No abstract

BackgroundThe recently suggested distinct pathogenic pathways for myeloperoxidase (MPO) and proteinase 3 (PR3) anti-neutrophilic cytoplasmic antibodies (ANCA) associated vasculitis could result in different modes of presentation and outcome. Moreover, kidney outcome was related to a new histopathologic classification of pauci-immune glomerulonephritis. As reports were not always concordant, possible because differences in severity of organ lesions and ethnicity, we evaluated the outcome of a cohort of Central-East European patients with crescentic glomerulonephritis in relation with ANCA specificity and histopathological classification.MethodsSeventy-five patients were consecutively diagnosed by kidney biopsy (76 % MPO-ANCA specificity, 52 % crescentic) and followed for a median period of 3.2 years. Study end-points were response to therapy, end stage renal disease (ESRD) and death.ResultsPR3-ANCA patients were younger, in higher proportion male and had higher Birmingham Vasculitis Activity Scores (BVAS). The kidney disease was severe at presentation (median creatinine 5 mg/dL; 27 % required temporary dialysis) and worst in PR3-ANCA positive patients (50 % patients needed temporary dialysis vs. 19 %). The lung was the second most affected organ (31 % severe lung hemorrhage). Lung and kidney damage were related; the odds of hemorrhagic alveolitis in patients needing dialysis at presentation were 4 (95 % CI 1–13; p = 0.006) times higher than in those who did not. The rate of response to therapy (without signs of active vasculitis and stable or declining serum creatinine) was 60 % and was associated with dialysis independency, older age and higher platelet number at presentation. The probabilities to survival 1 and 5 years for kidney and patient were 93 and 64 %, and respectively 88 and 67 %. Kidney survival was predicted by response to therapy and dialysis independency at presentation. Patients with BVAS < 15 and responding to induction therapy had better chances of survival. Neither response to therapy nor outcome was influenced by ANCA specificity or by the histopathological class.ConclusionsWhen kidney damage is severe in ANCA vasculitis, the need of dialysis at presentation and the response to induction therapy overcome the prognostic utility of both ANCA specificity and histopathological class.

As Kauppila score correlates with RRI in non-dialysis CKD patients, it could be a fast, convenient and relatively inexpensive tool for estimating RRI, and consequently the intrarenal vascular status, but further research is warranted.

We report a patient with IgM-predominant type I cryoglobulinemia (CG), who presented to our nephrology department with acute kidney injury. He was previously diagnosed with sensorimotor neuropathy, which was in remission with maintenance dose of corticosteroids. Upon admission, there were ulcerated, necrotic cutaneous lesions localized to the inner aspect of the thighs bilaterally. Further workup revealed a mucosa-associated lymphoid tissue lymphoma, causing type I CG. Screening tests for hepatitis viruses were negative at this time. Under treatment with diuretics and high-potency glucocorticoids, the patient had an acceptable recovery of renal function and was referred to oncology for treatment. Unfortunately, three months later the patient succumbed to fulminant hepatitis, presumably secondary to reactivation of an occult hepatitis B/D co-infection. We further conducted a literature review to better describe patient characteristics and renal involvement in type I CG.

Psoriasis vulgaris is a complex immune-mediated disorder that manifests as a chronic skin disorder, characterized by well-circumscribed inflammatory, erythematous plaques. In this case report, we present a patient with generalized pustular psoriasis (GPP) who presented to the nephrology department with rapidly progressive decline in renal function. The diagnosis of GPP was made a month ago, secondary to a coagulase-negative staphylococcal superinfection. Intrinsically, this introduced a diagnostic challenge as the presumed diagnosis of immunoglobulin A (IgA) nephropathy had to be distinguished from IgA-dominant infectionrelated glomerulonephritis. We further discuss the current evidence and immunohistological profiles of IgA nephropathy in psoriasis and detail the evolution of renal function of our patient over 25 months after he presented to our department.