[Chronic obstructive lung disease and alpha-2-macroglobulin deficiency in serum--case report]

The protease-antiprotease concept has contributed substantially to the pathogenetic understanding of different internistic disease patterns including the generalised, histologically mostly panlobular pulmonary emphysema. Attention is presently focused on alpha-1-antitrypsin protease inhibitor deficiency for which substitute solutions are already available. We present a case report on a patient of 40 years of age suffering from a severe, basally located pulmonary emphysema (as was evident on x-ray examination) with respiratory global insufficiency and an obstructive component that was only partially reversible under medication. Laboratory chemistry revealed that this was associated with an alpha-2-macroglobulin deficiency. Possibly the deficiency in this protease inhibitor of low specificity but broad spectrum of action may contribute to a better understanding of some of the types of emphysema that had so far been subclassified as cryptogenic although bearing traits of "proteinase inhibitor deficiency".