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Make the grade for Wegener's granulomatosis after kidney transplantation.

Schewior L, Dragun D, Rudolph B, Schaeffner E - NDT Plus (2009)

Bottom Line: While relapse of AAV is high in dialysis patients (up to 50%), it decreases after KTx (8.6-22.2%).Therapeutic guidelines for the management of AAV after KTx do not exist and clinical management is a controversial discussion.Case 1 experienced repeated relapses after KTx and was finally successfully treated with rituximab.

View Article: PubMed Central - PubMed

Affiliation: Department of Nephrology and Intensive Care Medicine, Charité Campus Virchow.

ABSTRACT
Antineutrophil cytoplasmic antibodies-associated vasculitis (AAV) is a well-described cause of multiple organ involvement including rapidly progressive pauci-immune crescentic glomerulonephritis. Kidney transplantation (KTx) is considered the treatment of choice in patients with end-stage renal disease (ESRD) due to AAV. Patient and graft survival in AAV after KTx is favourable and comparable with other non-diabetic causes of ESRD. While relapse of AAV is high in dialysis patients (up to 50%), it decreases after KTx (8.6-22.2%). Yet, relapse may occur at any time after KTx and transplant involvement has been documented in at least 25 cases. Therapeutic guidelines for the management of AAV after KTx do not exist and clinical management is a controversial discussion. We present two unusual cases of young males with smouldering AAV who recently underwent KTx at our hospital. Case 1 experienced repeated relapses after KTx and was finally successfully treated with rituximab. Case 2 received rituximab pre-emptively before living kidney donation and remained free of flairs. Prompted by theses two cases, we reviewed the literature focusing on the right point of time for transplantation, risk assessment, role of antineutrophil cytoplasmic antibodies, clinical presentation of flairs and immunosuppression in smouldering Wegener's granulomatosis (WG) and in relapse, including individualized treatment with rituximab.

No MeSH data available.


Related in: MedlinePlus

CT-scan in a patient with concealed relapse of pulmonary Wegener's granulomatosis. (A) 6 months after KTx presenting with mild dyspnoea, microscopic haematuria: typical radiological manifestation of pulmonary WG with smooth and speculated nodules in the upper-right lobe. Cavitations are characterized by walls and partially shaggy, irregular inner borders. (B) Follow-up CT-scan 6 weeks after the second relapse of AAV and treatment with PPH, CYP and consecutively rituximab: discrete residual bilateral granuloma. The areas of consolidation are seen in association with small pulmonary nodules and foci of calcification.
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Figure 2: CT-scan in a patient with concealed relapse of pulmonary Wegener's granulomatosis. (A) 6 months after KTx presenting with mild dyspnoea, microscopic haematuria: typical radiological manifestation of pulmonary WG with smooth and speculated nodules in the upper-right lobe. Cavitations are characterized by walls and partially shaggy, irregular inner borders. (B) Follow-up CT-scan 6 weeks after the second relapse of AAV and treatment with PPH, CYP and consecutively rituximab: discrete residual bilateral granuloma. The areas of consolidation are seen in association with small pulmonary nodules and foci of calcification.

Mentions: Typical clinical presentation of WG is characterized by a rapid onset of glomerular haematuria, haemoptysis and nasal mucosal involvement, occasionally with cutaneous vasculitis. In contrast, symptoms at relapse after KTx vary widely with respect to onset, severity and organ involvement. Reports by Haubitz and Gera describe affection of the eyes as well as arthralgia and rush in some patients, while all of them had mild general symptoms with manifestation of the upper respiratory tract without KTx involvement [1,14]. Another case reported a catastrophic onset of relapse with KTx failure [21]. In our first index case, the patient presented with mild arthralgia 5 months after KTx, while imaging and histology revealed relapse of WG with renal and pulmonary involvement (Figures 1A and 2A). As shown here, the discrepancy between mild clinical symptoms and severe organ affection can be striking and the clinical differentiation between relapse and ‘smouldering’ disease even impossible. Hence, mild dyspnoea and microscopic haematuria seem to be the predominant symptoms of relapsing WG after KTx, but other individual symptoms such as arthralgia, bursitis, granular tissue and erythema can also predict disease activity [22].


Make the grade for Wegener's granulomatosis after kidney transplantation.

Schewior L, Dragun D, Rudolph B, Schaeffner E - NDT Plus (2009)

CT-scan in a patient with concealed relapse of pulmonary Wegener's granulomatosis. (A) 6 months after KTx presenting with mild dyspnoea, microscopic haematuria: typical radiological manifestation of pulmonary WG with smooth and speculated nodules in the upper-right lobe. Cavitations are characterized by walls and partially shaggy, irregular inner borders. (B) Follow-up CT-scan 6 weeks after the second relapse of AAV and treatment with PPH, CYP and consecutively rituximab: discrete residual bilateral granuloma. The areas of consolidation are seen in association with small pulmonary nodules and foci of calcification.
© Copyright Policy - creative-commons
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4421184&req=5

Figure 2: CT-scan in a patient with concealed relapse of pulmonary Wegener's granulomatosis. (A) 6 months after KTx presenting with mild dyspnoea, microscopic haematuria: typical radiological manifestation of pulmonary WG with smooth and speculated nodules in the upper-right lobe. Cavitations are characterized by walls and partially shaggy, irregular inner borders. (B) Follow-up CT-scan 6 weeks after the second relapse of AAV and treatment with PPH, CYP and consecutively rituximab: discrete residual bilateral granuloma. The areas of consolidation are seen in association with small pulmonary nodules and foci of calcification.
Mentions: Typical clinical presentation of WG is characterized by a rapid onset of glomerular haematuria, haemoptysis and nasal mucosal involvement, occasionally with cutaneous vasculitis. In contrast, symptoms at relapse after KTx vary widely with respect to onset, severity and organ involvement. Reports by Haubitz and Gera describe affection of the eyes as well as arthralgia and rush in some patients, while all of them had mild general symptoms with manifestation of the upper respiratory tract without KTx involvement [1,14]. Another case reported a catastrophic onset of relapse with KTx failure [21]. In our first index case, the patient presented with mild arthralgia 5 months after KTx, while imaging and histology revealed relapse of WG with renal and pulmonary involvement (Figures 1A and 2A). As shown here, the discrepancy between mild clinical symptoms and severe organ affection can be striking and the clinical differentiation between relapse and ‘smouldering’ disease even impossible. Hence, mild dyspnoea and microscopic haematuria seem to be the predominant symptoms of relapsing WG after KTx, but other individual symptoms such as arthralgia, bursitis, granular tissue and erythema can also predict disease activity [22].

Bottom Line: While relapse of AAV is high in dialysis patients (up to 50%), it decreases after KTx (8.6-22.2%).Therapeutic guidelines for the management of AAV after KTx do not exist and clinical management is a controversial discussion.Case 1 experienced repeated relapses after KTx and was finally successfully treated with rituximab.

View Article: PubMed Central - PubMed

Affiliation: Department of Nephrology and Intensive Care Medicine, Charité Campus Virchow.

ABSTRACT
Antineutrophil cytoplasmic antibodies-associated vasculitis (AAV) is a well-described cause of multiple organ involvement including rapidly progressive pauci-immune crescentic glomerulonephritis. Kidney transplantation (KTx) is considered the treatment of choice in patients with end-stage renal disease (ESRD) due to AAV. Patient and graft survival in AAV after KTx is favourable and comparable with other non-diabetic causes of ESRD. While relapse of AAV is high in dialysis patients (up to 50%), it decreases after KTx (8.6-22.2%). Yet, relapse may occur at any time after KTx and transplant involvement has been documented in at least 25 cases. Therapeutic guidelines for the management of AAV after KTx do not exist and clinical management is a controversial discussion. We present two unusual cases of young males with smouldering AAV who recently underwent KTx at our hospital. Case 1 experienced repeated relapses after KTx and was finally successfully treated with rituximab. Case 2 received rituximab pre-emptively before living kidney donation and remained free of flairs. Prompted by theses two cases, we reviewed the literature focusing on the right point of time for transplantation, risk assessment, role of antineutrophil cytoplasmic antibodies, clinical presentation of flairs and immunosuppression in smouldering Wegener's granulomatosis (WG) and in relapse, including individualized treatment with rituximab.

No MeSH data available.


Related in: MedlinePlus