Tubulointerstitial nephritis and uveitis syndrome (TINU) is a rare oculorenal inflammatory entity with a probable autoimmune etiology. Interstitial nephritis may be asymptomatic and usually has a benign course with spontaneous resolution. Uveitis, instead, is classically anterior, bilateral, and nongranulomatous, but it can be unilateral and presents as posterior uveitis or panuveitis, sometimes with a chronic or recurrent evolution. The frequent time lag of ocular and renal manifestations makes this diagnosis particularly challenging. The authors describe four cases of this rare entity, two with tubulointerstitial nephritis preceding ocular manifestations and the remaining, instead, with uveitis preceding renal involvement. The therapeutic approach included systemic corticosteroids in all cases. The addition of immunosuppressive therapy was required in three patients to achieve uveitis control. TINU is probably an underrecognized entity and should always be considered in the differential diagnosis of a chronic or recurrent idiopathic uveitis, especially in young patients who may have mild and asymptomatic renal disease.

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One of the most common causes of rapidly progressive glomerulonephritis (RPGN) is pauci-immune crescentic glomerulonephritis (CrGN). In the majority of cases, this condition has a positive serologic marker, the anti-neutrophil cytoplasmic antibodies (ANCAs), but in approximately 10% there are no circulating ANCAs, and this subgroup has been known as the ANCA-negative pauci-immune CrGN. RPGN can be associated with systemic diseases, but there are only few case reports describing the association with mixed connective tissue disease (MCTD). The authors report a case of ANCA-negative CrGN associated with a MCTD.

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Atheroembolic renal disease (AERD) is a kidney manifestation of atherosclerosis as a systemic disease. AERD is defined as a renal impairment secondary to embolization of cholesterol crystals with consequent occlusion of renal vascularization. The current case report describes one patient with multiple risk factors but without any inciting event history who presents a very atypical clinical course of a severe and massive atheroembolic disease that developed spontaneously and silently.