Goodpasture Syndrome

date : 27.01.2012

Dilyana Baleva

Diagnostic imaging department " St. Marina” Hospital Varna, Hr.Smirnenski Blvd 1, 9010 Varna

dilianabaleva@yahoo.com

Keywords: Diffuse pulmonary hemorrhage, Pulmonary-renal syndrome, Goodpasture syndrome, Diffuse alveolar changes, ground-glass changes

Clinical history: We present a case of a 22-year-old man, admitted in the pulmology clinic because of recurrent hemoptoe. No abnormal findings at auscultation. Laboratory – increased level of creatinin.

Imaging findings:

On the CXR subtle interstitial changes, ground-glass opacities and tiny patchy opacities are found, which are predominantly distributed in the lower fields (fig.1). On the CT bilateral diffuse ground-glass opacities are found with centrilobular distribution in apical lung segments with a widespread confluent ground-glass areas in the lower lungs. The periphery of lung parenchyma and subpleural areas are spared (fig.2, fig.3). Both kidneys are enlarged with diffusely thickened cortex (fig.4). We focused on the diagnosis of Goodpasture syndrome, which was later confirmed by renal biopsy. We also present images from a CT performed three months later during the course of immunosuppressive therapy. There is evidence of complete resolution of changes in lung parenchyma (fig.5, fig.6). The rapidly progressive course of glomerulonephritis led to endstage renal failure and a hemodialysis started after placement of a central dialysis catheter (fig.7).

Final diagnosis: Diffuse pulmonary hemorrhage in acute glomerulonephritis (Goodpasture syndrome)

Discussion:

Goodpasture syndrome is a very rare disease, with frequency of approximately 1/1.000.000 per year. It presents with a combination of rapidly progressive glumerolonephritis and pulmonary alveolar hemorrhage. Pulmonary alveolar hemorrhage has no pathognomonic imaging presentation. In general it is characterized by alveolar filling syndrome. Depending on severity, hemorrhage is presented as centrilobular ground-glass changes, large confluent areas of ground-glass changes or as alveolar consolidation (when massive intraalveolar haemorrhage is present). Findings are typically symmetrical and costo-diaphragmatic sinuses as well as apical and peripheral lung paranchyma are usually spared. A hint towards the diagnosis is the glomerolonephritic morphology of kidneys (ultrasound, CT, MRI) - bilaterally and symmetrically increased size with a overall thickened cortex, normal drainage and no signs of inflammatory perinephritis. Goodposture syndrome is an autoimmune disease. Its pathogenesis is based on the presence of antibodies against the glomerular basement membrane. A more accurate term, referring this type of glomerulonephritis with or without the presence of pulmonary hemorrhage is Anti-GBM-disease. Treatment is with immunosuppressive medications and in case of acute massive haemorrhage plasmapheresis is applied. Long-term monitoring of renal function markers is mandatory, moreover in advancing chronic kidney insufficiency, long-term dialysis and transplantation are considered.

Differential diagnosis:

A number of other conditions can present with the so-called pulmonary-renal syndrome: ANCA - positive vasculitides, thrombotic microangiopathies of different type, autoimmune rheumatologic diseases (most commonly SLE) and others. Differential diagnosis of diffuse alveolar hemorrhage: autoimmune diseases (Goodpasture's syndrome, systemic lupus erythematosus, antineutrophilic-cytoplasmic-Ab-associated vasculitis), coagulopathies, infections, inhalation of noxious gases. Diffferential diagnosis of diffuse ground-glass changes: 1.Alveolar syndrome in the presence of cardiogenic congestion/edema. Hint for the diagnosis is the presence of cardiomegaly, venous dilatation and septal type interstitial changes (in early stage). 2. Bilateral diffuse inflammatory changes - imaging findings can be indistinguishable. Diffuse ground-glass changes are particularly typical for Pneumocystis carinii and CMV - infections, which in turn occur with increased frequency during immunosuppressive therapy. 3. Acute respiratory distress syndrome (ARDS)

Take home messages:

The presence of diffuse alveolar symmetrical ground glass changes in a patient with hemoptoe should place Goodpasture syndrome as one of differential diagnostic options. Renal morphology assessment helps the correct diagnosis.

Bibliography:

1. Singh JP, Shrimali R, Garg L, Setia V. The radiological appearance of diffuse alveolar hemorrhage in good pasture's syndrome. Indian J Radiol Imaging 2004;14:17-9 2. Collins, J. and E. J. Stern. Ground glass opacity on CT scanning of the chest: What does it mean?APPLIED RADIOLOGY, December 1998 3. Specks U. Diffuse alveolar hemorrhage syndromes. Curr Opin Rheumatol. 2001 Jan;13(1):12-7. 4.Schreiber J, Knolle J, Kachel R, Schück R. Differential diagnosis of diffuse pulmonary haemorrhage. Pneumologie. 2006 Jun;60(6):347-54 5. Spyros A. P.,E. Manali, I.Kalomenidis, Giorgios E K, A. Karakatsani, C. Roussos. Bench-to-bedside review: Pulmonary–renal syndromes – an update for the intensivist. Crit Care. 2007; 11(3): 213.

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