Systemic lupus erythematosus anemia

Carcinoid syndrome, anemias, systemic lupus erythematosus... 

A decrease in erythrocyte production can be multifactorial. A deficiency in nutrients (such as iron, vitamin B12, and folic acid) is a common cause that often is easily treatable. In addition, patients with cancer and CKD are at risk for developing a hypoproductive anemia. Furthermore, patients with chronic immune-related diseases (such as rheumatoid arthritis and systemic lupus erythematosus) can develop anemia as a complication of their disease. Anemia related to these chronic inflammatory conditions is typically termed anemia of chronic disease. 

Besides anemia associated with cancer and CKD, anemia of chronic disease can result from inflammatory processes and occurs commonly in autoimmune disorders such as rheumatoid arthritis and systemic lupus erythematosus. In treating these types of anemia of chronic disease, the most important principle is treating the underlying disease. These patients also may have iron deficiency and should be treated in the manner already discussed. Erythropoietin therapy such as epoetin-alfa therapy at a dose of 150 units/kg three times a week also may be used in these patients. 

Iron deficiency anemia and systemic lupus erythematosus rarely occur. 

Azathioprine and mercaptopurine appear to be of definite benefit in maintaining renal allografts and may be of value in transplantation of other tissues. These antimetabolites have been used with some success in the management of acute glomerulonephritis and in the renal component of systemic lupus erythematosus. They have also proved useful in some cases of rheumatoid arthritis, Crohn s disease, and multiple sclerosis. The drugs have been of occasional use in prednisone-resistant antibody-mediated idiopathic thrombocytopenic purpura and autoimmune hemolytic anemias. 

The effectiveness of immunosuppressive drugs in autoimmune disorders varies widely. Nonetheless, with immunosuppressive therapy, remissions can be obtained in many instances of autoimmune hemolytic anemia, idiopathic thrombocytopenic purpura, type 1 diabetes, Hashimoto s thyroiditis, and temporal arteritis. Improvement is also often seen in patients with systemic lupus erythematosus, acute glomerulonephritis, acquired factor VIII inhibitors (antibodies), rheumatoid arthritis, inflammatory myopathy, scleroderma, and certain other autoimmune states. 

Certain autoimmune syndromes can be induced by drugs. Examples include systemic lupus erythematosus following hydralazine or procainamide therapy, "lupoid hepatitis" due to cathartic sensitivity, autoimmune hemolytic anemia resulting from methyldopa administration, thrombocytopenic purpura due to quinidine, and agranulocytosis due to a variety of drugs. As indicated in other... 

Azathioprine is an effective agent in suppressing the immune system in patients undergoing renal transplantation and in patients suffering from acute glomerulonephritis, the renal component of systemic lupus erythematosus, prednisone-resistant idiopathic thrombocytopenic purpura, and functioning autoimmune hemolytic anemia. Azathioprine depresses bone marrow functioning, which is its chief side effect. 

Autoimmune diseases have been reported to be more frequent in human subjects treated with several recombinant cytokines [38], For instance, increased titers or the new occurrence of autoantibodies have been observed in hepatitis C patients treated with the recombinant interferons-alpha (IFNa). Quite a few clinical case reports describe the development of organ-specific as well as systemic autoimmune diseases including systemic lupus erythematosus, insulin-dependent type I diabetes mellitus, autoimmune thrombocytopenia, autoimmune hemolytic anemia, myasthenia gravis, and autoimmune thyroiditis in patients under IFNa therapy. Although the mechanism involved is not fully elucidated, the available data support the pathogenic potential of IFNa in autoimmunity [31]. In contrast, autoimmune effects associated with IFNp therapy are thought to be of lesser concern based on the current clinical evidence [38], Thyroid autoimmunity in contrast to other autoimmune diseases is frequent in patients treated with recombinant interleukin-2 (rIL-2). Thus, among 281 previously euthyroid cancer patients treated with rIL-2, up to 41%... 

Antiphospholipid syndrome is a disorder of recurrent arterial or venous thrombosis, thrombocytopenia, hemolytic anemia, or a positive Coombs test, and in women recurrent idiopathic fetal loss, associated with raised concentrations of antiphospholipid antibodies. In systemic lupus erythematosus, the risk of this syndrome is about 40%, compared with a risk of 15% in the absence of antiphospholipid antibodies (539). However, only half of those with antiphospholipid antibodies have systemic lupus erythematosus, and the overall risk of the syndrome is about 30%. In patients who have antiphospholipid antibodies associated with chlorpromazine, there appears to be no increased risk of the syndrome. In contrast, in the primary antiphospholipid syndrome, the only clinical manifestations are the features of this syndrome. 

Thrombotic thrombocytopenic purpura is a rare acute or subacute disease in adults, rather similar to the hemolytic uremic syndrome in children, in which there is systemic malaise, fever, skin purpura, renal failure, hematuria and proteinuria. Hemorrhagic infarcts caused by platelet microthrombi occur in many organs in the brain they may cause stroke-like episodes (Matijevic and Wu 2006) although more commonly there is global encephalopathy. The blood film shows thrombocytopenia, hemolytic anemia and fragmented red cells. The differential diagnosis includes infective endocarditis, idiopathic thrombocytopenia, heparin-induced thrombocytopenia with thrombosis, systemic lupus erythematosus, non-bacterial thrombotic endocarditis and disseminated intravascular coagulation. 

In addition to penicillamine nephropathy, other side effects of the drug may be related to the widespread deposition of immune complexes (Figure 3). Dense, granular immunoglobuhn deposits have been identified at the epidermodermal junction in 4 rheumatoid arthritis patients who developed toxic reactions, such as severe rashes, thrombocytopenia, aplastic anemia, and proteinuria. Three of 4 penicillamine-induced systemic lupus erythematosus syndrome patients had similar findings on skin biopsy [161]. 

Repeated and prolonged exposure to PPD is believed to increase the risk of non-Hodgkin s lymphomas and multiple myeloma and cancer of fhe bladder [34-35]. Hair dye formulations containing PPD was incriminated in the increased risk of systemic lupus erythematosus (SEE) and breast cancer however other studies, showed that there is no significant relationship [36-37]. Aplastic anemia due to PPD exposure also has been reported [38]. 

Procainamide may induce a syndrome similar to systemic lupus erythematosus. This syndrome consists of arthralgias, myalgias, pleurisy, rash, fever, and elevated nuclear antibodies. Patients who are slow acetylators are at increased risk for developing this syndrome. While some studies have reported that less than one in 500 on chronic procainamide therapy developed this syndrome, others have reported this syndrome in up to 30% of patients on long-term therapy. Other side effects with chronic use include development of neutropenia, thrombocytopenia, hemolytic anemia, agranulocytosis, liver failure, a myasthenia-like syndrome, and psychosis with hallucinations. 

Systemic lupus erythematosus skin rasb (leadingto exfoliative dermatitis or severe erythema multiforme) fatal aplastic anemia fatal nephrosis has occurred Somnolence, fatigue (adults) agitation, insomnia (children) Somnolence, dizziness, ataxia, fatigue, and nystagmus... 

There is also a significantly increased incidence of IgA deficiency in patients with autoimmune or potentially autoimmune disorders, and usually it is not clear which came first. It can be argued that autoimmunity is a complication of immune imbalance subsequent to inborn IgA deficiency (H24). With inborn absence of IgA, exposure to normal human colostrum, plasma, and saliva can result in the production of antibodies to IgA. By the time such patients are discovered the etiological mechanisms are often obscured and IgA treatment is out of the question. The incidence of IgA deficiency is known to be 1-4% in the following conditions Still s disease, systemic lupus erythematosus, rheumatoid arthritis, Sjogren s disease, warm hemolytic anemia, megaloblastic anemia, idiopathic pulmonary hemosiderosis, thyrotoxicosis, and cirrhosis. 

Tubular Unresponsiveness to Aldosterone Certain medical conditions, such as sickle cell anemia, systemic lupus erythematosus, and amyloidosis, can produce a defect in tubular potassium secretion, possibly as the result of an alteration in the aldosterone-binding site. The exact mechanism of the tubular unresponsiveness is unknown. 

Anemias, systemic lupus erythematosus, vestibular dysfunction Compiled from House and Stark/ Cliatto/ and Chen et ai/... 

The antirheumatic action of D-penicillamine initially had limited application. However, in the early 1970s controlled trials have established the drug s intrinsic activity. There are many similarities to chrysotherapy both in clinical response (lowering of rheumatoid factor serum titer, decreased inflammatory response) and adverse effects (glomerulonephritis). However, some are potentially very serious, particularly aplastic anemia and systemic lupus erythematosus (SLE). The most important hint as to the uncertain mechanism of penicillamine s disease-suppressive properties is probably the considerable decrease in IgM (rheumatoid factor) levels. 

Systemic lupus erythematosus (SLE) is multisystem autoimmune disease characterized by the production of autoantibodies against a host of nuclear antigens. Lupus, the Latin word for wolf, has been used for at least seven centuries to describe the malar rash that some think resembles an animal bite. The disease was recogifized initially as a skin disease. Hebra and Kaposi were the first to report systemic involvement of the disease. Their work published in 1874 described the association of lupus with anemia, lung involvement, mental status changes, and death. In three papers published between 1895 and 1903, Sir William Osier described the systematic involvement of lupus, the first detailed description recogifizing the multiple systems affected by the disease. 

TOXICITY Common dose-related side effects are G1 complaints (nausea, vomiting, and anorexia) and CNS effects (drowsiness, lethargy, euphoria, dizziness, headache, and hiccups) some tolerance to these effects develops. Parkinson-like symptoms and photophobia also have occurred. Restlessness, agitation, anxiety, aggressiveness, inability to concentrate, and other behavioral effects have occurred primarily in patients with a history of psychiatric disturbance. Urticaria and other skin reactions, including Stevens-Johnson syndrome, as well as systemic lupus erythematosus, eosinophilia, leukopenia, thrombocytopenia, pancytopenia, and aplastic anemia have been attributed to the drug, and deaths have resulted from bone marrow depression. 

As with phenobarbital, serious toxicity for primidone is rare, although it may cause disabling sedation. Irritability, and decreased mental functioning In a number of persons. Ataxia, dysphoria, idiosyncratic rash, leukopenia, agranulocytosis, lymphadenopathy, hepatitis, and a systemic lupus erythematosus-like syndrome have been reported adverse effects for primidone. Deficiencies of folic acid and of vitamins D and K are possible with long-term therapy of primidone, as Is a folate-responsive megaloblastic anemia. Measurement of the complete blood cell count should be performed at 6-month intervals (40). 

Rituximab has been approved in the United States for relapsed or refractory LG/F NHL and in Europe for relapsed or refractory LG/F NHL and for aggressive NHL. Multiple other indications have been explored including hematologic malignancies - CLL, multiple myeloma, and so on autoimmune disorders - Immune Thrombocytopenic Purpura (ITP), heuma-toid arthritis. Systemic Lupus Erythematosus (SLE), hemolytic anemias, and so on (Figs. 5 and 6) [4, 59, 60]. 

Idiopathic thrombocytopenic purpura Acquired Factor VIII inhibitors Pure red cell aplasia Systemic lupus erythematosus Rheumatoid arthritis Hemolytic anemias... 

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