Amyloidosis of the Lower Respiratory Tract
Authors: C. B. Prowse
Journal: Thorax (1958)

This paper reports two new examples of a rare form of amyloidosis affecting the lower respi- ratory tract, and reviews previous cases.A short survey of amyloid disease is included.The cases under consideration belong to the localized tumour-forming variety of the disease and do not include examples of the primary systemic form in which bronchopulmonary deposits are present.HISTORY Rokitansky ( 1842) is generally credited with the first account of lardaceous disease although it had been observed long before.Jeremiah Wainewright gave an unmistakable description of it in 1737, as did John Abercrombie in 1828.Lardaceous disease became established as a clinical entity between 1850 and 1888, with several reported series of sufferers, notably 83 by Dickinson (1879), 58 by Charleswood Turner (Dickinson, 1879), 36 by Samuel Wilks (1856), and 244 by Hilton Fagge (1876), who analysed necropsy records of 20 years at Guy's Hospital.Virchow (1858) was the first to describe the minute pathology of the condition, which he named amyloid disease.In 1886 Wild described a woman with amyloid *deposits of unusual distribution.The heart, intestines, tongue, peritoneum, urinary bladder, and lungs were chiefly affected, whereas the spleen, liver, and kidneys were completely free from deposits.Furthermore there was no antecedent chronic sepsis.Wichmann (1893) referred to local and generalized forms of amyloidosis, and Lubarsch (1929), reporting three cases resembling that described by Wild (1886), classified the disease into typical, common, or secondary, and atypical, primary, or systematized forms, suggesting diag- nostic criteria.Primary amyloidosis was first recognized clinically by Reimann, Koucky, and Eklund in 1935; they classified amyloidosis under four headings, namely, (1) primary, (2) secondary, (3) tumour-forming, and (4) associated with multiple myeloma, but they commented that the groups overlapped, particularly the first, third, and fourth.Group 4 they regarded as secondary, in spite of a resemblance to the primary form in the character and distribution of the amyloid deposits.Tumour-forming amyloidosis they regarded as primary in type and characterized by small, solitary, or multiple tumours in the eye, bladder, urethra, pharynx, tongue, and, especially, the respiratory tract.Primary amyloidosis is rare, and in 1950 Higgins and Higgins could find records of only 71 authentic cases, including one of their own.These 71 cases do not include examples of the primary tumour-forming variety of the disease.Burow described an amyloid tumour of the larynx in 1875, and in 1883 Balser reported a man who had "tracheo-and broncho-stenosis with amyloid in the wall of the airway."Kramer and Som, reviewing the literature in 1935, found reports of 95 cases with primary tumour-forming amyloidosis of the respiratory tract, in 36 of which the larynx alone was involved, and with the tongue in another eight; the trachea was affected alone in 13 cases, and with a bronchus in another four; the lung alone in four cases.Stark and McDonald (1948) and Stark and New (1949) found 15 out of 28 cases in the Mayo Clinic records up to 1949 in which they were able to confirm the diagnosis, and among these the larynx was involved in eight, in one of which a bronchus was also affected.More recently Schottenfeld, Arnold, Gruhn, and Etess (1951) in America and Whitwell (1953) in England collected the records of primary tumour-forming amyloidosis of the lower respiratory tract.Five of Schottenfeld's cases, however, had amyloid deposits only in the larynx and trachea, or the trachea by itself, and because no bronchus or lower part of the respiratory tract was involved, these have been ex- cluded from the present review.Schottenfeld and others described one new case (1951), Noring and Paaby (1952), Schmidt, McDonald, and Clagett (1953), and Gordon (1955) one each, and Whit- well 10, from the earlier literature as well as one of his own.Gordon referred to two cases recorded by Lunzenauer (1952), who found des- on July 11,