Alveolar hemorrhage

Introduction Pulmonary hemorrhagic-nephritis syndrome is also known as anti-base glomerulonephritis, Goodpasture syndrome or Goodpasture disease. May be caused by viral infection and / or inhalation of certain chemical substances. It is a serious damage of the glomerular and alveolar wall basement membrane caused by anti-base membrane antibody, clinical manifestations of pulmonary hemorrhage, rapid glomerulonephritis and serum anti-glomerular basement membrane (GBM) antibody-positive triad. Most patients progress rapidly and the prognosis is dangerous.

Cause

Etiology

A clear cause has not been confirmed, but more speculation is related to infection, especially viral infection. It was also reported that there was a history of exposure to gasoline and hydrocarbons (hydrocarbons) before getting sick. Therefore, these chemicals and/or viruses may be considered as causative factors.

[Pathogenesis]

It has been recognized that the principle of renal pathogenesis is the immune response process against basement membrane antibody type nephritis. Due to certain pathogenic factors, the primary lesions of alveolar septum and pulmonary capillary basement membrane, the latter stimulate the body to produce anti-lung basement membrane antibodies, causing a series of immune responses to alveoli under the action of complement. Due to the presence of cross-antigen between the alveolar wall basement membrane and the glomerular basement membrane, the endogenous anti-lung basement membrane antibody can immunoreact with the glomerular basement membrane and damage the glomerulus.

[pathological changes]

Diffuse bleeding on the surface of the lungs, edema and stagnant bleeding on the cut surface. Microscopic examination showed alveolar hemorrhage. The alveolar cavity often engulfed phagocytic cells containing hemosiderin, and the focal alveolar fibrous tissue proliferated. Immunofluorescence showed that the alveolar septum and pulmonary capillary membrane had immunoglobulin and C3 linear deposition, and the pathological changes of the kidney were like rapid glomerulonephritis. In addition, early glomerular capillaries showed focal and segmental necrosis, and lymphocytic infiltration around the glomeruli was characteristic.

an examination

Related inspection

Alveolar-arterial oxygen partial pressure difference (PA-aDO2) chest perspective

1, according to repeated hemoptysis, hematuria, X-ray signs and sputum hemosiderin cells can be diagnosed, pure lung performance should be differentiated from idiopathic pulmonary hemosiderosis. It is easier to diagnose the symptoms of kidney, but it should be differentiated from those with necrotizing vasculitis with pulmonary and renal manifestations and uremia with hemoptysis.

2, pulmonary hemorrhage - nephritis syndrome is mainly characterized by pulmonary and renal syndrome and acute nephritis, so this disease needs to be differentiated from a variety of diseases based on these two manifestations.

Differential diagnosis

Pulmonary and renal syndrome

Diseases that can cause pulmonary-renal syndrome include a variety of pulmonary hemorrhagic-nephritis syndromes, such as ANCA-associated systemic vasculitis, SLE, and nephritis caused by infection. In addition, hemoptysis can also occur in pulmonary embolism caused by renal vein thrombosis and congestive heart failure caused by end-stage renal failure. Ent et al reported that in 2 children, immune complex deposition caused pulmonary hemorrhage and glomerulonephritis. Hernandez reported that a patient with idiopathic Bronchiolitis Obliterans had a progressive nephritis, and histological examination showed extensive deposition of IgA in both the lung and kidney. In immune complex nephritis, glomerular capillaries have granular deposits. Electron microscopy shows electron dense substances, serum anti-GBM antibodies are negative, and circulating immune complexes can be positive, which is not difficult to distinguish from pulmonary hemorrhagic-nephritis syndrome.

2. Lupus nephritis

Patients with this disease with acute nephritis may have symptoms of acute renal failure with pulmonary hemorrhage, which is easily confused with pulmonary hemorrhagic-nephritis syndrome. However, the disease is more common in young women, generally with multiple systemic damage such as skin and joints. Serum immunological examination can help diagnose.

3. Small vasculitis nephritis

Such diseases may have pulmonary hemorrhagic manifestations and approximate pulmonary hemorrhagic-nephritis syndrome. However, the disease is more common in middle-aged and elderly people aged 50-70 years. It has obvious systemic symptoms such as fatigue, hypothermia, and weight loss. Anti-neutrophil ytoplasmic antigens (ANCA) are positive. Among them, Wegener's granulomatosis may be interstitial inflammation, and both may exist at the same time.

In many vasculitis, there are two types of small vasculitis, Wegener granulomatosis and microscopic vasculitis. The target antigens of these two small vasculitis are Proteinase 3 and Myeloperoxidase, respectively. Antibodies (c-ANCA and p-ANCA) are the original sin antigens that cause small vessel damage and have important diagnostic value for small vasculitis. In Wegener granulomatosis and microscopic vasculitis, the upper and lower respiratory tract and kidney are most often involved. Wegener granulation The morphological changes of the swelling are various, and the ulceration changes mainly in the oropharynx, paranasal sinus, trachea, etc., and the granulomatous changes are optional. Therefore, histological examination, especially small biopsy Wegener granulomatosis cannot be easily ruled out. Wegener granulomatosis and microscopic vasculitis can be characterized as focal segmental necrotic glomerulonephritis in the kidney, often accompanied by crescent formation, within the glomerulus. Immunoprecipitation is rare.

4. Acute nephritis with left heart failure

The disease may have blood stasis and dyspnea, similar to pulmonary hemorrhagic-nephritis syndrome, but the disease is more common in adolescent patients. There is a history of streptococcal infection, often due to severe hypertension, water and sodium retention, edema, congestive heart failure. Renal biopsy can be identified.

5. Rapid progressive nephritis

The immune pathogenesis of acute nephritis (crescent nephritis) In addition to anti-GBM nephritis, immune complex nephritis and cellular immune vasculitis can also cause typical crescentic nephritis and acute renal failure.

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