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Intestinal biopsies from 146 patients with adult coeliac disease and 13 patients with intestinal villous atrophy of different aetiology were assessed for the presence of subepithelial collagen and compared with a group of 20 control subjects. Subepithelial collagen was a common and non-specific finding observed in intestinal biopsies from patients suffering from adult coeliac disease (36%) and tropical sprue. In adult coeliac disease the described subepithelial changes usually regress following treatment, though marked subepithelial collagen deposition may indicate a poor prognosis. The study showed that the presence of marked subepithelial collagen in a flat jejunal biopsy does not define a separate clinical entity.

Although cardiac involvement in Whipple's disease has been reported previously, the extent and significance of such involvement has not been stressed. In our series of 19 patients, 58% had clinical cardiac findings and 79% had gross cardiac lesions at autopsy. Histologically there were PAS-positive macrophages in the pericardium, myocardium, and valves of each of these patients. These collections of macrophages were associated with chronic inflammatory cells and foci of fibrosis with resultant adhesive pericarditis, focal myocardial fibrosis similar to the fibrosis of idiopathic cardiomyopathy, and valvular fibrosis with deformity grossly resembling the valvular lesions of chronic rheumatic heart disease. Four of the patients with mitral valvular deformity had cardiac murmurs, and ECG changes were noted in six patients with mitral myocardial fibrosis. In addition, pericardial friction rubs were heard in two patients. By electron microscopy rod-shaped bodies, presumably bacteria, were noted in the mitral valve and myocardium. The structures are identical to those described in the small intestinal mucosa of patients with Whipple's disease and presumably are the causative agent of the pancardiopathy.

A man of 22 with Whipple's disease suffered from an acute febrile state and unconsciousness. Focal inflammatory lesions accompanied by large numbers of peculiar macrophages occurred in thalamus and basal ganglia. The electron microscopic findings conform to previous reports on brain involvement in Whipple's disease.

Berichtet wird uber den ungewohnlichen Verlauf eines Morbus Whipple. Unter antibiotischer Therapie tritt klinisch eine vollige Beschwerdefreiheit ein. Mehrfache Kontrollbiopsien zeigen eine hochgradige und progrediente Kollagenisierung des Stratum proprium mucosae, die besonders auch pericellular und im Bereich der epithelialen Basalmembran entwickelt ist. Lymphocyten, Plasmazellen und interepitheliale lymphoide Rundzellen sind stark vermehrt. Immunologisch ist Serum-Komplement (C3-Fraktion) erniedrigt. Immunhistologisch findet sich nach Inkubation mit Patientenserum eine cellular-cytoplasmatische Fluorescenz mit Anti-Komplement von subepithelial und interepithelial gelegenen Zellen. Die Befunde werden im Sinne eines hyperreaktiven Vorganges, der moglicherweise zur Induktion eines cellularen Immunmechanismus gefuhrt hat, diskutiert.

Although Whipple's disease is clinically recognized for its features of arthritis and diarrhea, pericarditis and cardiac valvular disease are frequently present and a significant number of such patients die suddenly and unexpectedly. This report includes special postmortem cardiovascular examinations in a 55-year-old man who died of Whipple's disease. Pericarditis and valvular disease were extensively present. There was also focal myocardial degeneration, including the sinus node, A-V node and His bundle. Typical Schiff-positive bacilli were found in the tunica media and endothelium most numerously in the small coronary and hepatic arteries, less in splenic and renal arteries, and least in small pulmonary arteries. Large coronary arteries and the aorta were not involved. Three stages of bacterial invasion of the arteries included a noninflammatory phase in which the bacilli were most numerous, a pancreatic phase in which bacilli were distinctly less numerous, and a healed scarring of arteries previously damaged. The combination of pericarditis, valvular disease of the heart, coronary and systemic bacterial arteritis and focal myocardial degeneration and myocarditis is unusual for Whippl'e disease. Studies of other cases are warranted to determine whether bacterial arteriopathy and arteritis have previously been overlooked or if the present case is unique. Evidence that the conduction system of the heart may be involved indicated that careful attention to cardiac rhyth and conduction is merited in future studies of patients with Whipple's disease.

AS an unusual cause of malabsorption, Whipple's uf disease (intestinal lipodystrophy) has excited the interest of clinicians because of the diffuse manifestations, the progressive clinical course and speculation over possible pathogenesis. The typical clinical picture is that of a debilitating disease manifested by abdominal pain, steatorrhea, gastrointestinal bleeding, lymphadenopathy, arthritis and pigmentation that, without treatment, usually leads to death within twelve months of the diagnosis. Diagnosis is based on the histologic demonstration of an accumulation of macrophages filled with cytoplasmic inclusions that give a positive periodic acidSchiff (PAS) reaction. These macrophages are present in the small-bowel mucosa, peripheral lymph nodes . . .

Two patients with Whipple's disease are described in whom the diagnosis was established by peroral small intestinal biopsy. Scurvy occurred in one patient.

Es werden klinische und neuropathologische Befunde von zwei Beobachtungen von Morbus Whipple mit Befall des ZNS mitgeteilt. Bei beiden Patienten war das klinische Bild von einem schweren organischen Psychosyndrom bestimmt; bei dem einen wurden zusatzlich Blickparesen und Myoklonien beobachtet. Die cerebralen Symptome waren 10 Monate, bzw. 2 Jahre vor dem Tode aufgetreten. Die extracerebralen Symptome (rezidivierende Schube einer Polyarthritis, Entzundung der serosen Haute und bei einem auch intermittierende Fieberschube) hatten bereits 10, bzw. 20 Jahre vor dem Tode begonnen.

SummaryA malabsorption syndrome denoted by jejunal lipodystrophy, steatorrhea, and osteomalacia has been produced in adult cotton top marmosets (Saguinus oedipus) fed a high cholesterol, high coconut oil containing diet. The diet included sufficient amounts of all presently known nutritional essentials for this primate. The lipodystrophy was marked by huge collections of neutral fat in the absorptive cells of the jejunal villi. The fat filled cells apparently created an absorptive block which contributed to the development of steatorrhea and osteomalacia.

A duodenal biopsy from an untreated patient with Whipple's disease was studied by light and electron microscopy. The specimen contained innumerable instances of binary fission of the rod-shaped bodies characteristic of the disease. The findings add further support for a bacterial concept of Whipple's disease.

A 50 year old man lost 50 lb, passed bulky stools, and complained of joint pain. He was pigmented and had large nodes. PAS dye stained granules in mesenteric and axillary nodes and macrophages in the jejunal mucosa. Antibiotic treatment resulted in remission of symptoms.

This brief review indicates the large variety of gastrointestinal and hepatic diseases in which fever may occur and be a puzzling symptom to explain or a symptom of prognostic importance. In the differential diagnosis of fever, consideration should be given to a possible gastrointestinal cause. In patients with known liver disease fever not only introduces the need for searching diagnostic studies but also for possible revision of the prognosis.

As A test of your awareness of radiographic patterns, attempt to list at least six entities which could have the appearance demonstrated in Figures 1 through 3 above. Figures 1 and 2 represent our own patient. Figure 3 represents a patient with a more severe form of the same condition and is used here to emphasize characteristic radiographic features of the disease. Radiological Findings Small to medium-sized nodules with ill-defined borders are dispersed throughout both lungs (Figs. 1–3). A close-up view of the right lung in Figure 1 (Fig. 2) confirms that the borders of the nodules fade imperceptibly into the surrounding lung tissue and that several nodules in the mid-lung fields are actually confluent. We have labelled such findings the “shaggy nodule pattern.” The nodules in Figure 3 are similar, but many of them also show cavitation. The pleura, node-bearing areas, heart, and bone structures are unremarkable. Radiological Discussion The indistinct margins of the multifocal pulmonary nodules indicate ...

A middle-aged white male presented with weight loss, low grade fever, and increasing abdominal girth. A small right-sided pleural effusion and moderate ascites were noted on physical examination. The pleural fluid reportedly contained an acid-fast bacillus, but it was sterile. The ascitic fluid was a sterile exudate. Following antituberculous therapy his pleural effusion and ascites resolved. Laboratory studies returned to normal. When streptomycin was discontinued, while isoniazide and p-aminosalicylic acid were continued, his previous symptoms, abnormal physical findings, and deranged laboratory studies recurred. Light and electron microscopy of peroral small bowel biopsy established the diagnosis of Whipple's disease. Peritoneoscopy, prior to starting any therapy, disclosed multiple raised nodules over the upper parietal peritoneum. Light microscopic examination of biopsies of the peritoneal nodules revealed diastase-resistant periodic acid- Schiff-positive material. After 7 months of tetracycline therapy, the nodules almost totally resolved. The presence of exudative ascites and multiple small peritoneal nodules or both should alert the clinician to the possibility of Whipple's disease.

The pathologic changes in the brains of two cases of Whipple's Disease have been described. The lesions are considered specific and, thus, constitute additional evidence of the systemic nature of the disease.

Whipple's disease is a systemic condition characterized by diarrhea, intestinal malabsorption, abdominal pain, lymphadenopathy, and a polyarthritis, which may antedate the other symptoms by several years. In the first forty-two years after Whipple's original description of this disease in 1907 (10), only ten references to it could be found in the English literature, but more recently it has attracted a great deal of attention, and in the past decade it has been the subject of over fifty articles. Very little, however, has been written about the detailed radiologic features (3, 6, 8). The present report is concerned with 3 cases of Whipple's disease, with special attention to the radiologic findings. One of these cases, originally published by Jones, Benson, and Roque (5), is of particular interest because of reversion of a grossly abnormal small-bowel roentgen picture to normal following administration of steroids and ACTH. The eight-year remission in this instance is the longest yet recorded. Only 2 othe...

A diagnosis of Whipple's disease was made by peroral jejunal biopsy in a 48 year old woman with pleuropericarditis of unknown etiology who had been followed for 4 years prior to the onset of clinical and laboratory signs of malabsorption. Pretreatment immunologic investigations revealed lymphopenia, decreased immunoglobulin G (IgG) with normal immunoglobulin A (IgA) and M (IgM) serum levels, and marked impairment of delayed hypersensitivity. Following successful treatment, the total lymphocyte count and serum IgG level returned to normal, and there was no demonstrable impairment in delayed hypersensitivity. This latter observation is in contrast with recent reports of immunologic investigations in patients with Whipple's disease which demonstrate that most patients continue to manifest significant deficits in delayed hypersensitivity despite successful therapy.

Whipple's disease (intestinal lipodys­ trophy), from the time of its first descrip­ tion in 1907/ was considered, universally, to be a fatal disease that could be diagnosed only by laparotomy or at autopsy until about 1950. Such concepts are now known to be erroneous. The rapidly growing literature, during the preceding 10 years, clearly in­ dicates a renewed interest in this unusual pathologic entity. This is largely due to the ease of diagnosis once suspected, to new research techniques, especially peroral small bowel biopsy and electron microscopy, and to the gratifying response to appropriate treatment. Recent reviews by Gross,2 Eng­ land,3 and HoW emphasize the inconsistent therapeutical effect of adrenocorticosteroids and antibiotics and leave one uncertain as to which of these agents was responsible for the recovery observed. Although clinical remission after treatment has been reported in a number of patients, the period of follow­ up has been too brief to justify the conclu­ sion that complete recovery had taken place. In fact, entirely normal tissue obtained by small bowel biopsy after therapy has been reported in only 3 eases. 4 - 6 The purpose of this report is to review our experience in the treatment of this disease with steroids and antibiotics, to show the extent of the clinical, biochemical, radiologic, and histo­ logic remission observed in our patients and finally to present data whieh would allow one to form an opinion as to which of these agents was responsible for recovery.

Only in the past three or four years has it been recognized that Whipple's disease (intestinal lipodystrophy) can be successfully treated with long-term administration of antibiotics. From the time of Whipple's initial description in 1907 (17) until the past decade, the disease had been considered invariably fatal, although Paul-ley (11) as early as 1952 reported a case of Whipple's disease which responded to tetracycline therapy. There were also a number of reports of long-term remissions attributed to cortisone (15). It was not until 1960 (5), however, that the very impressive results of long-term antibiotic therapy were reported. Subsequent articles (1, 14) have substantiated this observation, and it is our opinion that no one should die of Whipple's disease if it is correctly diagnosed and treated. The major clinical manifestations of Whipple's disease are diarrhea, steatorrhea, arthralgias, increased skin pigmentation, lymph node enlargement, and serous effusions. Although these symptoms obviously re...

A typical case of Whipple's disease is reported, substantiated by histopathologic study. Treatment with steroids has been used with good results. A prolonged clinical remission has occurred, although persistance of pathologic changes in the small intestine has been demonstrated. This disease is a generalized one with polyserositis and malabsorption, the etiology of which is unknown. A genetic factor has been suggested, with the presence of a basic defect in intestinal cell absorption. That it may represent a general sensitivity reaction with inflammation, is possible. It may be amenable to steroid therapy.