Case Report: Bilateral Lung Transplantation for Rapidly Progressive Undifferentiated Interstitial Lung Disease-A Cautionary Tale.

Interstitial lung disease is fast becoming one of the most common indications for lung transplantation (LTx); however, LTx for Goodpasture's syndrome with pulmonary involvement has not been previously described in the literature. In this report, we outline the case of a young male with undifferentiated rapidly progressive interstitial lung disease who ultimately received a bilateral sequential LTx after deterioration requiring extracorporeal membrane oxygenation. The original disease soon recurred in the graft, and unfortunately, the patient did not survive. The diagnosis of Goodpasture's syndrome was made postmortem and was not clearly evident on examination of the native explanted tissue, nor was there an elevated titer of antiglomerular basement membrane antibodies during his initial work-up. We hypothesize that the donor and recipient's HLA profile made him more susceptible to aggressive disease. In hindsight, active Goodpasture's disease would have been a contraindication to proceed to transplantation. This case is a cautionary reminder of the high stakes of performing LTx without a certain diagnosis.

[Benefit of Howell-Jolly bodies detection: finding of an acquired hyposplenism in a patient with Goodpasture syndrome]. / Intérêt de la détection des corps de Howell-Jolly : hyposplénisme acquis dans le cadre d'un syndrome de Goodpasture.

Howell-Jolly bodies are intraerythrocytic inclusions corresponding to a small portion of chromatin. Red blood cells that contain these nuclear remnants are removed from the circulation by the spleen. In most cases, presence of Howell-Jolly bodies on a blood smear is the result of functional asplenia and splenectomy. Observation. We report incidental finding of numerous Howell-Jolly bodies in a patient followed by the nephrology department. This microscopic observation of blood smear led to a diagnostic imaging and to the evidence of a reduced spleen, possibly favoured by a history of Goodpasture syndrome in this renal transplant patient without splenectomy. Vaccination and antibioprophylaxy were proposed to prevent infectious risk linked to this splenic hypoplasia. Conclusion. Seeking of Howell-Jolly bodies could be made in every condition associated with a risk of splenic hypoplasia to prevent infectious risk.

A rare fatal complication of bleeding immediately following polytetrafluoroethylene arteriovenous grafting in a lady with Goodpasture syndrome.

We report a 28-year-old female with no history of allergies and recent onset of Goodpasture syndrome who developed life-threatening bleeding immediately after placement of a polytetrafluoroethylene (PTFE) graft as an access for hemodialysis in the left upper limb by an experienced vascular surgeon. In spite of transfusing fresh frozen plasma, packed cells and cryoprecipitate, her prothrombin time (PT), activated partial thromboplastin time and international normalized ratio became progressively worse which were normal at the beginning of the surgery. She had profound hypotension and succumbed within 8 hours. We suspect a rare phenomenon of the interaction of her blood with the PTFE graft causing activation of bleeding and coagulation factors leading to disseminated intravascular coagulation (DIC).

[Anesthesia for living donor renal transplantation in a patient with Goodpasture's syndrome with a history of repeated alveolar hemorrhage].

A 20-year-old woman was scheduled for living renal transplantation from her mother. She was diagnosed with Goodpasture's syndrome at 8 years of age and had a previous history of alveolar hemorrhage 5 times. She developed renal failure, hypertension and required hemodialysis. She had no history of surgery. Blood test was not remarkable except anemia and elevated blood urea nitrogen and serum creatinine levels. General anesthesia was induced and maintained with fentanyl, remifentanil and propofol. After tracheal intubation, the lungs were mechanically ventilated with a pressure control mode and the peak airway pressure was initially adjusted to 9 cmH2O for maintaining airway pressure at a low level to prevent alveolar injury, which was increased to 12 cmH2O at the end of surgery for maintaining a tidal volume of approximately 250 ml. No hemorrhage was detected in the airway during anesthesia, blood gas data were within the normal range, and the tracheal tube was removed after surgery in the operating room. Postoperative course was uneventful. Antihypertensive agents were discontinued and she was successfully weaned from hemodialysis.

Treatment of severe psoriasis with etanercept in a pancreas-kidney transplant recipient.

Severe psoriasis is a rare condition under immunosuppressive therapy. We describe a 42-years-old man with psoriasis since the age of 22 years. The patient underwent a combined pancreas-kidney transplantation at the age of 32 because of Goodpasture syndrome with renal and pulmonary involvments and type 1 diabetes mellitus. Seven years later a pancreas retransplantation was performed due to nonfunction of the original pancreas allograft. Despite intensive systemic immunosuppression, consisting of prednisone, tacrolimus, and mycofenolate mofetil, and topical treatment with dithranol and steroids, there was significant worsening of psoriasis. In October 2009 we initiated therapy with etanercept (25 mg s.c.) twice weekly under close clinical and laboratory monitoring. Improvement was rapid with a decrease in Psoriasis Area and Severity Index (PASI) from 25.2 to <5 during the first months of treatment without a severe infection or other adverse reaction. Graft functions were not affected by the treatment. The patient remains till now on the same regimen and is almost free of skin manifestations. To our knowledge so far only 2 cases of etanercept therapy in psoriasis have been reported in liver transplant recipients. In both cases the treatment was well tolerated and effective. Psoriasis therapy in organ transplant recipients represents a major challenge. Biological agents such as etanercept may provide an effective option for refractory cases.

Kidney transplant after preexisting posterior reversible encephalopathy syndrome induced by Goodpasture's syndrome.

Posterior reversible encephalopathy syndrome is characterized by varying neurologic symptoms associated with brain vasogenic edema. Posterior reversible encephalopathy syndrome can be associated with severe hypertension (eg, in eclampsia or HELLP syndrome), but it also has been observed without hypertension and in several clinical conditions including infections and autoimmune disorders. The literature offers several reports of posterior reversible encephalopathy syndrome detected or induced after bone-marrow and solid-organ transplant, or induction by immunosuppression. We describe what is, to the best of our knowledge, the first case of man who successfully underwent a kidney transplant with preexisting posterior reversible encephalopathy syndrome induced by Goodpasture's syndrome.

[Kidney allograft: a target for systemic disease]. / Le rein transplanté : une cible des maladies systémiques.

Recurrence of disease after transplantation is frequent and represents the third cause of allograft loss. Recurrence of lupus nephritis after transplantation is rare. Kidney transplantation in patients with antiphospholipid syndrome or lupus anticoagulant is challenging due to the high risk of immediate post-transplant thrombosis and bleeding risk associated to the subsequent anticoagulation. Moreover, vascular changes associated to the presence of antiphospholipid antibodies negatively impact allograft rate survival. Recurrence of pauci immune glomerulonephritis or Goodpasture syndrome is exceptional.

[Goodpasture's syndrome with neurologic involvement and negative ANCA]. / Síndrome de Goodpasture asociado con vasculitiscerebral ANCA negativa.

Goodpasture's syndrome is a rare autoimmune disorder characterized by rapidly progressive glomerulonephritis (RPGN) and alveolar hemorrhage in the presence of antiglomerular basement membrane (anti-GBM) antibodies. Central nervous system involvement is highly unusual in the absence of anti-neutrophil cytoplasmic antibodies. We report the case of a 20-year-old man with RPGN accompanied by bloody sputum, tonic-clonic seizure and high titers of anti-GBM antibody. After treatment with immunosuppressants and plasmapheresis, the patient showed reduced anti-GBM antibody titers and improved neurologic and respiratory symptoms, but renal failure persisted, requiring hemodialysis. Twenty months later, with the disease in remission, he underwent deceased-donor renal transplantation.

Mycobacterium gordonae pulmonary disease associated with a continuous positive airway pressure device.

There are no formal recommendations on the proper handling and decontamination of respiratory devices for home use. We describe the case of a kidney transplant recipient who developed Mycobacterium gordonae pneumonia transmitted by his continuous positive airway pressure (CPAP) machine.

Retransplantation in Alport post-transplant anti-GBM disease.

BACKGROUND: Post transplant anti-glomerular basement membrane (GBM) disease affects up to 5% of patients with Alport's syndrome. Defects in the COL4A5 gene are responsible for most cases, and alpha 5(IV)NC1 is the usual target for alloantibodies. Gene deletions are more commonly associated with this complication than are point mutations. The disease is severe in renal allografts and nearly always results in graft loss. METHODS: Three cases of retransplantation in Alport's syndrome are described here in detail. All cases were started on immunosuppressive therapy early in the course of their disease and one patient (case 2) received pre-emptive anti-T-cell therapy (Campath IH). Anti-GBM antibodies in these cases were investigated by standard anti-GBM enzyme-linked immunosorbent assay (ELISA), by indirect immunofluorescence, and by Western blotting using collagenase-digested human GBM and recombinant type IV collagen NC1 domains made in insect cells. RESULTS: All cases showed early antibody and complement fixation to human GBM. Target alloantibodies were to alpha 5(IV)NC1 domain predominantly. Cases two and three gave negative results on standard ELISA for anti-GBM antibodies. Pathologic examination revealed crescentic glomerulonephritis, which was rapid in onset in case 1, blunted and less aggressive in case 3, and case 2 developed segmental necrosis without crescent formation. Neutrophilic infiltrates were an early feature in all 3 cases. All cases are compared with a review of all retransplanted cases in the literature. CONCLUSION: Alport anti-GBM disease is a severe disease in retransplanted patients. Anti-T-cell therapy seemed to modify the pathologic findings but did not prevent graft loss. Longer term plasma exchange and mycophenolate mofetil may attenuate the illness, but in these cases did not prevent graft loss. Western blotting detected alloantibodies to alpha 5(IV) NC1 domain and is more sensitive and specific for this disease than standard ELISAs.

[28-year-old woman with Goodpasture's syndrome and nonspecific colitis]. / Zespól Goodpasture'a wspólistniejacy z nieswoistym zapaleniem jelit u 28-letniej kobiety.

Goodpasture's syndrome usually becomes manifest with progressive glomerulonephritis and pulmonary hemorrhage. The diagnostic hallmark is the demonstration of anti-basement membrane anti-alpha3 type IV collagen antibodies (anti-GMB antibodies) in patient sera. Herein is reported the case of 28-year-old woman with Goodpasture's syndrome who developed the acute symptoms of nonspecific colitis which required surgical intervention. Immunosuppression along with plasmapheresis was successful in the treatment of life-threatening pulmonary hemorrhage, but not for the improvement of renal function.

Successful renal transplantation in a child with ANCA-associated microscopic polyangiitis.

Crescentic glomerulonephritis (CGN) is a clinicopathologic entity which is characterized by severe renal dysfunction of rapid onset with glomerular crescents. Type III CGN is associated with the absence of glomerular immune complex deposition (pauci-immune) and is associated with antineutrophil cytoplasmic antibody (ANCA). Microscopic polyangiitis and idiopathic pauci-immune necrotizing glomerulonephritis (NCGN) are strongly associated with ANCA directed against myeloperoxidase (anti-MPO). We describe here an unusual pediatric patient with MPO-ANCA-associated rapidly progressive glomerulonephritis (RPGN), emphasizing the management and outcome of the disease.

Outcome of renal transplantation in patients with pauci-immune small vessel vasculitis or anti-GBM disease.

AIM: Pauci-immune small vessel vasculitis (SVV) and anti-GBM disease are the most common causes of rapidly progressive glomerulonephritis (RPGN) and they frequently lead to end-stage renal disease. For renal replacement therapy, renal transplantation is the preferred treatment option. However, in patients with glomerular diseases, the outcome of renal transplantation can be adversely affected by recurrence of the original disease. The information in the medical literature on the outcome of renal transplantation in patients with RPGN is limited because most data are derived from case studies and from studies involving a small number of patients. METHODS: We studied the outcome of renal transplantation in patients with pauciimmune SVV or anti-GBM disease, transplanted in our center between 1968 and 2000. Patient and graft survival were compared with a matched control group from our hospital. We specifically looked for any evidence of recurrent disease. RESULTS: Included in the study were 43 patients (31 male, 12 female) with a mean age (+/- SD) of 48 +/- 15 years at transplantation. Patients were diagnosed as Wegener's granulomatosis (n = 8), microscopic polyangiitis (n = 7), renal limited vasculitis (n = 18) and anti-GBM disease (n = 10). The average follow-up was 62 +/- 57 months. No graft was lost due to recurrence of the underlying disease. One patient with Wegener's granulomatosis had a relapse with only extrarenal manifestations 5 months after transplantation. Patient and graft survival at 5 years after transplantation were 77% and 60%. Survival rates were not significantly different from a matched control group of renal transplant patients with other underlying diseases, 79% and 56%, respectively. Patients with pauci-immune SVV or anti-GBM disease developed significantly more malignancies than the control group (p = 0.02). CONCLUSIONS: Recurrence of pauci-immune SVV and anti-GBM disease after transplantation is rare. Renal transplantation can be successfully performed in patients with pauciimmune vasculitis or anti-GBM disease. Physicians should be aware of the greater risk of developing malignancies, especially skin cancer.

[Cadaveric renal transplantation for Goodpasture's syndrome: a case report].

A 19-year-old man with a history of histologically-proven Goodpasture's syndrome (hemoptysis, rapidly progressive glomerulonephritis, and positive anti-glomerular basement membrane (anti-GBM) antibody) was maintained on hemodialysis for 21 months. After steroid pulse therapy and plasmapheresis, his anti-GBM antibody disappeared. His stable condition on dialysis and a session of plasmapheresis prior to surgery allowed him to undergo cadaveric renal transplantation from a 34-year-old man. The blood type was identical (group A and Rh (+)), and there was 1 and 0 mismatch of HLA class 1 and 2, respectively. The initial immunosuppressants included cyclosporine, methylprednisolone, mizoribine, azathioprine, and anti-lymphocyte globulin (ALG). The transplanted kidney regained function on day 6 and he was free from hemodialysis. Circulating anti-GBM antibody was negative after surgery. The graft has functioned well for almost 4 years after transplantation without any episodes of renal or pulmonary complications. To the best of our knowledge, this is the first report of renal transplantation for Goodpasture's syndrome in the Japanese literature.

Ulcerative colitis in a renal transplant patient with previous Goodpasture disease.

A renal transplant was performed in a 6-year-old boy who developed end stage renal disease (ESRD) after presenting with antiglomerular basement membrane (anti-GBM) disease. At 10 years of age he developed ulcerative colitis while being immunosuppressed with cyclosporin, prednisone, and azothioprine. He had a pancolectomy, and at 14 years has no symptoms of ulcerative colitis or anti-GBM disease. HLA typing revealed that he was homozygous for HLA DR2. The co-occurrence of anti-GBM disease and ulcerative colitis has not previously been described. Although there is no known common etiology for these two autoimmune diseases, we propose that the patient's homozygosity at HLA DR2 may have predisposed him to both.