Microscopic Polyangiitis Causing Diffuse Alveolar Hemorrhage and Rapidly Progressive Glomerulonephritis

Lababidi, Mohamad Hani; Odigwe, Chibuzo; Okolo, Chukwuka; Elhassan, Ahmed M.; Iroegbu, Nkemakolam

doi:10.1080/08998280.2015.11929311

Cited by 4 publications

(3 citation statements)

References 12 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…MPA is a pauci-immune, ANCA-associated necrotizing vasculitis that typically affects small blood vessels with histological absence of granuloma [1,2] . It is one of the pulmonary-renal vasculitis syndromes that causes DAH in the lungs (more frequently affected) and acute kidney injury (AKI) in the kidneys [1] .…”

Section: Discussionmentioning

confidence: 99%

“…It is one of the pulmonary-renal vasculitis syndromes that causes DAH in the lungs (more frequently affected) and acute kidney injury (AKI) in the kidneys [1] . The incidence of MPA is about 1 in 100,000 per year, with a slight male predominance and a mean age at onset of approximately 50 years [2,3] . DAH occurs in 11% of patients with MPA associated with extrathoracic involvement.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

The Clinical Significance of a ‘Crazy-Paving’ Pattern on Chest Radiology

Ehab¹,

Braga²

2018

European Journal of Case Reports in Internal Medicine

View full text Add to dashboard Cite

A crazy-paving pattern is a non-specific radiological sign which is characterized by the presence of diffuse ground-glass attenuation associated with interlobular septal thickening and intralobular lines. It was initially described as a pathognomonic sign of pulmonary alveolar proteinosis. However, it can be also found in many other diffuse acute and chronic lung diseases including diffuse alveolar haemorrhage (DAH), a rare and life-threatening clinical syndrome which can be caused by many conditions, the most frequent of these being capillaritis associated with systemic autoimmune diseases.In this case report, we describe an 82-year-old female patient with acute respiratory failure and bilateral pulmonary infiltrates with the characteristic crazy-paving pattern. The final diagnosis was isolated DAH induced by microscopic polyangiitis. The patient was treated with IV high dose prednisolone and cyclophosphamide and was mechanically ventilated. Nevertheless, her clinical status progressively deteriorated and she died after 3 days from acute respiratory distress syndrome.LEARNING POINTSA crazy-paving pattern is a non-specific radiological sign which is characterized by the presence of diffuse ground-glass attenuation associated with interlobular septal thickening.The presence of a crazy-paving pattern together with a progressive fall in haemoglobin levels suggests diffuse alveolar haemorrhage (DAH).A panel for collagen vascular diseases and vasculitis should be immediately performed in patients suspected of having DAH.

show abstract

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

The Clinical Significance of a ‘Crazy-Paving’ Pattern on Chest Radiology

Ehab¹,

Braga²

2018

European Journal of Case Reports in Internal Medicine

View full text Add to dashboard Cite

show abstract

“…In contrast, whenever clinicians encounter alveolar haemorrhage, they should consider the possibility of MPA [14,15]. Clinicians should bear in mind that alveolar haemorrhage may appear in chronic and asymptomatic fashion [16].…”

Section: Discussionmentioning

confidence: 99%

Various patterns of acute alveolar haemorrhage in patients with microscopic polyangiitis: a clinicopathological study of four cases

Yorita¹,

Sakamoto²,

Tsuji³

2018

pjp

View full text Add to dashboard Cite

It is well known that acute alveolar haemorrhage (AAH) is attributed to capillaritis in most cases with microscopic polyangiitis (MPA). In this article, we explore the cause of alveolar haemorrhage in MPA patients. In the present study, we extracted four autopsy cases of MPA with AAH. Patient's sex and age, cause of alveolar haemorrhage, therapy, follow-up duration, and cause of death were investigated. As a result, alveolar haemorrhage was caused by diffuse alveolar damage (DAD) due to candidiasis or influenza virus infection, haemorrhagic infarct due to aspergillosis, capillaritis due to MPA, vasculitis due to cytomegalovirus (CMV), and herpes simplex virus (HSV) infection. All patients received corticosteroid therapy, and one patient additionally underwent administration of cyclophosphamide. The duration of follow-up ranged from one to 26 months with a mean of eight months. All patients died of respiratory failure. In summary, clinicians and pathologists should recognise some causes of alveolar haemorrhage in MPA patients, which include DAD, haemorrhagic infarct, virus-associated vasculitis, or MPA-associated capillaritis.

show abstract

Rapidly worsening pneumonia in a middle-aged woman: a rare diagnosis and a crucial management decision

et al. 2021

View full text Add to dashboard Cite

A 54-year-old Indian female presented to the hospital with a 4-day history of fever, shortness of breath and blood-streaked sputum. Chest radiograph showed consolidation and she was admitted as a case of bilateral bronchopneumonia. She was started on broad-spectrum antibiotics and antivirals but she continued to deteriorate clinically with increasing oxygen requirement and worsening lung infiltrates. Since lab investigations also revealed significant anaemia and renal impairment, vasculitic workup for pulmonary renal haemorrhagic syndrome was ordered even though she had no haemoptysis in the hospital and haemoglobin was stable after initial blood transfusion. High-resolution computed tomography scan was done on day 5 to assess progression and for any clue to diagnosis. It showed extensive bilateral air space consolidation and ground-glass opacities with suggested radiological differential diagnosis of cryptogenic organising pneumonia or pulmonary haemorrhage. Pulmonology team did not recommend bronchoscopy at the time.In view of the clinical features of rapidly progressing pneumonia despite standard treatment, history of haemoptysis, anaemia, renal impairment and CT scan findings suggestive of pulmonary haemorrhage, pulse intravenous Methylprednisolone was initiated. The patient showed marked clinical and radiological improvement. The vasculitic workup later revealed positive myeloperoxidase antibody and a definitive diagnosis of microscopic polyangiitis was made. She was discharged in a stable condition and advised to follow-up in rheumatology where she continues to follow-up 2 years after her initial diagnosis.This is the first case reported in literature, where treatment was initiated for this rare disease entity within a week of presentation based on highly suggestive clinical, laboratory and radiological evidence without waiting for a confirmatory diagnosis. Delay in this rapidly deteriorating patient could have been fatal and our prompt action was crucial in securing a favourable outcome.

show abstract

Microscopic Polyangiitis Causing Diffuse Alveolar Hemorrhage and Rapidly Progressive Glomerulonephritis

Cited by 4 publications

References 12 publications

The Clinical Significance of a ‘Crazy-Paving’ Pattern on Chest Radiology

The Clinical Significance of a ‘Crazy-Paving’ Pattern on Chest Radiology

Various patterns of acute alveolar haemorrhage in patients with microscopic polyangiitis: a clinicopathological study of four cases

Rapidly worsening pneumonia in a middle-aged woman: a rare diagnosis and a crucial management decision

Contact Info

Product

Resources

About