Gene/Protein Disease Symptom Drug Enzyme Compound
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This series of articles on the management of glomerulonephritis (GN) has been prepared by a team of experts in the evidence-based format consistent with peer review of published data. Each author was asked to review the literature for his assigned histological type, with emphasis on therapy and limited to adult studies. The age limit was not considered for minimal change disease and focal segmental glomerulosclerosis, because of the high prevalence of these glomerulopathies in children. The particular treatment recommendations for each type of glomerular disease were graded by each author according to the amount of evidence provided in these reviewed studies. The first two articles concentrate on indications and techniques for kidney biopsy. Each subsequent article focuses on and describes the highest level of evidence supporting the recommendation for therapy in IgA nephropathy (Ig-GN), minimal change nephropathy (MCN) and focal segmental glomerulosclerosis (FSGS), membranous glomerulonephritis (MGN), lupus nephritis, ANCA-associated vasculitis, HCV-associated cryoglobulinaemia and renal involvement in paraproteinemic disorders. The article on IgA nephropathy emphasises the importance of carefully evaluating both clinical and histologic findings before settling on the treatment. The recent, renewed interest in steroids and many immunosuppressive agents is discussed in detail. Recommendations related to the patient's age are also provided. MCN and FSGS are treated together because these forms share similar evidence-based recommendations. For both of these diseases, in fact, the initial treatment approach in children should be prednisone or prednisolone for four to six weeks. The therapeutic response in adults is slower than in children, but adults experience fewer relapses and a more prolonged remission. There is also a discussion on treatment of relapse, frequent relapsing disease and true steroid-resistant disease as well as the role of new immunosuppressive agents. Membranous nephropathy is a frequent cause of nephrotic syndrome in adults and, in one third of these patients, leads to end-stage renal disease. However, the treatment of this form is as yet a matter of discussion. Based on extensive critical review of the literature, the following recommendations are put forward: (a) no treatment in the absence of nephrotic syndrome; (b) patients with heavy proteinuria should receive a 6-month treatment with i.v. methylprednisolone (MP) pulse therapy for three consecutive days followed by oral MP (0.4 mg/kg/day) (months 1, 3, 5) and chlorambucil or cyclophosphamide (months 2, 4, 6); (c) the dosage of chlorambucil or cyclophosphamide should be lowered in older patients; (d) cyclosporine is a second-choice treatment. The treatment of lupus nephritis depends on the histologic class. No specific treatment is usually necessary for class I and IIA. Oral steroids are indicated in patients with class IIb, proteinuria and active systemic disease. Steroids and azathioprine are the treatment of choice for patients with class III and IV, but cyclosporine can be an effective alternative therapy. Cyclophosphamide is more effective than azathioprine when severe acute renal involvement is present. The treatment of ANCA-associated vasculitis depends mainly on clinical presentation, oral prednisone + oral or i.v. cyclophosphamide are generally effective. In the most severe cases, the association of MP pulse therapy with cyclophosphamide is probably more effective. Plasma exchange is probably justified in unresponsive patients. Azathioprine should replace cyclophosphamide during the maintenance therapy. In HCV-associated mixed cryoglobulinemia the treatment also depends on the severity of renal involvement. The treatment for chronic HCV infection involves alpha interferon alone or preferably in combination with ribavirin. Aggressive therapy, including i.v. MP, plasmapheresis and cyclophosphamide is primarily reserved for patients with acute severe disease, as manifested by progressive renal failure, distal necroses requiring amputation, or advanced neuropathy. Uncontrolled studies suggest that this regimen can improve renal function. Renal involvement is a common problem in paraproteinemic disorders that include multiple myeloma, Waldentrom's macroglobulinaemia and monoclonal gammopathy. The most common renal diseases in this setting are cast nephropathy, primary amyloidosis cast nephropathy, primary amyloidosis, and light chain deposition disease that are related to the overproduction of monoclonal immunoglobulin light chains. The approach to therapy varies with the cause of the renal dysfunction. Patients with amyloidosis or light-chain deposition disease are generally treated with chemotherapy, but the most effective therapy for myeloma kidney is prevention by minimising the risk factors that promote light chain filtration and subsequent obstruction by cast formation within the tubules. Chemotherapy or stem cell or bone marrow transplantation to decrease filtered light chain load, prevent volume depletion and maintain high fluid intake to reduce light chain concentration within the tubular lumen are indicated in almost all the patients.
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PMID:[Instructions and implementations for percutaneous renal biopsy. Guidelines for the therapy of glomerular nephropaties]. 1466 2

Wegener's Granulomatosis (WG) is a rare, Multisystem disease of the medium and small sized arteries and veins. It most commonly involves the upper respiratory tract, lungs, and kidneys and often presents as chronic fatigue, upper respiratory infection, sinusitis, and otitis media. Symptoms can include fever, weight loss and fatigue, though these are not usually the primary presenting complaints. development of the disease is highly skewed across ethnicities, with up to 98% of cases being reported in caucasians. We present the case of a 56-year-old African-American male who presented primarily with complaints of uncontrollable fever of unknown origin (FUO) for past two weeks with accompanying sore throat, nasal congestion, night sweats, malaise, and unexplained weight loss of 10 pounds over the past month. Treatment with antibiotics for one week prior to admission showed no relief of symptoms. Chest x-ray showed focal course markings in the right upper lobe. Urinalysis revealed microscopic hematuria and leukocyturia. Chest and abdominal CT scans revealed a right lower lobe pulmonary nodule and heterogeneous areas of enhancement in the spleen. Head CT revealed right mastoid opification. Labs revealed proteinase-3 antibody titer > 100, which is characteristic of WG. Steroids and cyclophosphamide were started with relief of presenting symptoms. Renal biopsy showed pauci-immune P-ANCA associated crescentic and focally necrotizing glomerulonephritis and vascilitis. This case is unique in that the patient presented with primary complaint of FUO. WG should be considered as a rule-out in cases of uncontrollable FUO, even if none of the classic triad of symptoms is present. Though rare, WG should be considered in cases involving non-Caucasian patients.
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PMID:Wegener's granulomatosis presenting as fever of unknown origin in an African-American male. 1860 51

The treatment of recurrent glomerulonephritis (GN) is often empirical. Plasmapheresis has received the largest consensus for the treatment of focal glomerular sclerosis (FGS), whether associated with cyclophosphamide and steroids or not. To be effective, such therapy needs to be started as quickly as possible after the onset of proteinuria, and prolonged for months when recovery is delayed. Plasmapheresis and cyclophosphamide have also been used to treat GNs with glomerular crescents. However, there has been no consensus on the efficacy of such therapy. The recently introduced rituximab is the most innovative drug but also the most experimental. So far, it has been used for the treatment of ANCA-associated vasculitis, FGS and membranous GN, with results that are still under debate. Cyclophosphamide has been used in patients with severe recurrent GN, but the anedoctal cases described prevent us from drawing any firm conclusions. Steroids have been used for the treatment of many recurrent GNs, but yet again, without any standard protocol. They have been used both in children with FGS and in adults with aggressive GN or severe proteinuria. Both ACE inhibitors and angiotensinreceptor blockers have been suggested as first-line therapy in recurrent GN with proteinuria. This therapy is safe and can be even more effective than others. Finally, it must be kept in mind that the addition of immunosuppression in transplant patients can dramatically increase the risk of infective complications. Moreover, recurrent GNs are often associated with chronic allograft diseases that can cause graft worsening independently of any therapy.
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PMID:[Treatment of post-transplant glomerulonephritis]. 1904 93

The impairment of the peripheral nervous system is frequent in primary or secondary vasculitis. The diagnosis of painful multiple mononeuropathy requires neuromuscular biopsy. Distal sensorimotor polyneuropathies are misleading. Therefore clinical and electrophysiological asymmetry is helpful. Steroids and cytotoxic agents have been the mainstay of therapy for most vasculitic neuropathies but therapeutic strategies may vary for ANCA-associated vasculitis.
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PMID:[Vasculitis multiple mononeuropathies]. 1915 5

There have been great advances in the management of ANCA associated vasculitis over the past decades. We have gone from an era where the disease was almost universally fatal to trying to prevent long-term side effects of treatment regimens. With the ability to use pulse cyclophosphamide or rituximab as alternates to oral cyclophosphamide for induction of remission, side effects of therapy have been greatly reduced. New approaches have drastically changed our approach to maintenance and we now favor much longer durations of maintenance therapy, as they are more successful in preventing relapse. Steroids have long been the bane of treatment as they are associated with a significant risk of infection and metabolic consequences. We are now in a steroid-sparing and looking ahead to a steroid-free era with new data being published showing lower doses of steroids being equally effective and several ongoing seminal trials looking at agents that could completely replace steroids very early on.
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PMID:Updates in the treatment of granulomatosis with polyangiitis and microscopic polyangiitis: At a crossroad. 3263 67