A newly recognized type of familial glomerulopathy observed in patients of both sexes in six families is reported. Proteinuria, often within the nephrotic range, microscopic hematuria, hypertension and a slowly decreasing renal function over several years were common. No underlying systemic diseases were identified. Generally, light microscopy showed enlarged glomeruli with minimal hypercellularity and with extensive deposits in the mesangium and subendothelial space. By electron microscopy, granular deposits with some admixture of fibrils were most common. In one family, the deposits were predominantly fibrillary. Immunoglobulins and complement factors were inconstant or lacking. A main finding was a strong immune reactivity to fibronectin, corresponding to the distribution of the deposits. In one patient, the deposits recurred in a renal transplant. There was no indication of systemic deposition. Abnormalities in the metabolism of circulating fibronectin may play a pathogenetic role in this disease of probably autosomal dominant inheritance.

A detailed analysis of 60 autopsy cases of AN is presented. Renal papillary necrosis is the hallmark of AN. Special emphasis is given to early changes in the basement membranes and the ground substance of the renal papilla observed by light and electron microscopy. The "interstitial nephritis" in the renal cortex is secondary to papillary changes. The histologic picture of the cotex is often complicated by bacterial infection and, therefore, is complex. An unusual hyalinization of small vessels beneath the urothelium of the mucosa of the urinary tract, similar to that seen in the inner medullary zone, has been called "analgesic microangiopathy". Its significance is not clear. The renal papillae, the mucosal membranes of the urinary tract, the liver, the cartilages, and the skin often display a striking brownish discoloration caused by a lipid-containing pigment. Statistical data from out autopsies in Switzerland show no decrease in death rates of AN. This is in contrast to reports from Denmark and Sweden where the sale of phenacetin has been restricted by prescription.

Proteinuria and microhaematuria were observed in three siblings and one first-degree cousin. Histological examination of three kidney biopsies and one autopsy specimen shows the same diffuse glomerular lesions in all patients, characterized by mainly subendothelial but frequently transmembranous and mesangial deposits of a unique fibrillar structure, visible by electron microscopy. Examination by immunfluorescence gave inconstant findings. No serological abnormalities could be established. To our knowledge, such a pecular form of familial glomerulopathy has not been described so far.

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A 19-year-old patient was treated who developed a life-threatening pulmonary insufficiency after having suffered from a flu-like illness for 13 days. Lung biopsy revealed an early stage of idiopathic pulmonary fibrosis. Chemotherapy by prednisone alone proved ineffective. A combination of prednisone and massive doses of cyclophosphamide and vincristine, however, was effective in halting the rapid progression of the disease. The patient recovered gradually; the results of the pulmonary function tests were close to normal 2 weeks after the onset of therapy. Lung biopsy showed normal lung histology 7 months later. The patient has had no complaints at all for more than 3 years after the episode so that we may assume that he recovered fully.