Lardaceous Degeneration of the Intestines

SYNONYMS.—Albuminoid degeneration, Waxy degeneration, Amyloid degeneration, etc.

Lardaceous degeneration of the intestines is an affection of quite frequent occurrence in those persons who are the subjects of a like change elsewhere; for although it has been asserted that it may be present as a primary affection, it almost always succeeds the same form of degeneration in other organs. Since, for the most part, it only makes itself manifest at an advanced stage of the disease, its importance is usually masked by the grave constitutional condition of the patient, whose vital forces are wellnigh exhausted by the already advanced degenerations present elsewhere. The extensive implication of other organs and tissues in the same degenerative process also creates great obscurity in the symptomatology of intestinal lardaceous disease, and is doubtless the cause of the existing dearth of definite knowledge upon the subject.

That the intestines are comparatively frequently involved in lardaceous disease is shown by dead-house statistics. Thus, Charlewood Turner reported from the London Hospital that in 58 cases of lardaceous disease the intestines were affected 10 times; and Goodhart in 150 consecutive necropsies of lardaceous disease at Guy's Hospital reported implication of the intestines 63 times.

Transactions Path. Soc. London, 1879, p. 517.

Ibid., p. 533.

Although the bowels do not become affected as early as several other parts, they will almost certainly become involved should the patient's life be prolonged; and in those cases where death is a direct result of the degeneration the intestines share with the kidneys the chief responsibility. It is not, however, until an advanced stage of lardaceous degeneration that its presence in the alimentary canal is revealed by symptoms; indeed, many cases do not, throughout life, betray evidences of the pronounced alterations that are to be discovered after death. Even in extreme cases there are no symptoms that would, even with probability, be referred to lardaceous disease of the bowels in the absence of the same degeneration in other organs and parts. There are, then, no specific symptoms following lardaceous degeneration of the bowels.

Where the normal functions of the intestines can no longer be properly performed in consequences of the changes that have taken place in them, there results a moderate diarrhoea. At first the number of movements may not be increased; the evacuated matters are fluid and of a greenish or pale color. Usually, little or no pain is experienced, though at times and in certain individuals this may be severe and colicky. The diarrhoea is not always steadily progressive, but may from time to time disappear. With the progress of the disease it may become more free and persistent, and in the later stages hemorrhage from the bowels may be superadded. This may vary in amount, and where, as is often the case, the stomach participates in the degeneration, hæmatemesis may also appear. These hemorrhages may be insignificant, or may at once assume alarming proportions, and even bring to an unexpected termination the life of the individual. Already, at the outset of the intestinal symptoms, the general health will have shown evidences of profound alteration, but upon the supervention of the diarrhoea more rapid progress will be observed, consequent upon the increased nutritive disturbance. The use of remedies in temporarily controlling this diarrhoea may prolong for months the life they are powerless to save.

So far as concerns the intestinal affection, there is no special tendency toward febrile excitement. It must not be forgotten, however, that acute inflammatory attacks of various tissues and organs frequently arise in the course of lardaceous disease.

Though there seems to be reason to believe that mild degrees of lardaceous degeneration may sometimes be cured, especially when dependent on syphilis, there is but little hope of arresting its progress at the late stage when the bowels become implicated. Indeed, when pronounced degeneration of the bowels takes place the disease is usually nearing the end of its course; for it is a well-settled fact that in this degeneration extensive implications of organs may occur without markedly reducing the patient's general condition, so long as the kidneys and intestines remain unaffected. The cause of death is usually to be traced to these organs. Dickinson found that in 35 cases where death was apparently due to renal lardaceous disorder, the immediate result was brought about by diarrhoea in 13 cases. Presumably, in a large proportion of these lardaceous disease of the bowel was present.

Diseases of Kidney, Part ii., 1877, p. 496.

The degeneration usually affects the lower portion of the small and the upper part of the large intestine. Occasionally it will be found to have invaded the whole alimentary tract. As in lardaceous degeneration generally, the process begins in the small arteries and capillaries and veins, affecting primarily the arterial and venous muscular coats—not, however, according to the latest authorities, the muscular fibres themselves, but their perimysium and the cement substance, the degeneration being one limited to the connective tissues. In the mildest cases only some of the small vessels of the mucous membrane are involved, and no naked-eye changes can be detected. In more advanced stages the mucous membrane is pale and shows evidence of catarrh. Thickening occurs, and as the process advances a peculiar appearance is revealed which has been compared to that of wet wash-leather (Wilks). The iodine test now gives the mahogany-colored reaction of lardaceous matter, with the tissues affected, or, if the methyl-aniline-violet test of Cornil be employed, the lardaceous material will display a red-violet color, while normal structures will be tinged blue-violet. It is said to be better to make the test near a Peyer's patch, since the latter is seldom affected by the degeneration, and brings out, by contrast, the surrounding lardaceous material. This distribution of the material cannot be considered as constant, however, since Hayem found the patches of Peyer most frequently affected.

Cohnheim, Allgem. Path., 1882, p. 667.

Wilks and Moxon, Path. Anat., p. 404; Kyber, Virchow's Archiv, Bd. 81, H. 1 and 2.

In more advanced stages the surface may become irregular from glandular enlargement, and ulceration may occur. Microscopic examination shows the lardaceous material in the vessels, and also in the stroma of the mucous membrane and villi. The epithelium is not involved. The degeneration, at first confined to the mucous membrane, extends to the submucous tissue, the proper muscular coat of the intestines being often implicated—so far, at least, as concerns its connective tissue. In the more severe cases Hayem found the agminated and solitary glands extensively involved. Fine branches from affected vessels penetrate to the interior of the glands. In such cases the mesenteric glands will be found implicated. The degeneration of the vessels running through the gland structure causes disappearance of this substance by fatty degeneration, and occasions a reticulated arrangement of the lardaceous material, and, secondarily, ulceration. In a similar manner ulcers may arise in any part of the affected tract. Finally, the lardaceous material may involve the whole thickness of the gut.

Eberth, Virchow's Archiv, 80, S. 138.

The diagnosis of lardaceous disease of the bowels can only be made with certainty in the presence of pronounced albuminoid disease of other parts in association with the symptoms of intestinal disorder. It possesses no characteristic symptoms.

Inasmuch as the disorder invades the bowels only at a late stage of its existence, the prognosis acquires additional gravity. It is probable that advanced albuminoid disease is never cured; so much the more hopeless is it when affecting this tract. If unchecked, the diarrhoea rapidly saps the powers of life; if temporarily alleviated, the approach of death is more gradual.

Whatever attempts are to be made to cure the disease, they must be through the general system, and are identical with those directed toward the cure of lardaceous disease generally. Treatment directed to the intestines must be palliative. The diarrhoea must be combated by appropriate diet and the administration of such remedies as protect the surface of the mucous membrane and control the intestinal movement. Bismuth subnitrate in large doses is therefore indicated. Various astringents may be employed, while the use of opium often secures most gratifying relief. It should be given in generous doses. Preparations of the crude drug seem to answer better than its salts. The necessity of keeping the gut free from undigestible matters that may irritate the already badly-damaged mucous membrane is apparent. Patients with this form of lardaceous degeneration usually show the cachexia resulting from profound modifications of nutrition, and their intestinal symptoms can only be regarded as links in a long pathological chain. Hemorrhage will call for remedies that under ordinary circumstances are employed to control bleeding from the bowels.