Year : 2004 | Volume
: 14 | Issue : 1 | Page : 17--19
The radiological appearance of diffuse alveolar hemorrhage in good pasture's syndrome
JP Singh, R Shrimali, L Garg, V Setia
Mohan Dai Oswal Cancer Hospital, G.T. Road, Sherpur by pass, Ludhiana, India
J P Singh
Mohan Dai Oswal Cancer Hospital, G.T. Road, Sherpur by pass, Ludhiana
|How to cite this article:|
Singh J P, Shrimali R, Garg L, Setia V. The radiological appearance of diffuse alveolar hemorrhage in good pasture's syndrome.Indian J Radiol Imaging 2004;14:17-19
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Singh J P, Shrimali R, Garg L, Setia V. The radiological appearance of diffuse alveolar hemorrhage in good pasture's syndrome. Indian J Radiol Imaging [serial online] 2004 [cited 2020 May 30 ];14:17-19
Available from: http://www.ijri.org/text.asp?2004/14/1/17/28547
Diffuse alveolar hemorrhage (DAH) is a rare but serious and frequently life-threatening complication of a variety of conditions. DAH may result from coagulation disorders, inhaled toxins or infections. Most cases of DAH are caused by capillaritis associated with systemic autoimmune diseases such as antineutrophil cytoplasmic antibodies-associated vasculitis, anti-glomerular basement membrane disease and systemic lupus erythematosus. Early recognition is crucial because the prompt institution of supportive measures and immunosuppressive therapy is required for survival . Anti-GBM antibody associated disease (Goodpasture's Syndrome) is approximately six times more frequent in males than females and occurs most commonly in second and third decades but has a wide range . Goodpasture's syndrome in an elderly patient presenting alveolar hemorrhage is really unusual. Such a case is being presented here.
A 75 yrs old man came with a h/o hemoptysis for last 10 days and weight loss for last 25 days. On clinical examination there was pallor (++) and pedal edema (+). Investigations revealed Hb 8.2%, Urine albumin (+), Urea 109 mg/dl, Creatinine 2.6 mg/dl. Sputum culture was negative. Chest radiograph [Figure 1] showed patchy bilateral alveolar opacities near the lower zones; there was no cardiomegally, mediastinal widening, pleural effusion or atelectasis. CT examination [Figure 2] showed bilateral alveolar densities sparing the periphery of lung parenchyma. A radiologic diagnosis of non-cardiogenic pulmonary edema was made. Further investigations showed anti-GBM antibodies in serum. A brochoscopic lung biopsy was done which showed hemosiderin-laden macrophages and linear deposition of IgG along the alveolar basement membrane, diagnostic of Goodpasture's syndrome. Renal biopsy however could not be done because the patient refused. Patient was put on prednisolone 40 mg and a repeat chest radiograph was done about 2 months later [Figure 3] that showed almost complete resolution of lung opacities.
Pulmonary hemorrhage is generally due to neoplasm, tuberculosis, necrotising pneumonia or bronchiectasis. If these are not found, Kidney disease, including anti-glomerular basement membrane anti-body-induced bleeding (Goodpasture's syndrome) should be considered . The association between pulmonary hemorrhage and glomerulonephritis was recognized by Good Pasture in 1919 .
Diffuse pulmonary hemorrhage (DPH) is an uncommon condition that is difficult to differentiate radiographically from diffuse pneumonia or pulmonary edema. A triad of clinical and radiographic findings suggests diffuse pulmonary hemorrhage: hemoptysis, anemia and diffuse alveolar consolidation on the chest radiography . Goodpasture Syndrome is the primary disease in only a minority of patients with renal difficulty and hemoptysis. The classical features of goodpasture's syndrome (i.e. recurrent hemoptysis in a young man accompanied by little or no known kidney disease) are not always present .
In 1975, Thomas and Irwin divided DPH into three categories: one in which antiglomerular basement membrane (anti-GBM) antibody was present, a second in which immune complexes were found and a third in which neither could be demonstrated. A somewhat expanded variation of this classification system is shown in [Table 1] .
The chest radiograph usually provides further support for the diagnosis of DPH, but again is nonspecific. It most commonly shows the sudden appearance of a diffuse alveolar filling pattern that is often perihilar or basilar and is indistinguishable from pulmonary edema or diffuse infection such as viral or pneumocystis pneumonia . The radiograph is abnormal in 80% of cases and most commonly shows diffuse bilateral patchy consolidation in the mid and lower zones with sparing of apices and costophrenic angles . Resolution, often with a reticular pattern, is rapid and the radiograph may revert to normal in less than two weeks . However, accentuated vascular markings tend to persist after repeated episodes of bleeding due to presence of siderophages in the interstitium and if the bleeding continued over a sufficiently long period, permanent reticulonodular infiltrates develop, resembling the presence of idiopathic pulmonary hemosiderosis .
Unfortunately, the radiographic abnormalities are never specific for DPH, since a diffuse alveolar filling pattern can be caused by any substance filling the alveoli, i.e, edema fluid (pulmonary edema) or inflammatory exudates (pneumonia). There are no associated radiographic findings (pleural effusion, adenopathy, atelectasis, etc.) that point toward the diagnosis of DPH. The lack of cardiomegally and pulmonary vascular congestion point away from cardiac pulmonary edema .
The course of DPH is usually fatal unless treated. The renal manifestations require treatment with high-dose prednisolone and cyclophosphamide. In patients with severe renal failure, dialysis may be necessary and ultimately transplantation if recovery does not occur. In exceptional cases bilateral nephrectomy has been necessary to control alveolar hemorrhage when all else has failed. Relapse of disease may occur during treatment, when it is more often due to an intercurrent infection than to rise in antibody titer. Once successfully treated, recurrence is rare but has been reported, on one occasion following exposure to hydrocarbon solvents. If relapse dose occur treatment remains the same .
We conclude that if we as a radiologist encounter diffuse, bilateral, patchy, basal consolidation on chest radiograph and alveolar densities with sparing of peripheral lung fields on CT we should keep the possibility of pulmonary hemorrhage in addition to the usual radiologic diagnosis of pulmonary edema of cardiac or non-cardiac origin or diffuse pneumonia as in an immunosupressed patient. Pulmonary hemorrhage can be the cause of hemoptysis even in older age group patients and this knowledge will allow us to suggest the diagnosis of DPH in appropriate situations and help in management of these critically ill patients.
|1||Specks U.Diffuse alveolar hemorrhage syndromes: Curr Opin Rheumatol 2001 Jan:13(1):12-7. |
|2||Watnick S. Morrosion G. Kidny. In: Tierney LM, McPhee SJ, Papadakis MA, editors. Current Medical Diagnosis and Treatment, 40th ed. New York: McGraw-Hill, 2001:916. |
|3||Schwartz EE, Teplick JG, Onesti G, Schwartz AB. Pulmonary Hemorrhage in Renal Disease: Goodpasture's Syndrome and other causes: Radiology 1977 Jan; 122: 39-46. |
|4||Flower CDR, Grainger RG, Armstrong JD, Wilson AG, Hansell DM. Diffuse Pulmonary disease. In: Grainger RG, Allison D editors. Diagnostic: A textbook of Medical imaging,3rd ed. New York: Churchill Livingstone, 1997: 410-11. |
|5||Albelda SM, Gefter WB, Epstein DM, Miller WT. Diffuse pulmonary Hemorrhage: A Review and classification Radiology 1985; 154: 289-297. |
|6||Seaton A. Some less common pulmonary diseases. In: Seaton A, Seaton D, Leitch AG editors.Crofton and Douglas's respiratory diseases 2, 5th ed. London: Blackwell science, 2000: 1330-33.|