A Rare Case of Primary Amyloidosis, Presenting with Severe Pulmonary Hypertension and Bilateral Pleural Effusion.

Pulmonary arterial hypertension secondary to vascular deposition is a rare presentation of Primary Amyloidosis. The natural history of pulmonary hypertension and concomitant right-sided cardiac failure is not very well understood and is associated with a very short median survival. This syndrome is a marker of advanced Amyloidosis Persistent pleural effusion is another much rarer presentation of pulmonary involvement in Primary Amyloidosis and is secondary to pleural infiltration, which sometimes is difficult to distinguish from the cardiomyopathic causes. The incidence of pleural effusion in primary Amyloidosis is 1–2%. This syndrome also has a very poor prognosis, and has limited response to treatment, however, pleurodesis has been temporarily useful in some cases.

Case report: A 74 year old African American female, non-smoker, with history of hypertension, who presented with shortness of breath and persistent productive cough and 18 pounds weight loss for the last six months. The initial chest x-rays showed bilateral pleural effusion and a transudate fluid on analysis. Cardiac workup for an atypical chest pain revealed moderate to severe pulmonary hypertension without myocardial infiltration on 2-D echocardiogram, and normal coronary arteries on cardiac catheterization. Extensive workup did not show any secondary cause of pulmonary hypertension, however, the patient gradually developed nephrotic range proteinuria and kidney biopsy showed infiltration of 10 nm fibrils, involving 3+ segmental mesangium, and 2+ segmental glomerular basement membranes. This was consistent with renal Primary Amyloidosis, AL Lambda type. A random urine and serum sample for immunofixation also revealed an M- spike of 23 mg/dl in gamma region. A concomitant bone marrow biopsy revealed presence of monoclonal IgG lambda plasma cell (CD38 bright) population, without any B-cell or T-cell markers.

Conclusion: Pulmonary hypertension and pleural effusion are two rare and lethal presentations of pulmonary involvements in Primary Amyloidosis, and it should be considered in the differential diagnosis of any patient presenting with pulmonary symptoms. There is no definite treatment of these syndromes, although combinations of steroids and melphalan, along with colchicines, vincristine, adriamycin, and thalidomide have been used with minimum satisfactory outcomes. Autologous stem cell transplantation (SCT) provides the opportunity to give higher doses of chemotherapy and may be of benefit in some cases.