Should immunosuppressives be stopped in GPA with dialysis?

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Should immunosuppressives be stopped in granulomatosis with polyangiitis (Wegener's granulomatosis) patients undergoing dialysis?

Immunosuppressives are frequently stopped in patients with granulomatosis with polyangiitis (GPA) (Wegener's granulomatosis) who develop end-stage renal disease. This is done because of the high frequency of infections reported among dialysis patients under immunosuppressives and the former impression that GPA patients no longer experience relapses after the development of end-stage renal disease. We present here a 22-year-old male patient with GPA who had gastrointestinal, genitourinary and respiratory system involvement. The patient died because of a gastrointestinal disease activation that occurred after immunosuppressives were stopped at the initiation of dialysis. The decision to stop immunosuppressives while starting dialysis should be made on an individual basis in patients with GPA, and the risks and benefits should be carefully evaluated.

from: Department of Internal Medicine, Cerrahpasa Medical School, University of Istanbul, Turkey.

as reported in: Büyüktas D, Hatemi G, Tascilar K, Fresko I, Yurdakul S. Clin. Exp. Rheumatol.. 2012 Jan-Feb:30(1 Suppl 70):S104-6.

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Granulomatosis with polyangiitis presenting as a renal tumor.
Inflammatory tumors of the kidney are uncommon and include primary inflammatory processes and systemic diseases such as sarcoidosis, IgG4 disease, and granulomatosis with polyangiitis (GPA). There are approximately 15 cases of the latter in the literature. Tumors in GPA are well described and have been reported in several organs including breast, orbit, mediastinum, central nervous system, and especially the lung. We report the case of a 48-year-old woman who presented with diffuse frontal headaches. Imaging showed both a cranial/sinus and renal mass. The pathology of the dura and nasal sinus biopsies were unrevealing. A nephrectomy was performed that demonstrated a discrete lesion with extensive necrosis, granulomatous inflammation, and crescentic pauci-immune glomerulonephritis, findings consistent with GPA.


from: Departments of *Pathology †Rheumatology, Harvard Medical School, Beth Israel Deaconess Medical Center ‡Department of Pathology, Harvard Medical School Massachusetts General Hospital, Boston, MA.
as reported in: Ward A, Konya C, Mark EJ, Rosen S. Am. J. Surg. Pathol.. 2014 Oct:38(10):1444-8. doi: 10.1097/PAS.0000000000000294.
See Published Case Report

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Refractory pituitary granulomatosis with polyangiitis (Wegener's) treated with rituximab.
Granulomatosis with polyangiitis (GPA), also known as Wegener's granulomatosis, is an autoimmune disease characterized by inflammation of blood vessels most often seen in the upper respiratory tract, lungs, kidneys, and skin. Central nervous system (CNS) involvement of GPA is rare, particularly in the pituitary, and can be difficult to treat. Case report. We present a 30-year-old woman with pituitary and ocular GPA, whose unusually recalcitrant disease led to the development of pan-hypopituitarism and near-total vision loss. After failing multiple systemic immunosuppressants, she was ultimately treated with the novel immunomodulatory agent rituximab together with pulse corticosteroids, which achieved a gratifying response. Pituitary and optic chiasm involvement is a rare complication of GPA. We believe this case illustrates the complexity of management of pituitary GPA and provides insight into the potential utility of the biologic agent rituximab in this disease.


from: Section of Endocrinology and Metabolism, Yale University School of Medicine, New Haven, CT 06519, USA.
as reported in: Hughes J, Barkhoudarian G, Ciarlini P, Laws ER, Mody E, Inzucchi SE, Woodmansee WW. Endocr Pract. 2013 Jan-Feb:19(1):e1-7. doi: 10.4158/EP12181.CR.
See Published Case Report

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Granulomatosis with polyangiitis: recurrence presenting as ependimoplexitis.
A 55-year-old man with granulomatosis with polyangiitis (GPA) developed continuous parietal headache, malaise, nasal crusting and dry cough. Neurological exam revealed only left hand hypoesthesia in 4th and 5th finger. Brain MRI showed enlarged right choroid plexus, hyperintense periventricular white matter, thalami and right side of corpus callosum. The suspected diagnosis was ependimoplexitis due to GPA, the patient received three 500 mg methylprednisolone pulses followed by 1 mg/kg of meprednisone with gradual tapering and was switched to oral cyclophosphamide. He had complete resolution of headache. An MRI following this treatment for relapse revealed only minimal ependimal changes.


from: Section of Rheumatology and Immunology, Centro de Educación Médica e Investigaciones Clínicas (CEMIC), Buenos Aires, Argentina..
as reported in: Pisoni CN, Ibañez S, Guevara M, Castro D, Romero Vidomlansky S. Clin. Exp. Rheumatol.. 2014 May-Jun:32(2 Suppl 82):S70-2.

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A typical Wegeners granulomatosis -- but not pauci-immune!
We present a the case of 58-year old man who was admitted to hospital with typical clinical features (bloody nasal discharge, arthralgia, acute kidney injury with a nephritic syndrome) consisting with Wegeners granulomatosis (WG). CT-scan showed pulmonary nodules and antineutrophil cytoplasmatic antibodies (ANCA) were elevated. A kidney biopsy showed a crescentic glomerulonephritis, but not pauci-immune-immune with a histopathological staining of a mesangioproliferative IgA-glomerulonephritis. The patient was put on prednisolone and i.v. cyclophosphamid (CYCLOPS-protocol (1). The anti-proteinase-3 antibody titer decreased and the CT-scan showed decreased activity of Wegener's granulomatosis (BVAS 26 dropped to 2) and the patient`s serum creatinine level was stable. The exact nosological relation of mesangial IgA-nephropathy to WG is still unclear. This case underlines that knowledge of renal histology is essential in the management of patients with renal disease, especially in patients with hematuria and/or proeinuria with positive ANCA.


from: Department of Internal Medicine, Robert-Bosch Hospital, Stuttgart, Germany.
as reported in: Joerg L, Kerstin A, Niko B, Dominik A, Martin K, Latus J, Amann K, Braun N, Alscher MD, Kimmel M. Minerva Urol Nefrol. 2012 Jun:64(2):149-52.

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Wegener's granulomatosis - a case report.
Wegener's granulomatosis is an uncommon multi-systemic disease characterized by necrotizing granulomatous inflammation of the upper and lower respiratory tracts and general focal necrotizing vasculitis (commonly known as Wegener's triad). The diagnosis of Wegener's granulomatosis is suggested from the clinical and laboratory findings and from the presence of circulating anti-neutrophil cytoplasmic antibodies (ANCA) although the absence of ANCA does not exclude the diagnosis. We described a case of 27-year-old man with pansinusitis, mild azotemia and initial respiratory tract symptoms such as foul smelling nasal discharge and epistaxis. Later he developed fever, poly-arthritis, abdominal pain and haematuria. There were multiple painful oral ulcers and skin showed multiple palpable purpuric rash. C-ANCA was positive. He was treated with IV methyl prednisolone 1gm daily for 3 days followed by oral prednisolone 1mg/kg body weight and oral cyclophosphamide. His condition improved dramatically and on follow up after 3 months he was reasonably well. In this report, we wanted to emphasize that Wegener's granulomatosis, although rare, should be considered in the above clinical scenario and treatment should be initiated as soon as possible.


from: Department of Medicine, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh.
as reported in: Islam MA, Azad AK, Islam MS, Shazzad MN, Haq SA. Mymensingh Med J. 2013 Jan:22(1):196-9.

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Pulmonary renal syndrome in a case of Wegener's granulomatosis.
We report a case of a 42-year-old patient who presented with Wegener's granulomatosis complicated by pulmonary renal syndrome, i.e., diffuse alveolar haemorrhage and rapidly progressive crescentic glomerulonephritis. The patient was treated with plasmapheresis and immunosuppressive drugs--intravenous cyclophosphamide and methyl prednisolone. The clinical, haematological and biochemical parameters improved substantially and remission is achieved.


from: Department of Pulmonary Medicine, Institute of Post Graduate Medical Education and Research, Kolkata, West Bengal, India.
as reported in: Kundu S, Misra S, Halder RK, Roychowdhury A. Indian J Chest Dis Allied Sci. 2013 Jan-Mar:55(1):49-52.

Interesting to me to read about cases in India and other places where we don't hear much about GPA. This case was very similar to mine.

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Not everything that looks like GPA is GPA!

Culture-negative subacute bacterial endocarditis masquerades as granulomatosis with polyangiitis (Wegener's granulomatosis) involving both the kidney and lung.
Subacute bacterial endocarditis (SBE) occasionally exhibits positive cytoplasmic anti-neutrophil cytoplasmic antibody (c-ANCA) of the anti-proteinase-3 (PR-3) type. Clinically, it mimics ANCA-associated vasculitis, such as Wegener's disease with glomerulonephritis. Lung abscesses are the most common manifestation of lung involvement. We herein report a case of culture-negative SBE strongly c-ANCA/PR3-positive accompanied by pulmonary involvement and glomerulonephritis. In this case, we took biopsies of both the lung and kidney, although renal biopsy is usually preferred over lung biopsy. The lung biopsy showed severe alveolar capillaritis, suggesting vasculitis consistent with polyangiitis. The renal biopsy revealed glomerulonephritis with a membranoproliferative pattern. To our knowledge, this is the first such reported case. A 68-year-old Chinese male patient presented to our hospital with a fever, cough, chest pain, and recurrent peripheral edema. He had a past medical history significant for treated schistosomiasis 20 years previously. Physical examination revealed palpable purpura, mild hypertension, hepatosplenomegaly, and a holosystolic cardiac murmur (Levine 2/6). Echocardiography showed tricuspid valve vegetations with moderate to severe regurgitation. Serum c-ANCA/PR3 and cryoglobulin were strongly positive. Renal biopsy results indicated membranoproliferative glomerulonephritis with several crescents. Chest CT revealed multiple intraparenchymal and subpleural nodules, and lung biopsy showed polyangiitis. The patient's ANCA titers, glomerulonephritis, and pulmonary injury all resolved after antibiotic therapy. SBE may present with positive c-ANCA/PR3, multiple pulmonary nodules, pulmonary polyangiitis, and glomerulonephritis clinically mimicking granulomatosis with polyangiitis (Wegener's granulomatosis).


from: Division of Nephrology, Department of Medicine, The third affiliated hospital of Sun Yat-sen University, 600 Tianhe Road, Guangzhou, 510630, People's Republic of China.
as reported in: Peng H, Chen WF, Wu C, Chen YR, Peng B, Paudel SD, Lou TQ. BMC Nephrol. 2012:13:174. doi: 10.1186/1471-2369-13-174.
See Published Case Report

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Wegener's granulomatosis with pulmonary fungal infection: a case report and brief review.
Wegener's granulomatosis (WG) is an autoimmune, necrotizing granulomatous disease of unknown aetiology that affects small and medium blood vessels, and is usually recurrent. Infection is the most frequent cause of death in patients with WG. A case of WG with pulmonary fungal infection in a 50-year-old man is reported. The patient was hospitalized following a 2-month history of haemoptysis and a 1-month history of intermittent fever. Examination and pathology results confirmed a diagnosis of WG with associated pulmonary fungal infection. The patient's condition was complicated by a septic pneumothorax and sinus formation after lung biopsy, and preexisting diabetes and hypertension, which worsened rapidly due to his critical condition. He was treated with glucocorticoids and cyclophosphamide therapy with the goal of controlling these complications, and had no recurrence within the 4-year follow-up period. This case demonstrated the utility of combined glucocorticoid and cyclophosphamide therapy for the treatment of infection in WG.


from: Department of Respiration, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, China.
as reported in: He Y, Liu J, Gao B. J. Int. Med. Res.. 2012:40(1):383-92.

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A case of exorbitism in association with Wegener's granulomatosis with renal involvement.
Wegener's granulomatosis (WG) is a necrotizing granulomatous vasculitis involving the nose, paranasal sinuses, lungs, and kidneys. Ocular involvement can occur in about 50% of cases. There are very few reports of WG with orbital inflammation and exorbitism. We report a case of a female patient who presented with exorbitism related to orbital inflammation secondary to WG, with renal involvement. A 29-year-old woman with a previous history of recurrent pan-sinusitis presented with bilateral exophthalmos and renal failure with rapidly progressive glomerulonephritis. Computed tomography showed extensive bilateral soft tissue in the retro-orbital area. Immunologic tests showed the presence of type-C anti-neutrophil cytoplasmic antibodies and renal biopsy revealed pauci immune crescentic glomerulonephritis. The patient was treated with corticosteroids and pulses of cyclophosphamide followed by azathioprine and trimethoprim-sulfamethoxazole. After a follow-up of 10 months, the renal outcome was favorable with improvement of renal function but there was persistence of exorbitism and loss of visual function. Our case suggests that WG should be considered in the differential diagnosis of persistent bilateral exophthalmos. Prompt recognition of this early manifestation is important for the institution of early treatment.


from: Department of Nephrology, La Rabta Hospital, Tunis, Tunisia.
as reported in: Beji S, Fatma LB, Chebbi A, Rais L, Krid M, Smaoui W, Maiz HB, Zouaghi K, Moussa FB. Saudi J Kidney Dis Transpl. 2012 Mar:23(2):330-3.