Microscopic Polyangiitis The Silica Facts
What is microscopic polyangiitis?
Microscopic polyangiitis (MPA) is an uncommon disease. It is the result of blood vessel inflammation (vasculitis), which can damage organ systems. The areas most commonly affected by MPA include the kidneys, lung, nerves, skin, and joints. MPA shares many common features with another form of vasculitis calledgranulomatosis with polyangiitis (Wegener's), and treatment approaches for these illnesses are similar.
What is vasculitis?
Vasculitis is a general term that refers to inflammation of the blood vessels. When inflamed, the blood vessel may become weakened and stretch forming an aneurysm, or become so thin that it ruptures resulting in bleeding into the tissue. Vasculitis can also cause blood vessel narrowing to the point of closing off the vessel entirely. This can cause organs to become damaged from loss of oxygen and nutrients that were being supplied by the blood.http://my.clevelandclinic.org/health/diseases_conditions/hic_Microscopic_Polyangitis
Microscopic polyangiitis
Specialty rheumatology
Microscopic polyangiitis is an ill-defined autoimmune disease characterized by a systemic, pauci-immune, necrotizing, small-vessel vasculitis without clinical or pathological evidence of necrotizing granulomatous inflammation.
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Silica
Although the most common health effect of silica exposure are the lung diseases occupational exposure to respirable crystalline silica is also associated with a number of other diseases, including systemic autoimmune diseases, such as rheumatoid arthritis, scleroderma, systemic lupus erythematosus (SLE), and some of the small vessel vasculitides and with renal diseases i.e. rapid progressive glomerulonephritis, nephrotic syndrome, and end stage renal disease.----------------------------------------------------------------------------------------------------------------------------------
Ausrtalian Government
Statement of Principles
concerning
MICROSCOPIC POLYANGIITIS
No. 14 of 2011
for the purposes of the
Veterans’ Entitlements Act 1986
and
Military Rehabilitation and Compensation Act 2004
Title
1. This Instrument may be cited as Statement of Principles concerning microscopic polyangiitis No. 14 of 2011.
Determination
2. This Statement of Principles is determined by the Repatriation Medical Authority under subsection 196B(3) of the Veterans’ Entitlements Act 1986 (the VEA).
Kind of injury, disease or death
3. (a) This Statement of Principles is about microscopic polyangiitis and death from microscopic polyangiitis.
(b) For the purposes of this Statement of Principles, "microscopic polyangiitis"means a necrotising vasculitis of capillaries, venules or arterioles that typically involves the lungs, kidneys and peripheral nervous system.
(c) Microscopic polyangiitis attracts ICD-10-AM code M31.7.
(d) In the application of this Statement of Principles, the definition of "microscopic polyangiitis" is that given at paragraph 3(b) above.
Basis for determining the factors
4. On the sound medical-scientific evidence available, the Repatriation Medical Authority is of the view that it is more probable than not that microscopic polyangiitis and death from microscopic polyangiitis can be related to relevant service rendered by veterans or members of the Forces under the VEA, or members under the Military Rehabilitation and Compensation Act 2004 (the MRCA).
Factors that must be related to service
5. Subject to clause 7, at least one of the factors set out in clause 6 must be related to the relevant service rendered by the person.
Factors
6. The factor that must exist before it can be said that, on the balance of probabilities,microscopic polyangiitis or death from microscopic polyangiitis is connected with the circumstances of a person’s relevant service is:
(a) being treated with propylthiouracil or hydralazine at the time of the clinical onset of microscopic polyangiitis; or
(b) inhaling respirable crystalline silica dust, at the time material containing crystalline silica was being:
(i) produced;
(ii) excavated;
(iii) drilled, cut or ground; or
(iv) used in construction, manufacturing, cleaning or blasting,
for a cumulative period of at least 5000 hours before the clinical onset of microscopic polyangiitis; or
(c) being treated with propylthiouracil or hydralazine at the time of the clinical worsening of microscopic polyangiitis; or
(d) inhaling respirable crystalline silica dust, at the time material containing crystalline silica was being:
(i) produced;
(ii) excavated;
(iii) drilled, cut or ground; or
(iv) used in construction, manufacturing, cleaning or blasting,
for a cumulative period of at least 5000 hours before the clinical worsening ofmicroscopic polyangiitis; or
(e) inability to obtain appropriate clinical management for microscopic polyangiitis.
https://www.legislation.gov.au/Details/F2010L03262
----------------------------------------------------------------------------------------------------------------------------------Diagnosis of perinuclear anti-neutrophil cytoplasmic antibody-associated microscopic polyangiitis in silicotics:
case report Ji-Won Lee1,3, Jun-Pyo Myong1,3*, Yeong-Jin Choi2 , Seyoung Lee1,3, Bum Seak Jo1,3 and Jung-Wan Koo1,3 Abstract Background:An association between silica exposure and autoimmune diseases including rheumatoid arthritis, systemic sclerosis, systemic lupus erythematosus, and anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis has been made. Case presentation: A 56-year-old male presented with silicosis and had an occupational history of precious metal processing for 30 years and a 30 pack-year smoking history. The patient was diagnosed with pneumoconiosis and received compensation. No other complications were reported for pneumoconiosis. The patient suddenly presented with a non-specific headache for several days and microscopic hematuria was identified upon examination in the outpatient clinic. Following several weeks, the patient presented with aggravated dyspnea and hemoptysis, and his Modification of Diet in Renal Disease estimated glomerular filtration rate indicated acute kidney injury. Diagnostic analysis revealed perinuclear ANCA-associated microscopic polyangiitis (p-ANCA-associated MPA). Conclusion: Exposure to silica dust was likely one of the cause of p-ANCA-associated MPA. Possible pathogenic mechanisms of autoimmune diseases in silicotics and emphasis of the necessity for early diagnosis are discussed.
Lee et al. Annals of Occupational and Environmental Medicine (2016) 28:21 DOI 10.1186/s40557-016-0108-1
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Microscopic polyangiitis associated with pleuropericarditis, pulmonary embolism and pulmonary hemorrhage as a complication of silicosis
Abstract
Silica (silicon dioxide) occupational exposure has been linked to both pulmonary and extra-pulmonary toxicity. Silicosis is the major pulmonary toxicity, which has also been associated with the development of collagen-vascular disease and with anti-neutrophil cytoplasmic antibody (ANCA)-positive vasculitis, especially perinuclear anti-neutrophil cytoplasmic antibodies (P-ANCA). The most common pulmonary manifestations of microscopic polyangitis (MPA) are interstitial fibrosis and alveolar hemorrhage. We describe a patient who had unusual presentation of microscopic polyangitis, characterized by lung hemorrhage, rapidly progressive glomerulonephritis, pleuropericarditis and pulmonary embolism that was associated with a history of silica exposure and radiologic evidence for silicosis.
In conclusion, we report a patient with silicosis and complicating microscopic polyangiitis characterized by lung hemorrhage, rapidly progressive glomerulonephritis pleuropericarditis and pulmonary embolism. This report strengthens the evidence supporting the causality of this association and reinforces the need for obtaining a detailed work history in patients who have systemic vasculitis.
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Microscopic Polyangiitis Accompanied by Pleuritis as
the only Pulmonary Manifestation of Occupational Silica Exposure
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Case Reports in Nephrology Volume 2014 (2014), Article ID 902089, 3 pages http://dx.doi.org/10.1155/2014/902089
Case Report
Microscopic Polyangiitis following Silicone Exposure from Breast Implantation
Department of Internal Medicine, Jacobi Medical Center, Albert Einstein College of Medicine, Bronx, NY 10461, USA
Received 31 July 2014; Revised 29 September 2014; Accepted 29 September 2014; Published 16 October 2014
Academic Editor: Sofia Lionaki
Abstract
Abstract
We describe a case of a patient who developed microscopic polyangiitis (MPA) in the setting of exposure to silicone after breast implantation. A 57-year-old Hispanic woman was admitted to our hospital with complaints of fever, cough, and hemoptysis. She had undergone silicone breast implantation two years prior to presentation. She was diagnosed as having microscopic polyangiitis (MPA) based on acute progressive renal failure, hematuria, pulmonary hemorrhage, and positivity for myeloperoxidase-anti-neutrophil cytoplasmic antibody (ANCA). A renal biopsy performed showed focal segmental necrotizing and crescentic glomerulonephritis. The patient received high dose steroids, cyclophosphamide, and plasmapheresis with remarkable clinical response. This case report raises the possibility of the development of MPA after silicone exposure from breast implantation.
1. Introduction
There have been a number of reported cases in which autoimmune syndromes have occurred following exposure to various chemicals. In 1964, Miyoshi et al. [1] first coined the term human adjuvant disease in their report of two patients who developed connective tissue-like disease after exposure to silicone-related substances during augmentation mammoplasty. Since this first observation, there have been numerous published cases relating silicone exposure to autoimmune rheumatic diseases. We, herein, report a patient who developed anti-neutrophil cytoplasmic antibody- (ANCA-) associated vasculitis following exposure to silicone from breast implantation.
Copyright © 2014 Judy Tan et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Reumatol Clin. 2014 May-Jun;10(3):180-2. doi: 10.1016/j.reuma.2013.04.009. Epub 2013 Jul 23.
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Microscopic polyangiitis secondary to silica exposure.
[Article in English, Spanish]
Abstract
Copyright © 2013 Elsevier España, S.L. All rights reserved.
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26 March 2014
NICE says yes to new treatment for rare autoimmune disease in final guidance
NICE has today published final guidance recommending rituximab (MabThera, Roche Products) as an option for inducing remission in adults with the rare autoimmune condition anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis
NICE has today published final guidance recommending rituximab (MabThera,
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