How long does idiopathic edema last

Idiopathic cyclic edema; Idiopathic orthostatic edema; Fluid retention syndrome; Idiopathic cyclic edema; Idiopathic orthostatic edema; Fluid retention syndrome; Cyclical edema See More. Summary Summary. Treatment Treatment. Most individuals with idiopathic edema experience a decrease in swelling and fluid retention with the introduction of a low sodium, low carbohydrate diet. For individuals already taking diuretics, a brief discontinuation may result in symptom improvement as well.

Individuals who do not respond to initial treatment may be difficult to treat; however, other therapies such as use of hypertension medications ACE inhibitors have shown success in some cases. Do you have updated information on this disease? We want to hear from you. Learn More Learn More. Where to Start MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic. In-Depth Information PubMed is a searchable database of medical literature and lists journal articles that discuss Idiopathic edema.

Click on the link to view a sample search on this topic. Have a question? References References. J Am Board Fam Med. When left untreated, the tendency for edema to develop upon abrupt cessation of chronic excessive purging can interfere with successful eating disorder treatment Brown and Mehler, In addition to discontinuing the purging behaviors, treatment of edema in AN-BP patients who purge or use laxatives involves addressing the core hyperaldosterone response and correcting electrolyte disturbances.

Similar to AN-R. One must take into account the avid response to saline due to elevated aldosterone levels, which can lead to an undesired further state of increased retention of salt and water. Thus, judicious administration of said fluids can help prevent a state of volume overload.

Once volume is corrected, the metabolic alkalosis will resolve. In addition to considering administration of gentle intravenous fluids, use of the potassium-sparing diuretic, spironolactone, is strongly recommended. Spironolactone antagonizes the effects of aldosterone in the distal renal tubule. Thus, it can minimize edema formation secondary to excessive aldosterone levels. Further, its mild diuretic effect can help treat edema that may still occur.

Effective daily dosing ranges from 25 mg to mg; lower doses may be utilized initially and up-titrated as needed for increased effect if edema formation and weight gain are ongoing. Some guidelines recommend starting with mg and up-titrating to a maximum effective dose of mg, with up-titration every 3 to 4 days Runyon, In our practice, we generally do not administer doses higher than mg. Alternative classes of more potent diuretics, such as thiazides and loop diuretics, should generally be avoided because they can exacerbate activation of the renin-angiotensin-aldosterone axis and worsen the metabolic alkalosis.

Rarely, they are judiciously required for severe edema formation with excessive edema formation despite spironolactone. However, in this complex situation, triple therapy must be initiated by those with specialized medical expertise. In addition to medical therapies, given the distressing nature of edema in eating-disordered patients, forewarning and reassurance regarding the transient nature of edema and therapeutic support are other important components of treatment Ehrlich et al.

Failure to recognize the importance of behavioral interventions or the distress caused by edema, as well as a lack of understanding of the physiologic changes associated with edema formation, may lead patients to avoid seeking care, given previous negative experiences with edema formation and weight gain in treatment settings. Development of edema in patients with eating disorders is a complex phenomenon.

When recognized early and treated appropriately, edema can be managed quite effectively, minimizing patient distress. Doing so requires an understanding of the distinct mechanisms that allow clinicians to judiciously manage therapeutic interventions. These include administration of intravenous fluids if the person is in an inpatient medical setting and use of the potassium-sparing diuretic spironolactone, or simply recommending leg elevation.

These interventions can mitigate the significant volume overload that can occur in patients with edema secondary to volume loss in the setting of purging, or diuretic or laxative use.

In addition, providing appropriate education and therapeutic support with leg elevation for those who develop edema from early treatment of AN-R can serve to further support patients in whom edema is a significant source of distress during treatment. Immunoglobulin G 2 antibody is not functional and may be responsible for false positives seen with binding assays such as immunoblot or enzyme-linked immunosorbent assay; thus a functional assay is still needed to identify the autoimmune subpopulation.

Release of leukotrienes and cytokines is observed in addition to histamine; thus, cellular infiltration ensues, which resembles that seen in the allergic late-phase reaction [ 17 ]. Diagram of the activation of cutaneous mast cells by IgG antibody directed to the IgE receptor. The lymphocytes are a mixture of the Th1 and Th2 cells [ 19 ]. Features that distinguish this inflammatory response from a typical allergic late-phase reaction are the aforementioned T helper subgroups compared with the Th2 predominance seen with atopy, and more neutrophils and monocytes, which may relate to the chemotactic activity of C5a.

When eosinophils are not evident, a major basic protein can be seen, suggesting degranulation and loss of identifying markers [ 20 ]. Additional mechanisms or abnormalities in cellular function have been observed, but these require corroboration and considerably more work. One group has reported the presence of IgG antibody to the low-affinity IgE receptor Fc e RII on eosinophils, which activates these cells, and the liberated products activate basophils and, presumably, cutaneous mast cells [ 21 ].

Another group has presented evidence that the tissue factor pathway of blood coagulation is activated in chronic urticaria with strong evidence that this is so [ 22 ] and proposes thrombin as a histamine-releasing agent.

The group does not, however, distinguish a primary process involving blood coagulation versus release of tissue thromboplastin as a consequence of histamine or leukotriene activation of endothelial cells. Lastly, in , Kern and Lichtenstein[ 23 ] noted that the basophils of chronic urticaria patients are hyporesponsive to anti-IgE. We know that chronic urticaria patients are basopenic[ 24 ] presumably caused by chemotactic migration into skin, but this implies a functional abnormality.

Luquin et al [ 25 ] confirmed hyporesponsiveness of basophils of patients with chronic urticaria to stimulation with anti-IgE and also noted hyperresponsive basophils ie, augmented histamine release from the same chronic urticaria basophils if they are incubated with normal serum. The nature of the serum factor and the mechanism of cellular hyperresponsiveness are unknown. However, hyporesponsiveness to anti-IgE was recently shown to define a subpopulation of patients, but it is not the same subpopulation as the autoimmune subgroup [ 26 ].

The defect seems to be caused by a decreased activity of critical phosphatases that normally limit basophil responsiveness. One report also found an abnormality in Ras signal transduction in chronic urticaria basophils [ 27 ].

These data all point to a cellular abnormality found in basophils of patients with chronic urticaria but not yet shown in mast cells that may be seen even in patients we currently call idiopathic. Treatment of chronic urticaria and angioedema has been reviewed in detail[ 28 ] and will be summarized. Second-generation nonsedating antihistaminics are tried first using a double dose if necessary. European guidelines[ 29 ] even suggest a 4-fold increase in dosage; however, cost in the United States would be particularly problematic.

Dietary measures to eliminate pseudoallergens[ 30 ] are advocated by some; however, the mechanism of the effect, if any, is not clear. If symptom control is not adequate, a first-generation antihistamine such as hydroxyzine or diphenhydramine can be used in divided doses at 25 to 50 mg QID for adults.

The H 2 receptor antagonists or leukotriene antagonists may be added; however, at best, they add only a small increment in symptom control. Acute episodes of angioedema can be treated with 40 to 60 mg prednisone in a single dose, which can be repeated the following day if needed. Corticosteroid is then discontinued without any taper. Cutaneous vasculitis presenting as urticaria with or without angioedema can be seen as part of a diffuse connective tissue disorder or can be seen as a separate entity with skin as the only organ affected [ 32 ].

The hives last longer hours. On biopsy, neutrophils predominate with fragmented cells leukocytoclastic angiitis. When there is no identifiable underlying disease, it is often designated as idiopathic leukocytoclastic angiitis. Angioedema may be seen, but urticaria predominates. Disorders to consider are systemic lupus erythematosus, cryoglobulinemia, polyarteritis nodosa, Wegener granulomatosis, and Sjogren syndrome. Treatment is directed to the underlying disorder as well as the skin manifestations.

Symptoms are often refractory to antihistamines, although they should be tried first. Patients may require low-dose daily corticosteroid to control symptoms; however some patients respond to dapsone, colchicine, or hydroxychloroquine. One specific entity known as the hypocomplementemic urticarial vasculitis syndrome is characterized by a circulating IgG antibody to C1q with lowC4 and C3.

The urticaria is particularly responsive to hydroxychloroquine. True allergic reactions are rare, and these appear as class-specific idiosyncratic reactions dependent upon the drug's inhibitory capacity for COX-1 inhibitors [ 33 ]. It is postulated that COX inhibition shunts arachidonic acid through the lipoxygenase pathway with excessive leukotriene production.

Leukotrienes, particularly LTC4 and LTD4, are vasoactive substances leading to erythema and edema formation, whereas LTB4 is chemotactic for a variety of cell types, particularly neutrophils, and to a lesser degree, eosinophils. Weak COX-1 inhibitors such as acetaminophen and salicylates other than aspirin are usually well tolerated, as are COX-2 inhibitors. Angiotensin-converting enzyme inhibitors, developed from peptides found in venom from the Malayan snake Bothrops jararaca and known to enhance bradykinin activity, were first introduced in for treatment of hypertension and congestive heart failure.

Furthermore, although angioedema may occur during the first week of therapy, some patients may have taken the ACE inhibitor without any problem for weeks or months before angioedema develops [ 34 ]. The ACE inhibitors are often overlooked as a cause of angioedema, and this may lead to unfortunate consequences because continuing administration tends to lead to more severe attacks [ 35 ]. The overall incidence of ACE-induced angioedema is reported to be about 0.

There is no preponderant age or sex bias. The ACE inhibitor-induced angioedema is not dose related; however, it may occur almost immediately after the first dose, and is class-, but not drug-, specific and is therefore unlikely to be immunologically mediated. The ACE catalyzes the transformation of angiotensin I to angiotensin II which is vasoconstrictive and raises the blood pressure [ 39 ]. The ACE inhibitor effects upon both of these enzymatic pathways lead to lowering of blood pressure and a potential elevation of bradykinin levels [ 41 ].

Bradykinin, a nonapeptide, is powerfully vasoactive in human skin, [ 42 ] and angiotensin convertase inhibition has been demonstrated to cause elevation of plasma kinins in human subjects [ 41 ]. A dual ACE and neutral peptidase inhibitor, omapatrilat, carries an even higher risk of angioedema than pure ACE inhibitors alone [ 43 ]. Captopril has also been shown to inhibit degradation of the vasoactive neuropeptide substance P in the rabbit, [ 44 ] but this is probably a less important pathway in man.

Why only a small minority of patients receiving ACE inhibitor treatment develops this dramatic complication is unclear [ 45 ]. The ACE is the predominant inactivator of bradykinin and is found along the endothelial cells of the pulmonary vasculature [ 46 ]. With ACE inhibition, bradykinin accumulates and interacts with vascular B-2 receptors to cause vasodilation and to increase vascular permeability, with a concomitant increase in cyclic guanosine monophosphate and release of nitric oxide.

When ACE is inhibited, bradykinin inactivation is slow and dependent on plasma carboxypeptidases CpN or in serum, CpU , which remove C-terminal Arg, [ 40 ] leaving an octapeptide that, during chronic inflammatory states, can interact with B-1 receptors.

The other products of ACE do not react with either bradykinin receptor. These products have no activity on kinin receptors. The remainder can become symptomatic weeks, months, or occasionally years later. It shows a predilection for the head, neck, lips, mouth, tongue, larynx, pharynx, and subglottal areas[ 47 ] without urticaria.

Mouth involvement may not only compromise the airway but cause dysarthria, thus preventing an adequate case history. Intestinal edema may also occur, causing abdominal pain that may not be accompanied by visible muco-cutaneous angioedema.

Thus, sudden abdominal pain, diarrhea, and vomiting in an adult should prompt questioning about recent intake of an ACE inhibitor [ 48 , 49 ]. Hereditary angioedema HAE, vide infra may present with an identical history and clinical picture, and like ACE inhibitor-induced angioedema, is not associated with urticaria. This seems to be a presentation associated with bradykinin-mediated swelling; ACE inhibition leads to an abnormality in bradykinin degradation, whereas C1 INH deficiency causes overproduction.

Emergency treatment should take into account the risk of relapses after apparent recovery from the initial episode, despite withdrawal of the offending drug [ 50 , 51 ]. Therefore, patients should be admitted for observation at least overnight.

Other emergency procedures and drug administration are essentially the same as treatment of acute allergic angioedema vide supra. Epinephrine may slow or stop the rate of swelling; antihistaminics and steroid, although often administered, are unlikely to have any effect. The patient should not be subsequently prescribed another ACE inhibitor because the reaction is class-, but not drug-, specific and a Medic Alert bracelet should be worn.

It is also advisable to check the complement C4 level because patients with preexisting angioedema, including HAE caused by C1 esterase inhibitor deficiency, are predisposed to develop angioedema in response to ACE inhibitors [ 52 ]. In general, angiotensin II receptor antagonists are tolerated by patients who have reacted to ACE inhibitors [ 53 ].

Severe face, tongue, and pharyngeal edema in a patient treated with an ACE inhibitor. Having encountered, in , a year-old woman with recurrent attacks of angioedema, Osler obtained a history of similar episodes of edema in no less than 5 generations of the patient's family, beginning with the woman's great grandmother born in Thus, Osler established the dominantly inherited basis of a subset of patients with relapsing angioedema.

Hereditary angioedema is a dominantly inherited disease that affects about , persons. It has been reported in all races, and there is no sex bias in the classical forms types 1 and 2. The classical type of HAE is caused by a quantitative type 1 or functional type 2 defect in the plasma inhibitor of the first component of complement C'1 INH. The C1 INH gene has been mapped to chromosome 11 with considerable genetic heterogenicity when mutations responsible for the diseases are defined.

Recently, a third type 3 form of HAE has been reported by several groups occurring exclusively in women with quantitatively and functionally normal C1 INH activity with a relationship to estrogenic activity.

The disease was first demonstrated by Virginia Donaldson[ 54 ] to be caused by a defect in the serpin serine protease inhibitor C1 INH. This deficiency causes an abnormal increase in activation of C1, leading to consumption of C2 and C4 and correspondingly low plasma levels of these proteins.

It also causes excessive formation of the enzyme kallikrein, resulting in increased transformation of kininogen to kinins including the highly vasoactive nonapeptide bradykinin. The evidence is now substantiated that bradykinin is the cause of the swelling[ 56 - 62 ] rather than any vasoactive peptide resulting from complement activation.

The HAE type 2 includes 2 variants, one with a functionally inactive protein present in normal immunoreactive amounts and a second with a nonfunctioning inhibitor present in increased concentration and bound to albumin [ 67 ].

Diagrammatic representation of the plasma kinin-forming cascade indicating steps inhibitable by C1 INH. All functions of factor XIIa and kallikrein are affected. Patients are usually asymptomatic up to puberty.

Although swellings may develop without any preceding trauma, more than half of patients with HAE report some, usually minor, localizing injury such as dental maneuvers. Other precipitating factors include vigorous exercise, alcohol consumption, emotional stress, and hormonal factors [ 68 ]. Coadministration of angiotensin convertase enzyme inhibitors and estrogens are contraindicated in HAE. There is also a transitory prodromal nonpruritic urticarial eruption in some patients. The main sites of cutaneous involvement are the face, hands, arms, legs, genitalia, and buttocks, and the swellings may slowly spread and persist for 3 to 4 days.

Involvement of the mucosae is especially feared, and glossal, pharyngeal, or laryngeal involvement can result in respiratory obstruction, asphyxia, and a fatal outcome. Presentation with abdominal pain and symptoms of intestinal obstruction is also common, and any patient presenting to the emergency department with these symptoms, and evidence of multiple abdominal surgical scars should be screened for HAE [ 69 ]. That pulmonary involvement is very rare is probably because of high tissue levels of kininases that rapidly inactivate kininlike peptides.

There is an increased frequency of several autoimmune diseases with HAE. Associated coagulopathies have also been reported[ 71 , 72 ] due, at least in some cases, to abnormally high levels of C4-binding protein, which also binds and inactivates proteins S, [ 71 ] a cofactor for the protein Ca inactivation of activated coagulation factors V and VIII. For acute emergency episodes, antihistamines and corticosteroids are ineffective, although subcutaneous adrenaline 0.

If the patient has serious respiratory obstruction, intubation or tracheostomy should be carried out and may be lifesaving. However, most acute episodes are non-life threatening, and the mainstay of emergency medical treatment is intravenous fresh frozen plasma or C1 INH concentrate [ 72 ]. The recent availability of lyophilized vapor-heated C1 INH concentrate[ 73 ] has largely removed concerns about transmission of human immunodeficiency virus, viral hepatitis, and other infections.

In this study, the product used Immuno, Vienna, Austria after reconstitution with saline contained plasma units in a mL vial and was administered at a dose of 25 plasma units per kg body weight to a total of plasma units repeated once if necessary. It is usually effective in diminishing swelling within 3 to 4 hours, and often within minutes. New agents now available for treatment of acute episodes of angioedema include an inhibitor of kallikrein[ 74 ] and a bradykinin receptor antagonist [ 75 ].

Each of these is given by a subcutaneous injection. Androgens were first demonstrated to be effective for prevention of episodes of HAE by Spaulding in , [ 76 ] and antifibrinolytic agents[ 77 ] have also been used. Other reversible side effects in women include deepening of voice, menstrual irregularities, acne, and hirsutism.

In patients in whom attacks are infrequent and not life-threatening, it may be sufficient to restrict treatment to avoidance of known provoking factors including estrogens and ACE inhibitors together with administration of C1 INH concentrate or fresh frozen plasma prophylactically before dental treatment or other minor surgical procedures. The clinical picture including mucocutaneous swellings, respiratory obstruction, and abdominal symptoms did not differ significantly from types 1 or 2 HAE.

There was no urticaria. However, C1 INH concentrate therapy was ineffective. Provoking factors included estrogens and pregnancy. These and other authors propose an X-linked inheritance for this new entity [ 81 ]. Angioedema clinically identical with the hereditary varieties can be caused by acquired C1 INH deficiency. In , Costanzi et al [ 82 ] reported a patient with cold urticaria who had acquired C1 INH deficiency associated with a monoclonal cryoglobulin.

Subsequently, a large number of other patients with a similar syndrome were reported mostly occurring in the setting of lymphoproliferative disease. Occurring in older age groups and without a family history, the underlying mechanism seems to be overconsumption of C1 INH. One of the earliest descriptions of the acquired form of this disease was seen in patients with lymphoma who have circulating low-molecular-weight IgM and depressed C1 INH levels.

This entity has an unusual complement utilization profile because C1q levels are low and C4, C2, and C3 are depleted. The low C1q level differentiates this condition from the hereditary disorder [ 83 - 85 ]. The depressed C1 INH level may be caused by depletion secondary to C1 activation by circulating immune complexes or C1 interaction with a tumor cell surface antigen. For B-cell lymphoma, the most common associated malignancy, C1 fixation and C1 INH depletion are caused by an anti-idiotypic antibody bound to immunoglobulin on the surface of the B cell [ 86 ].

Patients with connective tissue disorders such as systemic lupus erythematosus or carcinoma[ 87 , 88 ] can present with acquired C1 INH deficiency, and like patients with the hereditary form, will respond to androgen therapy, which enhances C1 INH synthesis. This form of acquired C1 INH deficiency seems to be increasingly recognized. Under normal circumstances, C1 INH is a substrate for the enzymes it inactivates: the active enzyme cleaves C1 INH, which exposes the active site in the inhibitor.

The cleaved C1 INH then binds stoichiometrically to the enzyme and inactivates it. Thus, cleaved functionless C1 INH circulates an unopposed activation of the complement- and kinin-forming cascade.

One circumstance in which the 2 forms of acquired C1 INH deficiency merge is in an occasional patient with monoclonal gammopathy, in which the monoclonal immunoglobulin is in fact an antibody to C1 INH [ 94 , 95 ].

The type 2 variety can be most readily determined by immunoblot with antibody to C1 INH. The presence of a C1 INH cleavage product at 95 kd differentiates the 2 forms of the acquired disorder, and it is not present in the hereditary disorder. If a family history is present, one needs to differentiate the 2 types of HAE.

Both protein and function will be low in parallel in type I HAE, whereas the protein level will be normal or elevated in type 2 form of this disorder with functional C1 INH diminished. Amino acid sequence analysis of the gene would be required to prove it.

Occasionally, the pattern of acquired C1 INH deficiency may be obtained in someone who does not have any of the aforementioned predisposing immune conditions. Such patients need to be evaluated carefully over time because there have been reports of angioedema of this sort preceding the diagnosis of the underlying disorder.

Treatment of acquired C1 INH deficiency requires, first, treatment of the underlying disease, if one has been identified, plus treatment with the aforementioned drugs, which is essentially the same as that for treatment of the hereditary disorder. The latter is clearly preferable to prevent volume overload and to be able to give enough C1 INH to bind the autoantibody so as to raise the C1 INH level significantly.

In a practical sense, this is often not feasible. Traditionally, this is assumed to be a subgroup of patients with chronic urticaria and angioedema, when the sole manifestation is angioedema. Symptoms can very from episodes of swelling that occur a few times a year, that is, every few months, to those with frequent episodes a few times each week. No allergic etiology can be found specifically no reactions to foods or drugs , their health is otherwise normal, complement studies C4, C1 INH by protein and function are normal, and there is no familial predisposition.

However, laryngeal edema and edema of the bowel is not seen, thus it differs from the angioedema associated with ACE inhibitors or C1 INH deficiency. In this sense, it resembles closely the angioedema that is seen accompanying urticaria in those with idiopathic or autoimmune chronic urticaria.

Nevertheless, differences have been noted. First, idiopathic angioedema has no sex predelineation or may be slightly more prevalent in men, whereas two thirds of patients with chronic urticaria are female. The pathogenesis of this disorder is not known. Recent attempts to further characterize these patients have divided them into 2 groups depending on responsiveness to antihistamine therapy [ 97 ]. The histaminergic group responds to antihistamine prophylaxis.

We first use nonsedating antihistamines cetirizine, desloratadine, fexofenadine, etc at double the usual dose. If angioedema continues, we try diphenhydramine at 25 to 50 mg QID.