In this article – which describes the historical journey from Bright’s Disease to Chronic Kidney Disease (CKD) – we will demonstrate how from the earliest times, future and current nephrologists have tried do create meaningful classification systems of what we now call CKD.
Let’s start by going back to 18th Century Europe.
The Italian physician Giovanni Battista Morgagni (1682–1771; from Padua) was a famous anatomist of Europe of this period. In 1761, he published the most important work of his life, the masterpiece in pathological anatomy, De Sedibus. Its full title was ‘De Sedibus, et causis morborum per anatomen indagatis libri quinque (or, ‘On the Seat and Cause of Diseases Shown by Anatomy’). The text is based on his pathological observations from about 700 autopsy dissections of patients whom he had treated during their lifetime.
Giovanni Morgagni
Morgagni linked the anatomic findings at autopsy, specifically atrophied kidneys, with the signs and symptoms of a disease now known as uraemia.
The object was to correlate patterns of findings in the ill person with localised structural abnormalities deep within the autopsied body. Classifications derived from morbid anatomy would replace nosographies based only on patients’ symptoms.
Richard Bright, a major figure in this movement, added an early laboratory manifestation, albuminuria, to the complex. His initial 1827 publication, the Reports of Medical Cases, based on 24 cases, suggested three forms of deranged kidney structure, accompanying albuminuric dropsy. The first was a kind of softening with yellow mottling.
The second form was one in which “the whole cortical part is converted into a granulated texture…” The third “is where the kidney is quite rough and scabrous to the touch externally, and is seen to rise in numerous projections not much exceeding a large pin’s head … [and there is a] contraction of every part of the organ ..”
These three descriptions hold little meaning for the nephrologist of the 21st century, who rarely sees or touches a fresh diseased kidney. Bright suggested the three forms might be stages of one process; though he seemed to favour three categories for a classification. So from the first publication on the disorder, Bright’s disease was not held to be one specific entity, not even by Bright.
Although not all subsequent physicians and pathologists would agree that Bright’s disease was inflammatory in nature (as did Bright), the term “nephritis” entered use by 1840 for pathological classification – though the broad category of illness continued to be called Bright’s disease. The story of the superseding and competing classifications is far too complex to explore except in the broadest of strokes. The microscope came to supplant gross observation and the touching of the renal surface.
The great pathologist and theorist Rudoph Virchow (1821–1902) in 1858 suggested “parenchymatous nephritis,” “interstitial nephritis,” and “amyloid degeneration.”
Briton George Johnson in 1873 proposed the separation of an acute form (“acute nephritis”), and three chronic varieties: “red granular kidney,” “large white kidney” and “lardaceous kidney” (which is the same as amyloid kidney).
William Osler (1849–1919) in his popular text ‘The Principles and Practice of Medicine’ favoured “acute Bright’s disease,” “chronic parenchymatous nephritis,” and “chronic interstitial nephritis.” Amyloid was dispatched to its own pathological category.
Into the 20th century, the extremely influential monograph by Franz Volhard (1872–1950) and Theodor Fahr (1877–1945) published in 1914, Die Brightsche Nierenkrankheit, provided a fresh – but still trinitarian – organisation: degenerative diseases, the “nephroses”; inflammatory diseases, the “nephritides”; and arteriosclerotic diseases, the “nephroscleroses.”
Thomas Addis of Stanford University in the 1920s offered a modification of this last framework which gained some popularity: “haemorrhagic Bright’s disease,” “degenerative Bright’s disease,” and “arteriosclerotic Bright’s disease.” It is significant that an arteriosclerotic category did not appear in the early 20th century; probably as a reflection (at the time) of the lack of understanding of atheroma and its manifestations.
Today, we find utility in thinking in terms of glomerular disease, tubulointerstitial disease, and vascular disease; but when appropriate seek a specific, causal diagnosis using biopsy or detection of marker molecules in blood or urine. It is interesting that a tripartite (pathological) classification has been favoured through the centuries. Though the modern CKD classification has 5 classes. However stages CKD1-2 are more like ‘risk factors for CKD’. They have been created largely to encourage primary care physicians to attempt to prevent ‘proper CKD’, i.e. that may eventually lead to dialysis and transplantation.
Of interest here, chronic as applied to renal disease for a long time referred to the pathological appearance more than to a defined clinical course. Chronic in good part meant sclerosis or fibrosis. Now, a diagnosis of CKD emphasises a uniform clinical picture and implies a disinterest in underlying structure. ‘CKD’ repudiates pathology.
This article is based on Chapter 2 in ‘Chronic Renal Disease‘ (2nd Edition; 2020), by Steven J. Peitzman.
Last Reviewed on 3 July 2024