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Microscopic Polyangiitis

Microscopic Polyarteritis

Microscopic polyangiitis (MPA) is a rare inflammatory disease of the small blood vessels. This type of vasculitis affects numerous organs.

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Presentation

The illness initially presents with a prodromal phase characterized by fever, myalgia, arthralgia, and weight loss. The manifestations that follow are reflective of which systems are affected. The kidneys are involved in as many as 90% of all patients. Hematuria is one of the common signs. Kidney disease can rapidly evolve into renal failure. Individuals with gastrointestinal (GI) disease exhibit nausea, emesis, diarrhea, abdominal pain, and bloody stools.

Additionally, vasculitis of the lungs may result in alveolar hemorrhage and pulmonary fibrosis. The former is typified by a sudden onset of dyspnea and is possibly accompanied by hemoptysis. Other symptoms of the respiratory disease are rhinitis, sinusitis, and epistaxis. Patients with neurologic sequelae experience mononeuritis multiplex, which affects peripheral or cranial nerves. Furthermore, severe complications may include stroke or seizures.

Approximately one-third of individuals have vasculitis of the skin, which results in purpura rash, and possibly splinter hemorrhages. Finally, episcleritis arises in ocular vasculitis.

Fever
  • The titer of MPO-ANCA decreased with the treatment, but fever recurred with prednisolone taper.[ncbi.nlm.nih.gov]
  • Herein we describe a patient who was diagnosed with MPA presenting with radiographic evidence of pulmonary interstitial fibrosis as an early clinical manifestation accompanied by constitutional symptoms such as fever and weight loss.[ncbi.nlm.nih.gov]
  • Azathioprine hypersensitivity is a clinical syndrome which may manifest from isolated fever and rash to multi-organ failure. This rare condition is usually self-limiting following the discontinuation of azathioprine.[ncbi.nlm.nih.gov]
  • Abstract A 68-year-old man, who had worked for processing quartz-containing stones for more than 50 years, complained of low-grade fever and arthralgia. Mediastinal lymph nodes were markedly swollen on chest computed tomography.[ncbi.nlm.nih.gov]
  • 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.[ncbi.nlm.nih.gov]
Weight Loss
  • Herein we describe a patient who was diagnosed with MPA presenting with radiographic evidence of pulmonary interstitial fibrosis as an early clinical manifestation accompanied by constitutional symptoms such as fever and weight loss.[ncbi.nlm.nih.gov]
  • 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.[ncbi.nlm.nih.gov]
  • We report a case of a 76-year-old woman, presenting with a persistent dry cough, subfebrility, arthralgia and myalgia, weight loss and a breast lesion.[ncbi.nlm.nih.gov]
  • A 76-year-old man was admitted with general fatigue, weight loss, fever, headache, renal failure, and a high serum level of myeloperoxidase-antineutrophil cytoplasmic antibody.[ncbi.nlm.nih.gov]
  • Eleven patients experienced weight loss, several had kidney involvement, and most had an increased erythrocyte sedimentation rate and C-reactive protein.[ncbi.nlm.nih.gov]
Rapidly Progressive Glomerulonephritis
  • Its typical clinical manifestations are rapidly progressive glomerulonephritis and alveolar hemorrhage. We describe a 30-year-old woman with rapidly progressive glomerulonephritis.[ncbi.nlm.nih.gov]
  • The first-line treatment of MPA is the association of steroids and cyclophosphamide, especially in the presence of a rapidly progressive glomerulonephritis.[ncbi.nlm.nih.gov]
  • KEYWORDS: Infection; Microscopic polyangiitis (MPA); Prognosis; Rapidly progressive glomerulonephritis (RPGN); Relapse[ncbi.nlm.nih.gov]
  • We report on the case of a 72-year-old woman with MPA who developed hemocholecyst in addition to alveolar hemorrhage and rapidly progressive glomerulonephritis.[ncbi.nlm.nih.gov]
  • 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[ncbi.nlm.nih.gov]
Fatigue
  • A 76-year-old man was admitted with general fatigue, weight loss, fever, headache, renal failure, and a high serum level of myeloperoxidase-antineutrophil cytoplasmic antibody.[ncbi.nlm.nih.gov]
  • She was admitted to our hospital with symptoms of general fatigue, fever, and edema of the lower limbs.[ncbi.nlm.nih.gov]
  • Clinical features may include constitutional symptoms like fever, loss of appetite, weight loss, fatigue, and kidney failure. A majority of patients may have blood in the urine and protein in the urine.[en.wikipedia.org]
  • Patients typically present with fever, fatigue, weakness and muscle and joint aches. These symptoms are very common among many different diseases, not just vasculitis.[clinicaltrials.ucsf.edu]
  • Patients who have MPA may feel generally ill and fatigued, have fever, or have loss of appetite and weight. They usually also have symptoms related to areas of involvement such as rashes, muscle and/or joint pain.[my.clevelandclinic.org]
Anemia
  • We reported an 11-year-old girl without respiratory symptoms with sever anemia which shows signs of hemolytic anemia in test data. Chest X-ray and computed tomography scans suggested alveolar hemorrhage.[ncbi.nlm.nih.gov]
  • The patient's health deteriorated at 1 week, with rapidly progressing anemia. Computerized tomography identified a large, right-sided, perirenal hematoma, with active bleeding.[ncbi.nlm.nih.gov]
  • All of the patients presented with normocytic normochromic anemia.[ncbi.nlm.nih.gov]
  • Microcytic anemia refractory to usual supplementation is an important clue to an alternative diagnosis. Accompanying pulmonary and renal involvement during the disease course suggests a multisystem disease.[ncbi.nlm.nih.gov]
  • In this report, a case of an adolescent presenting with symptomatic anemia and syncopal episodes is described. An extensive evaluation ultimately led to the diagnosis of MPA.[ncbi.nlm.nih.gov]
Rhinitis
  • Mild symptoms of rhinitis, epistaxis, and sinusitis may occur; however, if the upper respiratory tract is severely affected, the cause is more likely to be granulomatosis with polyangiitis.[merckmanuals.com]
  • Other symptoms of the respiratory disease are rhinitis, sinusitis, and epistaxis. Patients with neurologic sequelae experience mononeuritis multiplex, which affects peripheral or cranial nerves.[symptoma.com]
  • He reports also having allergic rhinitis in the past few weeks, which was strange given that he normally has allergic rhinitis in the spring.[medbullets.com]
  • It can cause: asthma allergic rhinitis ( cold-like symptoms caused by allergies ) a high temperature (fever) muscle and joint pain tiredness loss of appetite and weight loss It can also affect the nerves, causing weakness, pins and needles or numbness[nhs.uk]
  • ), although PR3–ANCA (c–ANCA) may be also present in 40 percent of patients. 9 The most common age of onset is 40 to 60 years and is more common in men. 9 Churg-Strauss Syndrome Churg-Strauss syndrome is a rare disease and has three phases: allergic rhinitis[aafp.org]
Abdominal Pain
  • Clinicians should consider the possibility of hemocholecyst in patients with MPA complaining of abdominal pain.[ncbi.nlm.nih.gov]
  • All patients had abdominal pain, four of seven (57%) had an acute kidney injury and three patients required renal replacement therapy.[ncbi.nlm.nih.gov]
  • Individuals with gastrointestinal (GI) disease exhibit nausea, emesis, diarrhea, abdominal pain, and bloody stools. Additionally, vasculitis of the lungs may result in alveolar hemorrhage and pulmonary fibrosis.[symptoma.com]
  • Gastrointestinal (GI): GI symptoms include abdominal pain, nausea, vomiting, diarrhea, and bloody stools.[merckmanuals.com]
  • Gastrointestinal symptoms are characterized by abdominal pain (32 to 58%) and digestive tract bleeding (29%). Peripheral neuropathy is found in only 14 to 36% of the cases, thus occurring less frequently than in PAN.[ncbi.nlm.nih.gov]
Vomiting
  • Digestive tract: Abdominal pain, nausea, vomiting, and diarrhea may occur. Stools may contain blood. Nerves: People may have tingling, numbness, or weakness in a limb. Other organs are affected less often.[msdmanuals.com]
  • Gastrointestinal (GI): GI symptoms include abdominal pain, nausea, vomiting, diarrhea, and bloody stools.[merckmanuals.com]
  • A 59-year-old Korean woman visited the Emergency Department in Presbyterian Medical Center for evaluation of epigastric pain and vomiting. She had a 20-year history of type 2 diabetes mellitus.[kjim.org]
  • Initially, they have: Fatigue Fever Weight loss Loss of appetite The following symptoms may develop: Skin: rash with reddish-purple spots Abdominal: nausea, vomiting, diarrhea, abdominal pain, and bloody stools Kidney: blood in urine Lung: shortness of[symptoma.com]
  • It can cause: a rash that looks like small bruises or reddish-purple spots joint pain tummy (abdominal) pain diarrhoea and vomiting blood in urine or poo It's not usually serious and tends to get better without treatment.[nhs.uk]
Nausea
  • Individuals with gastrointestinal (GI) disease exhibit nausea, emesis, diarrhea, abdominal pain, and bloody stools. Additionally, vasculitis of the lungs may result in alveolar hemorrhage and pulmonary fibrosis.[symptoma.com]
  • Digestive tract: Abdominal pain, nausea, vomiting, and diarrhea may occur. Stools may contain blood. Nerves: People may have tingling, numbness, or weakness in a limb. Other organs are affected less often.[msdmanuals.com]
  • Gastrointestinal (GI): GI symptoms include abdominal pain, nausea, vomiting, diarrhea, and bloody stools.[merckmanuals.com]
  • Your body will produce this increase in progesterone, which causes some of the symptoms of pregnancy, including breast tenderness and nausea. If your progesterone levels are too low, your uterus may not be able to carry the baby to term.[healthline.com]
  • […] glomerulonephritis Respiratory tract Dyspnea, cough, hemoptysis; lung infiltrate, interstitial lung disease, pulmonary hemorrhage Nervous system Peripheral neuropathy, especially mononeuritis Gastrointestinal tract Fecal blood, elevated liver enzymes; diarrhea, nausea[aafp.org]
Loss of Appetite
  • Clinical features may include constitutional symptoms like fever, loss of appetite, weight loss, fatigue, and kidney failure. A majority of patients may have blood in the urine and protein in the urine.[en.wikipedia.org]
  • Patients who have MPA may feel generally ill and fatigued, have fever, or have loss of appetite and weight. They usually also have symptoms related to areas of involvement such as rashes, muscle and/or joint pain.[my.clevelandclinic.org]
  • Common symptoms in MPA are tiredness, loss of appetite and joint and muscle aches. The other symptoms of MPA will depend on the organs involved and will vary markedly between different patients.[vasculitis.org.uk]
  • Initially, they have: Fatigue Fever Weight loss Loss of appetite The following symptoms may develop: Skin: rash with reddish-purple spots Abdominal: nausea, vomiting, diarrhea, abdominal pain, and bloody stools Kidney: blood in urine Lung: shortness of[symptoma.com]
  • The symptoms of polyarteritis nodosa vary from one patient to another and include fever, fatigue, weight loss, loss of appetite, joint and muscle pain, skin outbreaks, swollen legs, skin ulcers and/or nodules.[therapeutique-dermatologique.org]
Diarrhea
  • Individuals with gastrointestinal (GI) disease exhibit nausea, emesis, diarrhea, abdominal pain, and bloody stools. Additionally, vasculitis of the lungs may result in alveolar hemorrhage and pulmonary fibrosis.[symptoma.com]
  • Digestive tract: Abdominal pain, nausea, vomiting, and diarrhea may occur. Stools may contain blood. Nerves: People may have tingling, numbness, or weakness in a limb. Other organs are affected less often.[msdmanuals.com]
  • Gastrointestinal (GI): GI symptoms include abdominal pain, nausea, vomiting, diarrhea, and bloody stools.[merckmanuals.com]
  • […] glomerulonephritis Respiratory tract Dyspnea, cough, hemoptysis; lung infiltrate, interstitial lung disease, pulmonary hemorrhage Nervous system Peripheral neuropathy, especially mononeuritis Gastrointestinal tract Fecal blood, elevated liver enzymes; diarrhea[aafp.org]
  • GPA and MPA ( 15 %): infections, nausea, diarrhea, headache, muscle spasms, anemia, peripheral edema (other important adverse reactions include infusion-related reactions) ( 6.3 ).[dailymed.nlm.nih.gov]
Anisocoria
  • Anisocoria and decreased light reflex as well as limited supraduction, infraduction, and adduction were also observed in the eye. Left oculomotor nerve palsy was diagnosed. The palsy gradually improved with continued prednisolone treatment.[ncbi.nlm.nih.gov]
Arthralgia
  • We hereby present a 14-year-old girl with arthralgia, seizure, leukocytoclastic vasculitis, interstitial lung disease secondary to recurrent pulmonary hemorrhage, pauci-immune glomerulonephritis and high titers of MPO-ANCA, hence diagnostic of microscopic[ncbi.nlm.nih.gov]
  • Abstract A 68-year-old man, who had worked for processing quartz-containing stones for more than 50 years, complained of low-grade fever and arthralgia. Mediastinal lymph nodes were markedly swollen on chest computed tomography.[ncbi.nlm.nih.gov]
  • We report a case of a 76-year-old woman, presenting with a persistent dry cough, subfebrility, arthralgia and myalgia, weight loss and a breast lesion.[ncbi.nlm.nih.gov]
  • Before treatment could be initiated she developed small joint arthralgia and a glove and stocking axonal loss sensorimotor neuropathy.[ncbi.nlm.nih.gov]
  • The cutaneous involvement is present at an early stage of microscopic polyangiitis with other non-specific symptoms, such as arthralgias and myalgias.[medicaljournals.se]
Myalgia
  • We report a case of a 76-year-old woman, presenting with a persistent dry cough, subfebrility, arthralgia and myalgia, weight loss and a breast lesion.[ncbi.nlm.nih.gov]
  • The cutaneous involvement is present at an early stage of microscopic polyangiitis with other non-specific symptoms, such as arthralgias and myalgias.[medicaljournals.se]
  • The illness initially presents with a prodromal phase characterized by fever, myalgia, arthralgia, and weight loss. The manifestations that follow are reflective of which systems are affected.[symptoma.com]
  • […] necrotizing vasculitis predominantly affecting small to medium vessels involvement of respiratory tract HISTORY rhinorrhea, bloody nasal discharge, oral/nasal ulcers, sinus pain haemoptysis pleuritic chest pain haematuria stridor (subglottic stenosis) myalgias[lifeinthefastlane.com]
  • Musculoskeletal involvement (myalgias, arthralgias and arthritis) are present in 65 to 72% of the patients. Cutaneous lesions (purpura, splinter hemorrhages) are found in 44 to 58% of the patients.[ncbi.nlm.nih.gov]
Purpura
  • In two patients, a Henoch-Shönlein purpura-like eruption developed with necrotizing vasculitis localized in the upper dermis.[ncbi.nlm.nih.gov]
  • Four years later, purpura, peripheral neuropathy, and increased levels of myeloperoxidase anti-neutrophil cytoplasmic antibodies (583 EU/mL) and C-reactive protein (2.27 mg/dL) were observed, and a diagnosis of microscopic polyangiitis was made.[ncbi.nlm.nih.gov]
  • She also had microscopic polyangiitis clinically manifesting as crescentic glomerulonephritis and purpura with positive myeloperoxidase (MPO)-antineutrophil cytoplasmic antibodies (ANCA).[ncbi.nlm.nih.gov]
  • Multiple mononeuritis and purpura were observed. The urine was positive for occult blood and protein and the creatinine level was 1.2 mg/dL, indicating renal impairment.[ncbi.nlm.nih.gov]
  • Purpura and petechiae in 6 patients, livedo in 2 patients, and erythema in 7 patients, especially erythema on the hands and\or fingers in 4 patients, were observed.[ncbi.nlm.nih.gov]
Eruptions
  • This retrospective study analyzed clinical manifestations in patients of MPA with skin eruptions.[ncbi.nlm.nih.gov]
  • A case of a 7-year-old girl with microscopic polyangiitis (MPA) with a skin eruption characterized by maculopapular, erythematous and purpuric lesions on the face, elbows, and knees is presented.[ncbi.nlm.nih.gov]
  • In two patients, a Henoch-Shönlein purpura-like eruption developed with necrotizing vasculitis localized in the upper dermis.[ncbi.nlm.nih.gov]
  • Histopathologic examination of the lower extremity eruption revealed fibrinoid degeneration, nuclear dust, neutrophilic infiltration, and erythrocyte extravasation in the dermis (H&E, X400).[dermatologyadvisor.com]
  • —Cardiac ( —Skin (40%): Livedo reticularis, nodules, nonspecific maculopapular eruptions, and ulcerations are seen. Subcutaneous skin lesions are typical in the more limited form, cutaneous PAN.[rheumaknowledgy.com]
Splinter Hemorrhage
  • Cutaneous lesions (purpura, splinter hemorrhages) are found in 44 to 58% of the patients. Gastrointestinal symptoms are characterized by abdominal pain (32 to 58%) and digestive tract bleeding (29%).[ncbi.nlm.nih.gov]
  • The nails may contain thin purplish lines, indicating bleeding (called splinter hemorrhages). Rarely, the blood supply to the fingers and toes is reduced. Digestive tract: Abdominal pain, nausea, vomiting, and diarrhea may occur.[msdmanuals.com]
  • Nail bed infarcts and splinter hemorrhages may occur; digital ischemia occurs rarely. Respiratory: If the lungs are affected, alveolar hemorrhage may occur and may be followed by pulmonary fibrosis.[merckmanuals.com]
  • Approximately one-third of individuals have vasculitis of the skin, which results in purpura rash, and possibly splinter hemorrhages. Finally, episcleritis arises in ocular vasculitis.[symptoma.com]
Epistaxis
  • […] crescentic glomerulonephritis on renal biopsy Differentiated from granulomatosis with polyangiitis (GPA) by absence of granulomatous inflammation Clinical Presentation Constitutional – weight loss, fever, myalgias Otorhinolaryngological – oral ulcers, epistaxis[arupconsult.com]
  • She also complained of sinus congestion with occasional epistaxis. On exam, her nasal mucosa was significantly inflamed.[omicsonline.org]
  • Pulmonary hemorrhage Lung infiltrates Gastrointestinal (50%) Abdominal pain Severe form with bowel perforation Hepatomegaly Neurologic (30%) Peripheral neuropathy less frequent than PAN Central nervous system Ear, nose, throat (30-35%) Mouth ulcers Epistaxis[basicmedicalkey.com]
  • Mild symptoms of rhinitis, epistaxis, and sinusitis may occur; however, if the upper respiratory tract is severely affected, the cause is more likely to be granulomatosis with polyangiitis.[merckmanuals.com]
  • Other symptoms of the respiratory disease are rhinitis, sinusitis, and epistaxis. Patients with neurologic sequelae experience mononeuritis multiplex, which affects peripheral or cranial nerves.[symptoma.com]
Hematuria
  • After her second pregnancy and delivery when she was 32 years old, she developed proteinuria of 3.2 g/gCre, hematuria, and elevated serum creatinine level of 2.6 mg/dL.[ncbi.nlm.nih.gov]
  • The majority of these patients exhibited hematuria, proteinuria and renal insufficiency. The efficacy of steroid plus CTX or leflunomide was evident in these patients.[ncbi.nlm.nih.gov]
  • Microscopic hematuria was detected but a renal biopsy showed no abnormalities. Histological examination of a skin biopsy from a purpuric papule showed leukocytoclastic vasculitis of the small vessels in the entire dermis.[ncbi.nlm.nih.gov]
  • Her postoperative course was uneventful until she coughed up blood and had gross hematuria 3 days after the second surgery. Histological examination of a renal biopsy specimen revealed crescentic glomerulonephritis.[ncbi.nlm.nih.gov]
  • On admission, his blood pressure was 166/92 mmHg, and laboratory findings showed signs of inflammation, anemia, proteinuria, and hematuria.[ncbi.nlm.nih.gov]
Kidney Failure
  • CONCLUSIONS: Geographical differences in the incidence of RRT for kidney failure due to granulomatosis with polyangiitis and microscopic polyangiitis copied their distribution in the general population.[ncbi.nlm.nih.gov]
  • Clinical features may include constitutional symptoms like fever, loss of appetite, weight loss, fatigue, and kidney failure. A majority of patients may have blood in the urine and protein in the urine.[en.wikipedia.org]
  • Almost all people with microscopic polyangiitis have kidney problems that can lead to raised blood pressure and kidney failure. People may find that they're tired because of anaemia.[arthritiscare.org.uk]
  • He may suffer from complications like kidney failure, bleeding in the lungs or perforation in the digestive system . Blood tests, imaging studies like x-ray and CT scan and biopsy of the affected organ are used to diagnose the condition.[medindia.net]
Stroke
  • For stroke patients with acute nephritis, the possibility of ANCA vasculitis should be considered. Early diagnosis may improve the prognosis.[ncbi.nlm.nih.gov]
  • Author information 1 Adnan Menderes University, Faculty of Medicine, Department of Neurology, 09100 Aydin, Turkey. ozkulayca@hotmail.com Abstract Microscopic polyangiitis is a small vessel vasculitis which is rarely associated with ischemic stroke.[ncbi.nlm.nih.gov]
  • Furthermore, severe complications may include stroke or seizures. Approximately one-third of individuals have vasculitis of the skin, which results in purpura rash, and possibly splinter hemorrhages.[symptoma.com]
  • The National Institute of Neurological Disorders and Stroke (NINDS) collects and disseminates research information related to neurological disorders. Click on the link to view information on this topic.[rarediseases.info.nih.gov]
  • […] red (blood shot) eyes, painful, dry or gritty eyes, visual loss or other changes in vision Nerves - loss of sensation, weakness, unusual painful symptoms in the hands and feet (hotness, pins and needles or "electric shocks") and rarely paralysis or stroke[vasculitis.org.uk]
Seizure
  • We hereby present a 14-year-old girl with arthralgia, seizure, leukocytoclastic vasculitis, interstitial lung disease secondary to recurrent pulmonary hemorrhage, pauci-immune glomerulonephritis and high titers of MPO-ANCA, hence diagnostic of microscopic[ncbi.nlm.nih.gov]
  • The patient suffered from severe and protracted hypoglycaemia, complicated by a tonic-clonic seizure 7 days after escalation to therapeutic co-trimoxazole. Endogenous hyperinsulinaemia was confirmed and was attributed to co-trimoxazole use.[ncbi.nlm.nih.gov]
  • Furthermore, severe complications may include stroke or seizures. Approximately one-third of individuals have vasculitis of the skin, which results in purpura rash, and possibly splinter hemorrhages.[symptoma.com]
  • Rarely, cerebral hemorrhage, infarction, seizures, or headache results from cerebral vasculitis. Cardiac: Rarely, the heart is affected. Ocular: If the eyes are affected, episcleritis usually results.[merckmanuals.com]
  • […] dyspnea, cough Cardiovascular – Chest pain, symptoms of heart failure Gastrointestinal (GI) - GI bleeding, abdominal pain Neurologic - Peripheral nervous system involvement manifesting as mononeuritis multiplex (57%); CNS involvement manifesting as seizures[emedicine.medscape.com]

Workup

Individuals suspected to have vasculitis should be immediately evaluated with a detailed personal and family history, a physical exam, and comprehensive testing.

Laboratory tests

There are various potential findings in patients with MPA. For example, 80% are positive for ANCA, 60% for perinuclear ANCA (PANCA), and 40% for cytoplasmic ANCA (CANCA). Also, C3 and C4 complement studies are normal. Additional studies reveal leukocytosis and normocytic anemia on CBC and elevated ESR. Kidney function tests demonstrate increased blood urea nitrogen (BUN) and serum creatinine levels in 70% of patients, proteinuria in 80%, hematuria in almost 70%, and leukocyturia in 40%. Other results include the presence of erythrocyte casts and abnormal urine sediment. Note that blood cultures should be obtained to investigate bacterial endocarditis.

Imaging

The selection of imaging modalities is based on the present symptoms and complications. For example, patients with respiratory involvement should have a chest radiograph. This study typically displays bilateral nodular and patchy opacities, cavitary lesions, and parenchymal infiltrates. Furthermore, computed tomography (CT) and gastrointestinal endoscopy should be obtained for abdominal manifestations such as bleeding. Electromyography (EMG) is indicated in neuropathy while electrocardiography (ECG) is warranted for the evaluation of cardiac disease.

Other

Biopsies are performed for involved sites such as the skin, lungs, kidneys. and sural nerve. A muscle biopsy may be considered as well whether or not there is myalgia or other evidence of muscle involvement [10].

Creatinine Increased
  • Abstract A 76-year-old man developed fever and appetite loss, and then was referred to our hospital because of rapidly progressive renal insufficiency; his serum creatinine increased from 1.2 to 5.9 mg/dl within 1 month.[ncbi.nlm.nih.gov]
Microcytic Anemia
  • Microcytic anemia refractory to usual supplementation is an important clue to an alternative diagnosis. Accompanying pulmonary and renal involvement during the disease course suggests a multisystem disease.[ncbi.nlm.nih.gov]
White Matter Lesions
  • Brain computed tomographic and magnetic resonance imaging (MRI) scans revealed asymptomatic chronic cerebral hemorrhage and white matter lesions. She also later developed thalamic infarction.[ncbi.nlm.nih.gov]
Ischemic Changes
  • Histological findings suggested ischemic changes. Because mononeuritis multiplex and a fever spike appeared later, vasculitis was suspected. The perinuclear anti-neutrophil cytoplasmic antibody titer was elevated.[ncbi.nlm.nih.gov]
Liver Biopsy

Treatment

The treatment of MPA is centered on remission induction and maintenance.

Remission induction

Since glomerulonephritis is a common manifestation in patients with MPA, historically the regimen consisted of cyclophosphamide and glucocorticoids for both induction and maintenance [11]. However, cyclophosphamide is associated with serious adverse effects such as malignancy, hemorrhagic cystitis, and infertility [12]. Therefore, researchers sought to minimize the use of this toxic medication.

Currently, there are numerous approaches for the initiation of therapy. One method is to avoid cytotoxic drugs if possible. Hence, patients with mild to moderate renal disease may be spared the use of cyclophosphamide and require only glucocorticoids and mycophenolate mofetil [13]. Additional options include methotrexate [14] and leflunomide [15].

Another drug that represents an alternative to cyclophosphamide is rituximab. One trial compared the two medications [16] and observed that the six-month remission rate for rituximab was 63.6% in comparison to 53.1% with cyclophosphamide. Also, patients who continued taking low-dose glucocorticoids in conjunction with the respective medications had better remission rates. Also, rituximab offers a promising treatment for those with relapsing disease.

Remission maintenance

One trial investigated cyclophosphamide versus azathioprine for the management of the remission maintenance phase [17]. Prior to the treatment with one of these two agents, all patients received cyclophosphamide and prednisolone for remission induction. The relapse rate was similar in both groups, which was 15% in the cyclophosphamide patients and 10% in the azathioprine. The study notably reports that induction with cyclophosphamide and maintenance with azathioprine is as effective as prolonged therapy with cyclophosphamide.

Another trial evaluated the outcomes of maintenance therapy with methotrexate versus azathioprine after induction with cyclophosphamide [18]. The relapse rate was similar: 33% in the methotrexate group versus 36% in the azathioprine group.

Other

Additional therapies may be beneficial for patients with MPA. For example, plasma exchange has been compared with methylprednisolone as adjunctive agents. The study discovered that plasma exchange decreased the development of end-stage renal disease at the 12-month mark [19] although both groups had equal mortality rates.

Prognosis

Factors such as older age, the severity at onset, number of relapses, and the length of glucocorticoid therapy were correlated to long-term organ damage [8]. Also, according to one investigation, pulmonary involvement was associated with a greater risk for multisystem diseases affecting the heart and kidney [9].

Fortunately, treatment accounts for an improvement in 90% of affected individuals. Moreover, 75% of patients enter remission although 30% experience a recurrence in a year or two.

The survival rate at five years is about 75%. However, two of the leading causes of morbidity and death are necrotizing glomerulitis and hemorrhagic pulmonary capillaritis. Note that MPA has worse outcomes than some of the other vasculitis diseases.

Etiology

While the exact etiology is unknown, the cause is likely multifactorial in origin consisting of genetic and environmental interplay. Patients often have a positive family history of other autoimmune diseases.

MPA has been associated with the use of drugs such as propylthiouracil, penicillamine, and hydralazine. Additionally, silica exposure is another environmental factor related to the etiology of the disease.

Epidemiology

The incidence of this disease is estimated to be 1 in a population of 100,000. With regards to patient demographics, the average age of onset is 50 years. Also, there is a slight predilection for males [2] [3] [4].

Sex distribution
Age distribution

Pathophysiology

While the pathophysiology is not well-elucidated, there is a theory that ANCA may be implicated in the development of MPA. Specifically, researchers speculate that primed neutrophils expressing myeloperoxidase (MPO) adhere to the endothelium of blood vessels and/or glomeruli and subsequently become activated by interacting with MPO-ANCA [5]. Furthermore, animal studies have reported that MPO-ANCA plays a role in glomerulonephritis and pulmonary capillaritis [6] [7].

It is important to note, however, that not all patients with MPA are ANCA positive. This suggests that there are other mechanisms that contribute to the pathogenesis of MPA. Very importantly, it is likely that this disease develops in genetically predisposed individuals.

Histopathology

The histologic analysis may help to differentiate MPA from polyarteritis nodosa and small vessel vasculitis as MPA affects the venules, arterioles, and capillaries. Moreover, the walls of these smaller vessels may contain neutrophilic infiltration.

Findings indicative of glomerulitis include focal necrosis, crescent formation, and the absence of immunoglobulin. Evaluation of the lung tissue demonstrates alveolar capillaritis while the muscle and sural nerve likely exhibit necrotizing vasculitis.

Prevention

This disease cannot be prevented. However, early diagnosis and treatment can improve outcomes.

Summary

Microscopic polyangiitis (MPA) is described as systemic vasculitis that is associated with antineutrophil cytoplasmic antibody (ANCA). This inflammatory disease affects small blood vessels such as the capillaries of the lungs, kidneys, and other organs. While the etiology and pathophysiology are not fully understood, evidence points to the likelihood of genetic predisposition and environmental components. While ANCA is positive in the majority of patients, there are likely other pathogenic mechanisms. Note that there are similar vasculitides associated with ANCA such as granulomatosis with polyangiitis (previously named Wegener granulomatosis) among others [1].

MPA can involve numerous organs, thereby producing a wide spectrum of symptoms. It mainly affects the renal and pulmonary blood vessels. It can cause life-threatening complications such as necrotizing glomerulitis and diffuse alveolar hemorrhage.

Clinical assessment is comprised of the patient and family history, physical examination, and the appropriate tests. The latter includes ANCA studies, complete blood count (CBC), erythrocyte sedimentation rate (ESR), and urinalysis. Furthermore, biopsies of affected organs are obtained. Finally, imaging techniques are performed to evaluate the present manifestations.

The treatment focuses on two key phases, which are remission induction and remission maintenance. Numerous drug trials have been conducted to determine the most effective treatment regimens based on renal involvement, the severity of the overall disease, and other factors. The first phase can be treated with cyclophosphamide and glucocorticosteroids, or with rituximab. The maintenance program can utilize cyclophosphamide, azathioprine, or methotrexate. Since cyclophosphamide is associated with severe side effects, its use should be minimized as much as possible.

Due to the risk of morbidity and mortality associated with MPA, prompt recognition and treatment are paramount in these patients. Aggressive management is typically necessary to prevent organ damage and other severe outcomes.

Patient Information

What is microscopic polyangiitis (MPA)?

This is a rare disease that results from vasculitis, which is inflammation of the blood vessels. MPA can involve various organs such as the kidneys, skin, and lungs. It can cause severe damage to these organs.

What are the causes?

The cause of MPA has not been discovered yet. It is thought that the disease develops due to genetic and environmental factors.

There are ANCA antibodies in the majority of individuals that are affected with this disease. These antibodies attach to certain types of white blood cells, which in turn attach to the walls of blood vessels. This whole process leads to the secretion of substances that cause inflammation.

What are the signs and symptoms?

This disease can affect many organs and organ systems. Therefore, patients can have a wide spectrum of signs and symptoms. Initially, they have:

The following symptoms may develop:

Complications may include:

How is it diagnosed?

When an individual presents with the above symptoms, the clinician will obtain the patient and family history, perform a physical exam, and order the appropriate blood tests such as:

  • ANCA antibodies
  • Complete blood cell count
  • Erythrocyte sedimentation rate
  • Creatinine
  • Urinalysis

Imaging tests are performed depending on which symptoms are present:

  • Chest x-ray
  • CT scan of affected organs
  • Gastrointestinal endoscopy
  • Electromyography (EMG)
  • Electrocardiography (ECG)

Biopsy (sample tissue) of affected organs is also obtained.

How is it treated?

The treatment is administered into two main phases which are remission induction and remission maintenance:

The treatment regimen depends on the kidney function, how severe the disease is, and other important factors. A combination of medications is typically given.

Can it be prevented?

There is no way to prevent the development of MPA. However, early treatment can help prevent complications.

What is the prognosis?

Treatment significantly improves the outcome in the majority of patients. Also, many patients achieve full remission although some relapse.

It is very important that patients are diagnosed and treated as soon as possible in order to prevent organ damage and complications.

References

Article

  1. Buendía-Roldán I, Navarro C, Rojas-Serrano J. Diffuse alveolar hemorrhage: causes and outcomes in a referral center. Reumatologia Clinica. 2010; 6(4):196–198.
  2. Savage CO, Winearls CG, Evans DJ, et al. Microscopic polyarteritis: presentation, pathology and prognosis. Q J Med. 1985; 56(220):467-83.
  3. Jennette JC, Falk RJ. Small vessel vasculitis. N Engl J Med. 1997; 337(21):1512-23.
  4. Guillevin L, Durand-Gasselin B, Cevallos R, et al. Microscopic polyangiitis: clinical and laboratory findings in eighty-five patients. Arthritis Rheum. 1999; 42(3):421-30.
  5. Kallenberg CG, Heeringa P, Stegeman CA. Mechanisms of Disease: pathogenesis and treatment of ANCA-associated vasculitides. Nature clinical practice. Rheumatology. 2006; 2(12):661–670.
  6. Little MA, Smyth CL, Yadav R, et al. Antineutrophil cytoplasm antibodies directed against myeloperoxidase augment leukocyte-microvascular interactions in vivo. Blood. 2005; 106(6):2050–2058.
  7. Xiao H, Heeringa P, Hu P, et al. Antineutrophil cytoplasmic autoantibodies specific for myeloperoxidase cause glomerulonephritis and vasculitis in mice. J Clin Invest. 2002; 110(7):955–963.
  8. Robson J, Doll H, Suppiah R, et al. Glucocorticoid treatment and damage in the anti-neutrophil cytoplasm antibody-associated vasculitides: long-term data from the European Vasculitis Study Group trials. Rheumatology (Oxford). 2015;54(3):471-81
  9. Hassan TM, Hassan AS, Igoe A, et al. Lung involvement at presentation predicts disease activity and permanent organ damage at 6, 12 and 24 months follow - up in ANCA - associated vasculitis. BMC Immunology. 2014; 15:20.
  10. Hervier B, Durant C, Masseau A, et al. Use of muscle biopsies for diagnosis of systemic vasculitides. J Rheumatol. 2011; 38(3):470-4.
  11. Fauci A, Wolff S. Wegener's granulomatosis: studies in eighteen patients and a review of the literature. Medicine. 1973;52(6):53–61.
  12. Hoffman GS, Kerr GS, Leavitt RY, et al. Wegener's Granulomatosis: An Analysis of 158 Patients. Ann Intern Med. 1992; 116(6):488–498.
  13. Silva F, Specks U, Kalra S, et al. Mycophenolate Mofetil for Induction and Maintenance of Remission in Microscopic Polyangiitis with Mild to Moderate Renal Involvement--A Prospective, Open-Label Pilot Trial. Clin J Am Soc Nephrol. 2010; 5(3):445-53.
  14. De Groot K, Rasmussen N, Bacon PA, et al. Randomized trial of cyclophosphamide versus methotrexate for induction of remission in early systemic antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum. 2005; 52(8):2461–2469.
  15. Metzler C, Miehle N, Manger K, et al. Elevated relapse rate under oral methotrexate versus leflunomide for maintenance of remission in Wegener's granulomatosis. Rheumatology (Oxford, England). 2007; 46(7):1087–1091.
  16. Stone JH, Seo P, Spiera R, et al. The RAVE-ITN Research Group. Rituximab Versus Cyclophosphamide for Induction of Remission in ANCA-Associated Vasculitis: A Randomized Controlled Trial (RAVE). Arthritis Rheum. 2009; 60 (Supp 10):550.
  17. Jayne D. Randomised trial of cyclophosphamide versus azathioprine during remission in ANCA-associated systemic vasculitis (CYCAZAREM). J Am Soc Nephrol. 1999; 10:105A.
  18. Pagnoux C, Mahr A, Hamidou MA, et al. Azathioprine or methotrexate maintenance for ANCA-associated vasculitis. N Engl J Med. 2008; 359(26):2790–2803.
  19. Jayne DR, Gaskin G, Rasmussen N, et al. Randomized trial of plasma exchange or high-dosage methylprednisolone as adjunctive therapy for severe renal vasculitis. J Am Soc Nephrol. 2007; 18(7):2180–2188.

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Last updated: 2019-07-11 20:39