Six cases of microscopic polyarteritis exhibiting acute interstitial pneumonia.

Out of 21 autopsy cases of microscopic polyarteritis (MPA), six cases that died of acute respiratory failure are described. All of these six cases exhibited pauci-immune necrotizing glomerulonephritis. Of these six cases, systemic vasculitis and respiratory failure occurred almost simultaneously in two, whereas respiratory failure developed when vasculitis recurred in the other four. Pathologically, pulmonary changes were diffuse and almost uniform in each case. Interstitial inflammatory cells (alveolitis), hyaline membrane, restructuring, and fibrosis were observed. The degrees of these changes differed from case to case; four cases showed predominantly exudative changes and two cases exhibited proliferative or organizing ones. Clinical and pathological features were consistent with acute interstitial pneumonia (AIP). Immunofluorescent and ultrastructural studies did not suggest deposition of immune complexes at the lesions of alveolitis. An etiologic agent could not be identified in these cases. Some lung lesions including pulmonary hemorrhage have been described in association with MPA; however, cases such as those presented here have rarely been reported. It was considered that AIP could be one of the pulmonary disorders in MPA. Furthermore, the possible pathogenetic role of antineutrophil cytoplasmic antibodies (ANCA) in the evolution of present lung lesion is discussed. Although ANCA were not examined in the present cases, these antibodies should be investigated in future cases.

Streptococcal toxic shock syndrome: report of two cases.

Two Japanese cases of streptococcal toxic shock syndrome (STSS) are reported. The first patient was a 45-year-old male who developed necrotizing fasciitis and myositis of the left thigh, refractory hypotension, hepatic dysfunction and acute renal failure; the patient died despite treatment. Streptococcus pyogenes was isolated from the inflamed fascia. The second patient was a 69-year-old female who had coagulopathy, polymyositis and hepatic function abnormality. Streptococcus pyogenes was isolated from blood culture. She was immediately placed on high-dose ampicillin as well as other supportive measures, and she survived.

HTLV-I-associated myelopathy in a patient with chronic renal failure.

A case of human T-cell lymphotrophic virus type I (HTLV-I)-associated myelopathy (HAM) occurring in a 62-year-old Japanese male with a history of hemodialysis and repeated blood transfusions due to chronic renal failure (CRF) is reported. The patient was a lifelong resident of Chiba Prefecture, a nonendemic area for HTLV-I infections. The clinical course was characterized by abrupt onset and rapid progression of neurological signs and symptoms, which appeared to have responded quite well to prednisolone. Although patients with a history of CRF, hemodialysis, and transfusion of blood units which were not screened for anti-HTLV-I antibodies appear to belong to the high risk group for HTLV-1 infections and subsequent development of HAM, reports on such cases have been scanty. Only 3 cases have been reported to date. It appears quite possible that many patients with CRF and HAM remain misdiagnosed.

Coronary artery bypass grafting in a patient with cryoglobulinemia.

Coronary artery bypass grafting (CABG) was performed in a patient with cryoglobulinemia. In order to decrease the concentration of cryoglobulin, the patient underwent double filtration plasmapheresis (DFPP) pre- and postoperatively as well as during cardiopulmonary bypass. Bypass surgery was performed under total cardiopulmonary bypass, moderate systemic hypothermia, and ventricular fibrillation without aortic crossclamping. No adverse effects of cryoglobulin appeared during the peri- and postoperative course. Technical considerations for open heart surgery in patients with cryoglobulinemia are described.

Tubulointerstitial changes in systemic vasculitic disorders: a quantitative study of 18 biopsy cases.

Tubulointerstitial alterations were studied in 18 biopsy cases of systemic vasculitis. To evaluate the changes more objectively, two indexes, the number of interstitial inflammatory cells and tubulitis percentage, were used. The group means of both of these indexes were statistically higher than those of the autopsy control group, while 14 of the cases (77.8%) showed a significant difference (P less than 0.01) between individual means and the control group means. On the other hand, tubulointerstitial changes in systemic vasculitis seemed to be less prominent than drug-induced tubulointerstitial nephritis (TIN) according to these indexes. However, comparing these indexes with those of drug-induced TIN, six cases (33.3%) of systemic angiitis could be regarded as having developed pathologic changes similar to TIN. Statistical difference was not shown in the indexes between those patients with histologic evidence of necrotizing angiitis and those without it. Clinically, urinary N-acetyl-beta-D-glucosaminidase (NAG) was abnormally high in all three examined cases, reflecting the damage of renal tubular epithelial cells. Not only the vascular and glomerular lesions, but also tubulointerstitial changes, should be evaluated in the renal biopsy specimen of systemic vasculitis.

[A case of adrenal black adenoma associated with Cushing's syndrome].

A 36-year-old female was admitted to Asahi General Hospital because of hypertension. Laboratory findings showed elevated levels of urine 17 (11)-hydroxycorticosteroid and blood cortisol. Computerized tomography and ultrasound sonography demonstrated a round tumor at the suprarenal region. The black tumor, 5 X 3 X 2 cm, was removed by left adrenalectomy. Microscopic findings revealed adrenal adenoma consisting of compact cells containing numerous pigmented granules. Postoperative course was uneventful. Hormonal condition was recovered 11 months after operation.

Glomerulonephritis in patients with rheumatoid arthritis (RA). Report of five cases and review of the literature.

We reported five cases of glomerulonephritis developed in the course of rheumatoid arthritis (RA), which had no apparent relation with therapeutic agents. Systemic angiitis was observed in patient 1 and overlapping of systemic lupus erythematosus (SLE) with RA was implied in patient 2, while the other three patients did not show these changes. Renal biopsies were performed in all of the cases and glomerular deposition of immune complexes was suggested by both the immunofluorescent and electron microscopic findings, though the amount was variable. Furthermore, cases of glomerulonephritis in patients with RA were reviewed in the literature and classified into three groups: occurring in association with angiitis, overlapping with SLE or other collagen diseases, and developing without the above two factors. Group 3 was further divided into two subgroups according to immunologic abnormalities. All cases included in the three groups can be regarded as glomerulonephritis of RA. The cases of group 2, however, can more preferentially be considered to be caused by SLE or some other collagen disease that has overlapped with RA. Deposition of immune complexes was suggested in most of the cases examined with immunofluorescence. Various changes were seen in the glomerulonephritis of RA presented here (patients 1, 3, 4, and 5) and reviewed in the literature (groups 1 and 3). Immune complexes might be involved in the pathogenesis of glomerulitis.

Renal vascular lesions in severe hypertension. Transitional changes from benign to malignant nephrosclerosis.

Renal vascular changes in severe hypertension were studied. Twenty-five cases selected from 4,629 autopsies were classified into 2 groups according to the cause of death: group 1 (9 cases died of renal failure) and group 2 (16 cases of extra-renal death). Group 1 had been clinically diagnosed as malignant hypertension and had the hallmarks of malignant nephrosclerosis characterized by arteriolar fibrinoid necrosis and edematous intimal thickening. Group 2 had been clinically diagnosed as benign hypertension and basically exhibited the changes of benign nephrosclerosis. However, about half of the cases of group 2 had arteriolar fibrinoid necrosis, though the lesion was usually less extensive than in group 1. Immunofluorescence revealed similar deposits of immunoglobulins and fibrinogen in the site of fibrinoid necrosis observed in both groups. As for the changes of interlobular arteries, a quantitative analysis disclosed a distinctive difference between groups 1 and 2 with respect to the narrowing ratio of arterial lumina, though edematous intimal thickening was recognized on relatively rare occasions in the distal interlobular arteries in a few cases of group 2. From the results, the problem of transition from the benign to malignant nephrosclerosis was discussed.

Nephrotic syndrome developed in the healing stage of necrotizing angiitis.

A case of necrotizing angiitis is reported. After acute inflammatory changes had subsided, the patient developed the nephrotic syndrome. Postmortem examination disclosed a unique sclerotic glomerular lesion with deposition of eosinophilic material which contained IgM, IgA, C1, and C3. The lesion was distributed in rather selective areas perfused by some of the arcuate arteries.

Different histological manifestation of glomerular lesions in familial systemic lupus erythematosus.

Lupus nephritis in a sister and brother pair was histologically analyzed. The 12-year-old sister showed clinically progressive renal failure and her renal pathology disclosed segmental necrotizing and disorganizing glomerulonephritis. On the contrary, the 14-year-old brother exhibited nephrotic syndrome and his renal biopsy showed diffuse exudative and proliferative glomerulonephritis with segmental membraneous changes. Although the familial incidence of SLE suggests both genetic and environmental factors existing in the background of SLE of identical twins and siblings, the differences of clinical manifestation and histopathological findings imply that there might be little direct genetic and environmental control on determining the expression of the disease.