A case of systemic lupus erythematosus complicated by pure red cell aplasia and idiopathic portal hypertension after thymectomy

Abstract

We describe a 49-year-old woman who presented in 2002 with pure red cell aplasia (PRCA), systemic lupus erythematosus (SLE), and idiopathic portal hypertension (IPH) that developed following a thymectomy. She underwent a thymectomy at 40 years of age to treat myasthenia gravis. PRCA developed 3 years after the thymectomy and she was successfully treated with cyclosporin. Systemic lupus erythematosus and IPH were diagnosed 6 years later. We conclude that immunological dysfunction resulting from the thymectomy contributed significantly to the subsequent development of PRCA, SLE, and IPH in this patient. This is the first report to describe this extremely rare occurrence.     

A pair of monozygotic twins who are concordant for myasthenia gravis but became discordant for systemic lupus erythematosus post-thymectomy

We describe a pair of monozygotic twins who are concordant for myasthenia gravis but discordant for systemic lupus erythematosus (SLE). SLE developed in twin 1 18 years post-thymectomy and has been characterized by recurrent transverse myelitis and optic neuritis. Twin 2 remains well post-thymectomy, except for a skin rash and persistent leukopenia. Both twins have developed autoimmune thyroid disease. We review genetic and environmental factors of importance in the pathogenesis of SLE and discuss the possible role of thymectomy in the etiology of the disease.     

Pure red cell aplasia as presentation of systemic lupus erythematosus: antibodies to erythropoietin.

In this case report we describe two patients with pure red cell aplasia (PRCA) as an initial manifestation of systemic lupus erythematosus (SLE). Antibodies to erythropoietin were determined, by an ELISA method developed in our laboratory, in frozen serum obtained from one of the patients. A high titer of antibodies to erythropoietin was detected in serum obtained before treatment with high dose intravenous immunoglobulin (IVIG). The antibody titer declined after successful treatment. This observation suggests that antibodies to erythropoietin may contribute to the pathogenesis of SLE associated PRCA.     

Pure red cell aplasia induced by clomipramine hydrochloride in a patient with SLE

A 48-year-old woman, who had been suffering from systemic lupus erythematosus (SLE), developed normochromic normocytic anemia after receiving clomipramine hydrochloride. Her reticulocyte count was low, and a bone marrow aspirate revealed erythroid hypoplasia without involvement of other cell lines. Thus a diagnosis of pure red cell aplasia (PRCA) was made. The anemia gradually resolved following withdrawal of the drug. Although several drugs are known to cause PRCA, this is the first time that clomipramine hydrochloride has been reported to have such an effect. The underlying SLE in this case suggested the possible immunological pathogenesis of drug-induced PRCA.     

Systemic lupus erythematosus arising in a patient with epidermodysplasia verruciformis

Few cases have been published relating systemic lupus erythematosus (SLE) and epidermodysplasia verruciformis (EV). We describe a patient with this association. A 22-year-old female with classical skin lesions of EV since childhood together with systemic lupus erythematosus (SLE) which developed 10 years later. According to the knowledge of the authors, this case is the third report of association of EV and SLE and the first report of a patient with EV who developed SLE several years after initiation of EV.     

A sequence of pathologic events in a patient after thymectomy for myasthenia gravis

The case of a rare coexistence of myasthenia gravis (MG) with systemic lupus erythematosus (SLE) is described. MG was diagnosed prior to SLE which developed after thymectomy. The patient was affected by HCV viremia. Therefore, there were therapeutic problems. Metylase treatment was continued for several years and dopamine receptor agonist was effectively administered as adjunctive therapy in SLE.     

Pure red cell aplasia and lupus

OBJECTIVE: To review the clinical and laboratory features of all reported patients with systemic lupus erythematosus (SLE) and pure red cell aplasia (PRCA). METHODS: In addition to our patient, we identified cases reported during the years 1966-2000 by searching the MEDLINE literature (Winspirs). Clinical and laboratory features were compared with those reported in large series of patients with SLE but without PRCA. RESULTS: Twenty-three additional cases were identified. In most cases, SLE was diagnosed either before or concomitantly with the diagnosis of PRCA. The clinical and laboratory features were not significantly different from those reported in large series of patients with SLE, except for less pleuritis and a trend toward less proteinuria, hallucinations, thrombopenia, and leukopenia. The natural history of PRCA and SLE was similar to that reported for PRCA alone. The disease responded to prednisone in the majority of cases, but patients frequently remained steroid dependent. CONCLUSIONS: The association between SLE and PRCA is rare. The clinical and laboratory features of SLE in such patients are similar to SLE patients without PRCA with the exception of a decreased frequency of pleuritis. Response to treatment of PRCA in those with SLE is similar to patients with PRCA but without SLE.     

A case of pure red cell aplasia and immune thrombocytopenia complicating systemic lupus erythematosus: response to rituximab and cyclophosphamide

Pure red cell aplasia (PRCA) is a recognized but rare complication of systemic lupus erythematosus (SLE) and is characterized by the near absence of red blood cell precursors in the bone marrow but with normal megakaryocyte and granulocytes. We report a novel case of acquired PRCA occurring simultaneously with immune thrombocytopenia in the context of active SLE. Both syndromes were refractory to conventional treatment but responded to rituximab and cyclophosphamide.     

A case of pure red cell aplasia and systemic lupus erythematosus caused by human parvovirus B19 infection

Human parvovirus B19 (B19) rarely induces pure red cell aplasia (PRCA) in healthy hosts. Meanwhile B19 infection is often clinically similar to systemic lupus erythematosus (SLE), and several cases have been reported wherein B19 actually stimulated SLE exacerbation in an immunocompetent subject. An 82-year-old healthy woman was diagnosed to have complicated with B19 infection and PRCA. Four weeks later, she had high fever, polyarthritis, and oral ulcers, additionally diagnosed with SLE, and subsequently, 15 mg of prednisone was started. This is the first case wherein B19 infection caused both PRCA and SLE in a healthy patient as far as our investigations are concerned.     

Successful treatment of pure red cell aplasia in systemic lupus erythematosus with cyclosporin A

We report a patient with longstanding systemic lupus erythematosus (SLE) who developed pure red cell aplasia (PRCA). This condition is rare in connective tissue diseases and is reported in 32 previous cases of SLE in literature. Our patient recovered, apparently in response to treatment with high dosage of corticosteroids, but relapse occurred when the prednisone dosage was tapered down to 10 mg/day. The patient was successfully treated with cyclosporin A with no recurrence of the disease in the last 2 years.     

Congestive heart failure and axonal polyneuropathy induced by alfacalcidol in a patient with systemic lupus erythematosus

We describe the case of a 29-year-old woman with systemic lupus erythematosus (SLE), who developed congestive heart failure and severe axonal polyneuropathy after 9 months of treatment with alfacalcidol for the prevention of corticosteroid-induced osteoporosis. There was no evidence for the exacerbation of SLE. Moreover, both congestive heart failure and axonal polyneuropathy improved after discontinuation of the alfacalcidol without increasing the dose of corticosteroid. Since alfacalcidol is commonly used in the prevention of corticosteroid-induced osteoporosis, rheumatologists should be aware of the presence of this life-threatening adverse effect.     

Isoniazid-triggered pure red cell aplasia in systemic lupus erythematosus complicated with myasthenia gravis

A 47-year-old woman who had been treated for systemic lupus erythematosus (SLE) with myasthenia gravis (MG) was admitted to our hospital with acute onset of severe anemia after administration of isoniazid. Pure red cell aplasia (PRCA) was confirmed by elevated serum iron levels, reticulocytopenia and bone marrow aspiration showing a remarkable reduction of erythroblasts. Finally, cyclosporine A successfully improved PRCA. Although both SLE and MG have the potential complication of PRCA, we report here a case of isoniazid-triggered PRCA.     

Pemphigus vulgaris and systemic lupus erythematosus in a 46-y-old man.

Systemic lupus erythematosus (SLE) is an autoimmune disease that may affect many organs in the body. Skin manifestations are frequent and sometimes vesiculobullous lesions may apper such as in bullous lupus erythematosus. SLE may also be exceptionally associated with other blistering diseases such as pemphigus vulgaris (PV). We describe the case of a male diagnosed as having SLE, who one year later developed PV. Only three cases (all in women) of SLE associated with PV have been reported in the literature. Our case is noteworthy in that this exceptional association developed in a middle-aged male.     

Idiopathic portal hypertension associated with systemic lupus erythematosus

A case of idiopathic portal hypertension (IPH) associated with systemic lupus erythematosus (SLE) is reported in a 38-year-old man who had been diagnosed with SLE and treated for 18 years. Esophageal varices, found in 1994 on endoscopic examination, had been followed up for 2 years. On July 16, 1996, he was admitted to Nagoya University Hospital because there was a high risk of bleeding from the esophageal varices due to severe thrombocytopenia. As partial splenic embolization had temporarily controlled the thrombocytopenia, splenectomy and devascularization of the stomach vessels were performed after endoscopic ligation of the esophageal varices. Histological specimens of wedge biopsied liver showed chronic inactive hepatitis without cirrhosis. The presence of anticardiolipin antibody, indicated by positivity for lupus anticoagulant, was suggestive of the presence of a common immunological mechanism in the etiology of SLE and IPH. Received: January 20, 1999 / Accepted: July 23, 1999     

Congestive heart failure and axonal polyneuropathy induced by alfacalcidol in a patient with systemic lupus erythematosus

Abstract

We describe the case of a 29-year-old woman with systemic lupus erythematosus (SLE), who developed congestive heart failure and severe axonal polyneuropathy after 9 months of treatment with alfacalcidol for the prevention of corticosteroid-induced osteoporosis. There was no evidence for the exacerbation of SLE. Moreover, both congestive heart failure and axonal polyneuropathy improved after discontinuation of the alfacalcidol without increasing the dose of corticosteroid. Since alfacalcidol is commonly used in the prevention of corticosteroid-induced osteoporosis, rheumatologists should be aware of the presence of this life-threatening adverse effect.     

Treatment of acute neuropsychiatric lupus with intravenous immunoglobulin (IVIG): a case report and review of the literature

Neuropsychiatric lupus can be difficult to diagnose, and little prospective data exists to help direct management. In this case report we describe the acute onset of symptoms of depression, mania, and psychosis and their complete resolution 48 h following a 5-day treatment course of intravenous immunoglobulin (IVIG) in a 20-year-old woman with systemic lupus erythematosus (SLE). We review the literature on IVIG for the management of neuropsychiatric lupus. We propose that when more toxic therapies are refused or symptoms do not remit with other treatments, IVIG should be considered in patients with neuropsychiatric lupus.     

New onset systemic lupus erythematosus with pheochromocytoma

We describe a patient with positive antinuclear and anti-Smith antibodies, proteinuria, and thrombocytopenia suggesting systemic lupus erythematosus (SLE). During hospitalization, the patient developed labile hypertension, tachycardia, and intermittent fever. A computer tomography scan of the abdomen showed an extraadrenal mass, which was confirmed as a pheochromocytoma. After removal of the pheochromocytoma, the patient's symptoms resolved and her serology normalized. Previous case reports describe SLE patients with adrenal pheochromocytomas that presented many years after the diagnosis of lupus. This is a novel case of pheochromocytoma discovered at the onset of SLE, with resolution of SLE manifestations shortly after its removal.     

Systemic lupus erythematosus presenting as thrombotic thrombocytopenic purpura

We describe a 10-year-old girl, who presented with thrombotic thrombocytopenic purpura (TTP) and shortly thereafter developed systemic lupus erythematosus (SLE). The association between TTP and SLE is known, but this is the first report of SLE presenting as TTP.     

A case of idiopathic portal hypertension associated with systemic lupus erythematosus

A 29-year-old male who was diagnosed as having systemic lupus erythematosus (SLE) with hemolytic anemia and renal dysfunction at the age of 18 was diagnosed as having idiopathic portal hypertension (IPH) by angiography and liver biopsy. Improvement of thrombocytopenia and proteinuria, and transient increase of complement was observed after transabdominal devascularization with splenectomy (Hassab’s operation). The present case suggests that one of the aspects of the pathogenesis of IPH may be related with autoimmune mechanisms seen in patients with SLE.     

Pure red cell aplasia in procainamide induced systemic lupus erythematosus. Report and review of the literature

We describe a patient who developed pure red cell aplasia as part of a lupus-like syndrome while taking procainamide. Pure red cell aplasia resolved spontaneously after discontinuation of this drug. Although pure red cell aplasia has been reported in several patients with idiopathic systemic lupus erythematosus (SLE), this represents the first such report in a patient with drug induced SLE.