Microscopic Polyangiitis Presenting as a Febrile Illness With a Persistently Normal Urine Analysis

Microscopic Polyangiitis: Atypical Presentation With Extensive Small Bowel Necrosis, Diffuse Alveolar Hemorrhage, and Renal Failure

Microscopic polyangiitis is an uncommon systemic vasculitis of varying severity that is associated with myeloperoxidase (MPO) and perinuclear antineutrophil cytoplasmic (p-ANCA) antibodies. The most commonly affected organs are the lungs and kidneys. We report on a very unusual case of microscopic polyangiitis presenting with severe mesenteric ischemia in addition to diffuse alveolar hemorrhage and acute renal failure. The patient was initially diagnosed with acute pancreatitis at an outside facility given his severe abdominal pain and elevated pancreatic enzymes. Further investigations after transfer to our facility determined that the patient was actually suffering from a severe exacerbation of previously diagnosed microscopic polyangiitis. He quickly developed diffuse alveolar hemorrhage (DAH) necessitating intubation and acute kidney injury (AKI) requiring dialysis. He subsequently developed mesenteric ischemia and bowel necrosis resulting in emergent laparotomy and extensive small bowel resection. Physicians need to be aware that microscopic polyangiitis can very rarely present with severe involvement of the abdominal viscera and mesenteric vessels. Severe disease necessitates the use of high dose IV steroids, rituximab or cyclophosphamide, and plasma exchange (PLEX).

Pulmonary Limited MPO-ANCA Microscopic Polyangiitis and Idiopathic Lung Fibrosis in a Patient with a Diagnosis of IgA Nephropathy.

We present a case of a male patient with chronic renal insufficiency, due to crescentic glomerulonephritis with IgA deposits, who successively developed (idiopathic) thrombocytopenic purpura (ITP) and MPO-ANCA microscopic polyangiitis (MPA) with pulmonary fibrosis. The patient presented with cough, weight loss, and dyspnea on exertion. CT imaging and pulmonary function tests were compatible with interstitial pneumonitis with pulmonary fibrosis. Laboratory results showed high MPO-ANCA titers; the urinary sediment was bland. The patient was treated successfully with cyclophosphamide and methyl-prednisolone. This unique case illustrates the diagnostic and therapeutic challenges of an unusual presentation of microscopic polyangiitis presenting first as isolated kidney disease with recurrence in the form of pneumonitis without renal involvement, in association with renal IgA deposits and ITP as coexisting autoimmune conditions.

An Interesting Case of Microscopic Polyangiitis Presenting as Rapidly Progressive Pulmonary Fibrosis

An Interesting Case of Microscopic Polyangiitis Presenting as Rapidly Progressive Pulmonary Fibrosis

Occult microscopic polyangiitis presenting as pyrexia of unknown origin

Occult microscopic polyangiitis presenting as pyrexia of unknown origin

Microscopic polyangiitis presenting with peripheral and central neurological manifestations

Although peripheral neuropathy is a common complication of microscopic angiitis, manifestations involving the muscle and the central nervous system have been rarely reported. We describe a 48-year-old man who rapidly developed a clinical picture of mononeuritis multiplex. A month after the appearance of the primary symptoms, he became comatose and had left hemiplegia in relation with a massive cerebral haematoma. Laboratory data revealed signs of inflammation, glomerular dysfunction with microhaematuria, and positive myeloperoxidase-antineutrophil cytoplasmic antibodies. The neuromuscular biopsy disclosed a small-vessel vasculitis, consisting with microscopic angiitis, associated with myositis and extensive axonal loss. The patient had surgical evacuation of the haematoma and received immunosuppressive therapy with good outcome. Thus, microscopic angiitis should be considered as a differential diagnosis in cases of myositis and intracerebral haemorrhage.

Microscopic polyangiitis presenting with medullary infarct

Microscopic polyangiitis is a small vessel vasculitis which is rarely associated with ischemic stroke. Cerebrovascular disease has rarely been reported in connection with this disease. It may cause fatal hemorrhage, hemorrhagic conversion and multiple lacunar infarcts. We report here a 55-year-old woman with left medullary oblangata infarction without any symptoms of microscopic polyangiitis. During hospitalization, retinal ischemia, mononeuritis multiplex and pulmonary infiltration developed. Sural nerve biopsy was concomitant with small vessel vasculitis. Elevated CRP and sedimentation and positive P-ANCA led to confirmation of a diagnosis of microscopic polyangiitis. Our patient is a rare case of microscopic polyangiitis presenting with medullary infarction. Although the characteristics of this disease are well-known, the first symptom can be a medullary infarction, which has not been reported in literature before.

Unusual Serological Presentation of Microscopic Polyangiitis Presenting as Diffuse Alveolar Hemorrhage

Unusual Serological Presentation of Microscopic Polyangiitis Presenting as Diffuse Alveolar Hemorrhage

Microscopic Polyangiitis Presenting as Early Cardiac Tamponade

Microscopic Polyangiitis Presenting as Early Cardiac Tamponade

Microscopic polyangiitis presenting with capsular warning syndrome and subsequent stroke

A 55-year-old man developed ischemic stroke after three episodes of transient dysarthria and left hemiplegia, a typical manifestation of capsular warning syndrome. Magnetic resonance imaging of the brain showed bilateral basal ganglionic infarction. The patient had no significant risk of stroke. However, the systemic manifestations, an elevated titer of perinuclear anti-neutrophilic cytoplasmic antibody and a skin biopsy revealing leukocytoclastic venulitis confirmed the undrlying microscopic polyangiitis.

Microscopic polyangiitis presenting as a “pulmonary‐muscle” syndrome: Is subclinical alveolar hemorrhage the mechanism of pulmonary fibrosis?

Microscopic polyangiitis (MPA) may present with a syndrome that resembles idiopathic pulmonary fibrosis (IPF). We describe an MPA patient with the clinical presentation of a "pulmonary-muscle" syndrome in which interstitial lung disease antedated the onset of myopathy. Identification of vasculitis on muscle biopsy was instrumental in recognizing clinical, radiographic, and histopathologic features that were more characteristic of MPA than of IPF. Institution of glucocorticoid and cyclophosphamide therapy led to the induction of a complete remission. The histologic findings in this case implicate subclinical episodes of alveolar hemorrhage as the mechanism of interstitial lung disease in MPA.