A case of Sjögren syndrome with pulmonary amyloidosis complicating microscopic polyangiitis

Abstract

We present the case of a 71-year-old Japanese woman with Sjögren syndrome complicated with pulmonary amyloidosis who developed microscopic polyangiitis. She was admitted because of fever, weight loss, and paresthesia of the plantae and fingers. Chest computed tomography revealed bilateral multiple nodules with calcification in the lung. Transbronchial lung biopsy specimens showed AL-type (primary) amyloid deposits. The patient also tested positive for myeloperoxidase-antineutrophil cytoplasmic antibodies (MPO-ANCA), with changes in the MPO-ANCA titer that paralleled changes in the symptoms. Nerve conduction velocity findings showed mononeuropathy multiplex. Livedo vasculitis was observed on bilateral lower legs, and skin biopsy showed leukoclastic vasculitis. We diagnosed the patient as having microscopic polyangiitis.     

Microscopic polyangiitis associated with diffuse panbronchiolitis

There are several case reports of systemic vasculitis associated with chronic suppurative lung diseases. We describe a 46-year-old female, previously diagnosed as having diffuse panbronchiolitis (DPB), presenting with hemosputum and dyspnea. Her serum titer of MPO-ANCA was positive together with a high titer of BPI-ANCA. Chest X-ray and chest CT scan showed pulmonary hemorrhage, and the renal biopsy specimen revealed necrotizing, crescentic glomerulonephritis. She was diagnosed as having ANCA-associated vasculitis, and more specifically, microscopic polyangiitis accompanied by DPB. She was treated with methylprednisolone pulse therapy, followed by intravenous cyclophosphamide. This case suggested a possible association with chronic bacterial infection, which may play a role in the pathogenesis of ANCA-associated vasculitis.     

A case of Sjögren syndrome with pulmonary amyloidosis complicating microscopic polyangiitis

We present the case of a 71-year-old Japanese woman with Sjögren syndrome complicated with pulmonary amyloidosis who developed microscopic polyangiitis. She was admitted because of fever, weight loss, and paresthesia of the plantae and fingers. Chest computed tomography revealed bilateral multiple nodules with calcification in the lung. Transbronchial lung biopsy specimens showed AL-type (primary) amyloid deposits. The patient also tested positive for myeloperoxidase-antineutrophil cytoplasmic antibodies (MPO-ANCA), with changes in the MPO-ANCA titer that paralleled changes in the symptoms. Nerve conduction velocity findings showed mononeuropathy multiplex. Livedo vasculitis was observed on bilateral lower legs, and skin biopsy showed leukoclastic vasculitis. We diagnosed the patient as having microscopic polyangiitis.     

Pachymeningitis in microscopic polyangiitis (MPA): a case report and a review of central nervous system involvement in MPA

A case of microscopic polyangiitis (MPA) with pachymeningitis is described. The patient had renal, skin, gallbladder and peripheral nervous system involvement, simultaneously with pachymeningitis. Necrotizing glomerulonephritis with crescent formation, and necrotizing small vessel vasculitis in the kidney and skin were confirmed by biopsy. A highly elevated titer of antineutrophil cytoplasmic antibody for myeloperoxidase (MPO-ANCA) was observed. All of the clinical and laboratory abnormalities improved with high-dose pulse and conventional steroid therapy. The literature on central nervous system involvement in MPA and perinuclear-ANCA (p-ANCA)-related vasculitis is reviewed. This case serves to emphasize that pachymeningitis can occur as one of the features of MPA.     

A concomitant case of giant cell arteritis and microscopic polyangiitis with hemoperitoneum by rupture of the gastroepiploic artery

Abstract

Giant cell arteritis (GCA) mainly involves large-sized arteries, while microscopic polyangiitis (mPA), characterized by pauci-immune necrotizing vasculitis, mainly affects small-sized vessels. We report a very rare concomitant case of GCA diagnosed by temporal artery biopsy and mPA with a high titer of myeloperoxidase antineutrophil cytoplasmic antibody, exacerbation of interstitial pneumonia, and suspected rapidly progressive glomerulonephritis. The patient died by sudden rupture of the gastroepiploic artery (medium-sized vessel), which may have been triggered by GCA and/or mPA.     

A case of myeloperoxidase–antineutrophil cytoplasmic antibody positive-polyarteritis nodosa complicated by interstitial pneumonia and rapidly progressive renal failure

A 73-year-old woman was admitted to our hospital because of persistent high fever and cough, generalized myalgia, and renal dysfunction. Laboratory examination revealed severe inflammatory signs, pulmonary fibrosis, progression of renal impairment with active nephritic urinary sediments, and a high titer of myeloperoxidase-antineutrophil cytoplasmic antibody, indicating that she might have microscopic polyangiitis with interstitial pneumonia and rapidly progressive glomerulonephritis. Her renal biopsy, however, showed tubulointerstitial changes with mild glomerular abnormalities, and renal angiography revealed that she had vascular lesions of medium-sized arteries, which were compatible with classical polyarteritis nodosa. Tissue biopsy of the clinically affected organ should be considered in anyone suspected to have vasculitis.     

ANCA-associated vasculitis in idiopathic pulmonary fibrosis: A case report and brief review of the literature

Rationale:Idiopathic pulmonary fibrosis (IPF) is a progressive disease with poor prognosis. Patients with IPF represent a heterogeneous population with several described clinical phenotypes. More recently, the development of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis in IPF patients, with an incidence higher than that in the general population, has drawn attention.Patient concerns:A 64-year-old woman previously diagnosed with IPF presented to the emergency department with hemoptysis and hypoxemic respiratory failure.Diagnoses:High-resolution chest computed tomography revealed bilateral ground-glass opacities associated with areas of consolidation superimposed on the patient''s fibrotic background pattern. Diffuse alveolar hemorrhage was confirmed by the presence of hemorrhagic bronchoalveolar lavage fluid. Hematological and biochemical investigations revealed an inflammatory syndrome, moderate anemia, and rapidly progressive glomerulonephritis. Serological analysis revealed perinuclear antineutrophil cytoplasmic antibody positivity and high levels of antimyeloperoxidase antibodies antibodies. The patient underwent kidney biopsy, which revealed necrotizing glomerulonephritis. Clinical and laboratory findings were diagnostic of microscopic polyangiitis with lung and renal involvement.Interventions:Cyclophosphamide in combination with methylprednisolone was administered as remission induction therapy. The maintenance therapy consisted of mycophenolate mofetil and prednisone.Outcomes:The patient achieved clinical, radiological, and serological remission within six weeks of treatment.Lessons:The association between IPF and ANCA-associated vasculitis may represent a distinct clinical phenotype. Autoimmune testing for ANCAs should be considered part of the diagnostic work-up and follow-up of patients with IPF because of the clinical and therapeutic implications of developing vasculitis in an already vulnerable patient.     

Culture-negative subacute bacterial endocarditis masquerades as granulomatosis with polyangiitis (Wegener’s granulomatosis) involving both the kidney and lung

Background

Subacute bacterial endocarditis (SBE) occasionally exhibits positive cytoplasmic anti-neutrophil cytoplasmic antibody (c-ANCA) of the anti-proteinase-3 (PR-3) type. Clinically, it mimics ANCA-associated vasculitis, such as Wegener's disease with glomerulonephritis. Lung abscesses are the most common manifestation of lung involvement. We herein report a case of culture-negative SBE strongly c-ANCA/PR3-positive accompanied by pulmonary involvement and glomerulonephritis. In this case, we took biopsies of both the lung and kidney, although renal biopsy is usually preferred over lung biopsy. The lung biopsy showed severe alveolar capillaritis, suggesting vasculitis consistent with polyangiitis. The renal biopsy revealed glomerulonephritis with a membranoproliferative pattern. To our knowledge, this is the first such reported case.

Case presentation

A 68-year-old Chinese male patient presented to our hospital with a fever, cough, chest pain, and recurrent peripheral edema. He had a past medical history significant for treated schistosomiasis 20 years previously. Physical examination revealed palpable purpura, mild hypertension, hepatosplenomegaly, and a holosystolic cardiac murmur (Levine 2/6). Echocardiography showed tricuspid valve vegetations with moderate to severe regurgitation. Serum c-ANCA/PR3 and cryoglobulin were strongly positive. Renal biopsy results indicated membranoproliferative glomerulonephritis with several crescents. Chest CT revealed multiple intraparenchymal and subpleural nodules, and lung biopsy showed polyangiitis. The patient’s ANCA titers, glomerulonephritis, and pulmonary injury all resolved after antibiotic therapy.

Conclusion

SBE may present with positive c-ANCA/PR3, multiple pulmonary nodules, pulmonary polyangiitis, and glomerulonephritis clinically mimicking granulomatosis with polyangiitis (Wegener's granulomatosis).

     

Microscopic polyangiitis initiated with liver dysfunction, calf pain and fever of unknown origin

We report herein a case of microscopic polyangiitis (MPA), presenting onset with a spiking fever, liver/biliary dysfunction without jaundice and calf pain without elevation of serum creatine phosphokinase. During 1 month of careful examinations for initial diagnosis, the patient developed renal dysfunction and pulmonary hemorrhage. Based on the results of positive MPO-ANCA, renal and pulmonary involvements, the patient was diagnosed with MPA and treated with high-dose prednisolone and oral cyclophosphamide. Soon after initiation of the treatment, symptoms such as fever, calf pain, liver/biliary dysfunction and renal dysfunction disappeared with decrease of MPO-ANCA titer to the normal level.     

Pancreatic mass as an initial manifestation of polyarteritis nodosa:A case report and review of the literature

Classic polyarteritis nodosa(PAN) that targets mediumsized muscular arteries and microscopic polyangiitis(MPA),characterized by inflammation of small-caliber vessels and the presence of circulating myeloperoxidase anti-neutrophil cytoplasmic antibodies(MPO-ANCA),are distinct clinicopathological entities of systemic vasculitis.A 66-year-old woman presented with fever,cholestasis and positive MPO-ANCA.Radiological examination showed a pancreatic mass compressing the bile duct.Therefore,we performed pancreatoduodenectomy.Histopathological examination revealed that necrotizing vasculitis predominantly affecting the medium-sized vessels,spared arterioles or capillaries in the pancreas,a finding consistent with PAN.Unexpectedly,renal biopsy revealed small-caliber vasculitis and glomerulonephritis,supporting MPA.The initial manifestation of a pancreatic mass associated with vasculitis has only been reported in 7 articles.Its diagnosis is challenging because no reliable clinico-radiological findings have been observed.Clinicians should be aware of such cases and early diagnosis followed by immunosuppression is mandatory.Our findings may reflect a polyangiitis overlap syndrome coexisting between pancreatic PAN and renal MPA.     

Microscopic polyangiitis presenting as idiopathic pulmonary fibrosis: is anti-neutrophilic cytoplasmic antibody testing indicated?

We report a 55-year old woman with microscopic polyangiitis who presented with idiopathic pulmonary fibrosis and 1 year later developed hematuria and proteinuria. She had a high serum level of perinuclear anti-neutrophilic cytoplasmic antibodies. Renal angiogram was normal. The diagnosis of microscopic polyangiitis was confirmed by renal biopsy, which showed pauci-immune crescentic glomerulonephritis. The patient received immunosuppressive therapy and improved markedly. Consideration of small vessel vasculitis is important in the differential diagnosis of idiopathic pulmonary fibrosis.     

A case of interstitial pneumonia preceding microscopic polyangiitis]

We encountered a case of interstitial pneumonia preceding microscopic polyangiitis (MPA). A 64-year-old asymptomatic woman was found to have interstitial pneumonia on a chest radiograph taken during a work-up before an operation for cataract. Six months later she presented with non-productive cough, low grade fever and renal dysfunction. Interstitial pneumonia worsened with new lesions. The test for MPO-ANCA was positive, though it had been negative six months previously. The histopathology of the lung by video-assisted lung biopsy showed the usual interstitial pneumonitis pattern and small vessel pulmonary vasculitis. Renal biopsy showed necrotizing glomerulonephritis. She was given a diagnosis of MPA and was immediately treated with methylprednisolone pulse therapy and a combination of prednisolone and cyclophosphamide. She has remained stable for two years. This positive inversion of MPO-ANCA, while interstitial pneumonia advanced, was of interest concerning the etiology of MPA. We discuss the important topic of the mechanism of the development of MPA.     

An unusual presentation of antineutrophil cytoplasmic antibody-associated vasculitis: discrepancy between histopathology and clinical presentation

A 60-year-old man was admitted to our clinic with dyspnea, hemoptesis, anuria, nephritic syndrome, and a positive myeloperoxidase antineutrophil cytoplasmic antibody titer. He was diagnosed with antineutrophil cytoplasmic antibody-associated vasculitis due to Wegener granulomatosis, microscopic polyangiitis, or drug induction. Unexpectedly, histopathologic examination of the kidney biopsy specimen revealed the diagnosis of noncrescentic and nonnecrotizing glomerulonephritis. We report this case because of the unusual histologic type of renal involvement.     

Microscopic polyangiitis histologically confirmed by biopsy from nasal cavity and paranasal sinuses: a case report

Microscopic polyangiitis (MPA) is a systemic necrotizing vasculitis affecting small vessels without necrotizing granulomatous inflammation and is commonly associated with necrotizing glomerulonephritis. Diagnosis is based on typical clinical features, the presence of antimyeloperoxidase-antineutrophil cytoplasmic antibodies (MPO-ANCA), and histopathologic findings. Cases of pathologically proven small-vessel vasculitis in nasal biopsy specimens are sparse. Here we report a patient with MPA that was histopathologically confirmed by nasal and paranasal biopsy. A 67-year-old man presented with fever and general fatigue. Laboratory examinations showed severe inflammation and acute progressive renal failure. The serum MPO-ANCA level was elevated. The patient also had nasal polyps that seemed to be nonspecific chronic sinusitis. To obtain a pathologic diagnosis, bilateral ethmoidectomy and nasal polypectomy were performed. Pathological findings revealed vasculitis of small vessels in the mucosal surface. MPA was diagnosed on the basis of clinical symptoms, elevated MPO-ANCA and the pathological findings of the nasal and paranasal surgical specimen.     

Pulmonary fibrosis predating microscopic polyangiitis by seven years

A 63-year-old man, ex-smoker with renal failure of recent onset was admitted at the respiratory department with massive haemoptysis. Previous X-rays and CT scans showed pulmonary fibrosis of seven-year duration. Subsequently, he developed high fever, large haemoptysis, new infiltrates and respiratory failure despite broad-spectrum antibiotic treatment. Antineutrophilic antibodies of the perinuclear type with specificity against myeloperoxidase were detected and microscopic polyangiitis was diagnosed. Immunosuppressive treatment with methylprednisolone pulses and cyclophosphamide was started with initially favorable response, but later the patient developed a hospital-acquired pneumonia which was treated successfully with meropenem. As pulmonary haemorrhage recurred, he was transferred to intensive care for plasmapheresis which was considered the last treatment option. Unfortunately he died from septic shock.ConclusionAsymptomatic pulmonary fibrosis can predate microscopic polyangiitis by several years and is associated with unfavorable prognosis of the vasculitis. Appreciation of this finding would lead to faster diagnosis and better management of these patients.     

Rheumatoid arthritis complicated with myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA)-associated vasculitis: a case report

Abstract

This article describes a patient with rheumatoid arthritis (RA) with crescentic glomerulonephritis (CrGN) associated with myeloperoxidase–antineutrophil cytoplasmic antibodies (MPO-ANCA), who responded well to methotrexate (MTX). A 48-year-old woman with a 4-year history of RA was admitted with fever and elevated C-reactive protein. On laboratory evaluation, her level of MPO-ANCA was 422?EU, and urinalysis revealed proteinuria and hematuria. Because she was also suffering from episcleritis, vasculitis was considered. A renal biopsy was performed, which revealed necrotizing CrGN. We diagnosed RA complicated with MPO-ANCA-associated vasculitis. We considered treatment with high-dose oral prednisolone for vasculitis, but the patient refused this treatment. We started MTX at a dose of 8?mg/week for RA from the time of admission, and the patient responded immediately. Biochemical parameters, including C-reactive protein, erythrocyte sedimentation rate, rheumatoid factor, and MPO-ANCA, improved. Seven months later, MPO-ANCA had decreased to 46?EU. In clinical studies, few patients have been reported with RA complicated with ANCA-associated CrGN. This case differs from previous cases in the treatment given. No high-dose steroid with intensive immunosuppression or plasma exchange was required.     

A concomitant case of giant cell arteritis and microscopic polyangiitis with hemoperitoneum by rupture of the gastroepiploic artery

Giant cell arteritis (GCA) mainly involves large-sized arteries, while microscopic polyangiitis (mPA), characterized by pauci-immune necrotizing vasculitis, mainly affects small-sized vessels. We report a very rare concomitant case of GCA diagnosed by temporal artery biopsy and mPA with a high titer of myeloperoxidase antineutrophil cytoplasmic antibody, exacerbation of interstitial pneumonia, and suspected rapidly progressive glomerulonephritis. The patient died by sudden rupture of the gastroepiploic artery (medium-sized vessel), which may have been triggered by GCA and/or mPA.     

A case of ANCA-associated vasculitis with glomerular eosinophilic infiltration: a possible pathogenic implication

We present a 58-year-old male patient with myeloperoxidase antineutrophil cytoplasmic antibody-associated vasculitis with rapidly progressive glomerulonephritis. He failed to fulfill the common American College of Rheumatology criteria for eosinophilic granulomatosis with polyangiitis and was tentatively diagnosed with microscopic polyangiitis. Kidney biopsy showed pauci-immune crescentic necrotizing glomerulonephritis with neutrophilic and eosinophilic infiltration. Previous reports implicate eosinophils in the pathogenesis of this disease. Therefore, this case suggests that infiltrated eosinophils as well as neutrophils might play roles in the development of tissue injury in systemic vasculitis.     

Beneficial effect of gabexate mesilate on microscopic polyangiitis with renal dysfunction and pulmonary hemorrhage: A case report

Corticosteroids and cyclophosphamide are the mainstay of the treatment of microscopic polyangiitis involving pulmonary hemorrhage or rapidly progressive glomerulonephritis. However, patients with advanced age are unable to tolerate this combined therapy, because of a relatively high incidence of side effects including infection, hemorrhagic cystitis, and bone marrow suppression. The authors encountered an 80-year-old patient with pulmonary hemorrhage and renal dysfunction ascribed to microscopic polyangiitis and achieved successful treatment by employing gabexate mesilate in addition to corticosteroids. The present case suggests that gabexate mesilate may be a therapeutic option for microscopic polyangiitis with progressive renal failure and pulmonary hemorrhage.     

Fibrosing alveolitis predating microscopic polyangiitis.

A 65 year old male was diagnosed with "cryptogenic fibrosing alveolitis (CFA)" and treated successfully with Prednisone. In the year following Prednisone-tapering he presented with livedo reticularis, segmental pauci-immune glomerulonephritis and necrotizing vasculitis of the peripheral nerves, increased pulmonary fibrosis, and the presence of p-ANCA antibodies. Aggressive immunosuppressive treatment of this microscopic polyangiitis (MPA) was successful and also resulted in stabilization of the pulmonary fibrosis. This case illustrates that MPA may present itself monosymptomatic as CFA.