CRIOGLOBULINEMIA ESENCIAL MIXTA PDF

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Cryoglobulins are immunoglobulins that precipitate at cold temperatures. Their presence can be related to a type of vasculitis referred to as cryoglobulinemia. We describe the case of a patient with seronegative polyarthritis who developed acute arterial ischemia in association with cryoglobulinemia, with a good response to rituximab therapy..

Su presencia puede asociarse a un tipo de vasculitis denominada crioglobulinemia. Polyarthritis is a common reason for seeing the doctor and is a challenge for rheumatologists because of its extensive differential diagnosis.

In clinical practice, the development of new symptoms and analytical findings during patient follow-up can lead to a change in the diagnosis and, thus, of the treatment begun. Cryoglobulinemia is a vasculitis characterized by the involvement of small and medium-sized vessels.

The clinical manifestations of cryoglobulinemia can range from nonspecific symptoms to renal, neuropathic, joint and skin involvement. This means that, on occasion, these symptoms can be confounded with or overlap those linked to the CTD with which they are associated. We report the case of a patient whose presenting symptom was polyarthritis and who, during follow-up, was diagnosed with cryoglobulinemia associated with systemic lupus erythematosus SLE , with a good response to immunosuppressive therapy with rituximab.

She presented with a 2-month history of polyarthritis in hands, feet and ankles. Treatment was initiated with nonsteroidal anti-inflammatory drugs and prednisone, and she was scheduled to undergo a complete study of polyarthritis. Laboratory tests showed a C-reactive protein CRP of Thyroid hormones, liver function and renal function were normal.

Mantoux and booster, and serological testing for hepatitis B and C and human immunodeficiency virus were negative. Radiographs of hands, feet and thorax were normal. Despite the treatment, arthritis persisted in hands, carpal bones, feet and ankles. The patient stopped coming to our office.

In October of , she suddenly began to detect cyanosis and cold in the distal phalanx of the index finger of her right hand, and she was admitted to the hospital. She underwent computed tomography angiography, which showed an occlusion of the right radial artery at the level of the anatomical snuffbox; the rest of the study including supra-aortic trunks, study of upper limb arteries, lower limb arteries and abdominal aortography was normal, and there were no signs of vasculitis Fig.

Electrocardiogram and echocardiogram were normal. Laboratory tests revealed iron-deficiency anemia hemoglobin 8. During her hospital stay, treatment was begun with low-molecular weight heparin with acetyl salicylic acid ASA and intravenous alprostadil. The corticosteroid dose was increased and she continued with MTX and leflunomide which she had continued to take although she did not come for checkups.

She was discharged from the hospital, while awaiting the results of an analysis for cryoglobulins. Occlusion of right radial artery at the level of the anatomical snuffbox. In successive checkups in our offices, the patient showed improvement in terms of ischemia but polyarthritis persisted. The result for cryoglobulins was positive. Given the persistence of polyarthritis although she continued to take MTX and leflunomide and cryoglobulinemia, it was decided that she begin rituximab.

Anticoagulation was maintained with acenocoumarol for a year, until the cryoglobulins were negative, when antiplatelet therapy was begun with ASA. During follow-up, the patient was repeatedly positive for ANA at low titers, with a homogeneous pattern, and was negative for anti-dsDNA.

The immunological study was extended and we found antinucleosome antibodies and antibodies against the collagen-like region of C1q. She has not had any further ischemic episodes and there is no evidence of involvement of kidneys or of any other organ. We report the case of a patient with polyarthritis, a frequent cause for seeking advice from a rheumatologist, and a challenge for the latter because of its extensive differential diagnosis.

However, the development of new symptoms and analytical data recorded during follow-up, can make you reconsider the initial diagnosis or even change it, as occurred in the case we present.

Ultimately, the patient met the criteria for a diagnosis of SLE, as she was ANA-positive and anti-dsDNA-positive and had arthritis, lymphopenia according to the criteria of the Systemic Lupus International Collaborating Clinics 3 and mixed cryoglobulinemia.

We found antinucleosome and anti-C1q antibodies in this patient. The antinucleosome antibodies could be a more accurate marker for the diagnosis of SLE, 4,5 with a similar specificity and a slightly higher sensitivity than anti-dsDNA, but with a highly superior diagnostic odds ratio 41 vs These antibodies are capable of binding to different components of the glomerular basement membrane like collagen, membrane surface proteins, DNA-histone complexes and chromatin, 6 producing renal damage.

For this, they are considered nephritogenic, and have also been associated with disease activity measured using different questionnaires Systemic Lupus Erythematosus Disease Activity Index, European Consensus Lupus Activity Measurement, etc. The activation of the classical complement pathway begins with the molecule C1q, which is essential in the clearance of immune complexes and apoptotic cell debris.

Hereditary C1q deficiency is associated with high susceptibility for the development of SLE, especially the mucocutaneous manifestations. In any case, the use of corticosteroids and immunosuppressive therapy may have contributed to the maintenance of negative anti-dsDNA antibodies throughout her course. Moreover, the presence of cryoglobulins coincided in time with the autoimmune conditions described above.

They are classified according to 3 forms depending on the composition of the precipitate: - Type I cryoglobulinemia involves a single type of monoclonal immunoglobulin.

It is usually associated with lymphoproliferative diseases and multiple myeloma. The digital necrosis is adverse in the prognosis of MC, as it has been associated with a high risk of infections, sepsis and death.

The pathogenic role of antinucleosome and anti-C1q antibodies in the case reported is unknown to us. We consider that they are serological markers of SLE.

The usual clinical signs are the consequence of the deposition of circulating immune complexes in the wall of small and medium-sized vessels, which triggers an inflammatory response that leads to systemic vasculitis.

The use of plasmapheresis has been reported when the remaining approaches have been ineffective. The utilization of anti-CD20 monoclonal antibodies has been found to be beneficial in the treatment of systemic vasculitis and, although the data we have in cryoglobulinemic vasculitis not associated with hepatitis C virus is limited, certain case series support the use of rituximab in these patients. In conclusion, the particular feature of this case lies in the presentation of the MC in the setting of SLE, as an acute arterial ischemia right radial artery occlusion in a patient who had initially been diagnosed as having a seronegative polyarthritis.

Despite the presence of nephritogenic antibodies, the patient has not developed renal involvement, remains in remission and continues to take rituximab. The authors declare that no experiments were performed on humans or animals for this study. The authors declare that no patient data appear in this article. The authors declare they have no conflicts of interest. Isquemia arterial aguda en paciente con poliartritis. Reumatol Clin.

ISSN: Open Access Option. Previous article Next article. Issue 2. Pages March - April Download PDF. Corresponding author. This item has received. Article information. We describe the case of a patient with seronegative polyarthritis who developed acute arterial ischemia in association with cryoglobulinemia, with a good response to rituximab therapy.

Palabras clave:. Introduction Polyarthritis is a common reason for seeing the doctor and is a challenge for rheumatologists because of its extensive differential diagnosis. Foessel, J. Besancenot, G. Blaison, N. Magy-Bertrand, R. Jaussaud, Y. Etienne, et al. Clinical spectrum, treatment and outcome of patients with type ii mixed cryoglobulinemia without evidence of hepatitis C infection.

J Rheumatol, 38 , pp. Aletaha, T. Neogi, A. Silman, J. Funovits, D. Felson, C. Bingham 3rd, et al. Arthritis Rheum, 62 , pp. Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus. Arthritis Rheum, 64 , pp. Yu, M. Gershwin, C. Diagnostic criteria for systemic lupus erythematosus: a critical review.

J Autoimmun, 48—49 , pp. Bizzaro, D.

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Virus y vasculitis sistémicas

Existen tres tipos, siendo las de tipo II y III las que forman parte de las denominadas crioglobulinemias mixtas. Biologic and clinical significance of cryoglobulins. Am J Med , Nomenclature of systemic vasculitides: the proposal of an international consensus conference. Arthritis Rheum ; Causes and predictive factors of mortality in a cohort of patients with hepatitis C virus-related cryoglobulinemic vasculitis treated with antiviral therapy.

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Crioglobulinemia mixta esencial tipo II: presentación de un caso

Published studies are primarily clinical and epidemiological research but also basic. CiteScore measures average citations received per document published. Read more. SRJ is a prestige metric based on the idea that not all citations are the same.

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