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

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 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.     

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.     

A case of idiopathic portal hypertension associated with systemic lupus erythematosus

Abstract

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.     

A case of systemic sclerosis complicated by idiopathic portal hypertension: case report and literature review

We encountered a 62-year-old woman who had systemic sclerosis (SSc) complicated by idiopathic portal hypertension (IPH). She had a 10-year history of scleroderma and Raynaud's phenomenon. She also had pancytopenia, splenomegaly, and esophageal varices. Treatment with prednisolone and endoscopic variceal ligation resulted in improvement of her symptoms. According to our literature review, the prognosis of patients with SSc complicated by IPH is relatively poor. However, the factors that predict outcome of these patients have not been elucidated.     

Successful treatment of pure red cell aplasia associated with systemic lupus erythematosus with oral danazol and steroid

We describe a middle-aged Chinese systemic lupus erythematosus (SLE) patient developing steroid refractory and transfusion dependent red cell aplasia. Oral danazol 200 mg twice per day was started together with low-dose prednisolone therapy. There was no further recurrence of anemia 1 month after this combined therapy.     

Clinical aspects of pulmonary hypertension in patients with systemic lupus erythematosus and in patients with idiopathic pulmonary arterial hypertension

Clinical aspects and pathology of pulmonary hypertension (PH) in patients with systemic lupus erythematosus (SLE) have been reported to be similar to those in patients with idiopathic pulmonary arterial hypertension (IPAH). To determine whether PH in these patients is similar, we compared the clinical characteristics, hemodynamics at diagnosis, and survival in groups of patients with SLE–PH and IPAH. We reviewed the case records of 20 patients with SLE–PH and 34 patients with IPAH, who had been assessed by echocardiography or right cardiac catheterization at Asan Medical Center, Seoul, Korea, from January 1995 to October 2003. Clinical features, laboratory data, chest X-rays, electrocardiogram results, pulmonary function tests, pulmonary perfusion scans, echocardiographic findings, serologic profiles, and survival were compared in the two groups of patients. The mean follow-up period was 18.1±20.6 months for patients with SLE–PH and 33.0±23.4 months for patients with IPAH. During follow-up, 12 SLE–PH (60%) and 11 IPAH (32%) patients died. For SLE–PH, the 3-year survival rate was 44.9% and the 5-year survival rate was 16.8%. For IPAH, the 3-year survival rate was 73.4% and the 5-year survival rate was 68.2% (p=0.02). There were no other significant differences in clinical characteristics and laboratory data between the two groups. In contrast to previous reports that the prognosis of patients with pulmonary arterial hypertension associated with collagen vascular disease was better than that of patients with IPAH, we found that the prognosis of patients with SLE–PH was much worse than that of patients with IPAH.     

The clinical characteristics and therapy response of patients with acquired pure red cell aplasia

ABSTRACT

Objective: To summarize the clinical characteristics of acquired pure red cell aplasia (PRCA) patients diagnosed in our hospital in the last 10 years.

Method: The clinical features, immune state and treatment response of acquired PRCA patients diagnosed in our hospital from January 2007 to January 2017 were retrospectively analyzed.

Results: The results showed that thymoma (13.21%) and parvovirus B19 (11.32%) were the most common causes for secondary PRCA. Ferritin (Fer) levels and erythropoietin (EPO) levels were increased in PRCA patients. The total CR and PR rate of immunosuppressive therapy in our studies was 68.29% and 12.20%, respectively. Patients with EPO level >400 U/L and Fer level >200?ng/ml had significantly lower CR rate than others. The patients with EPO level >400 U/L also had longer hemoglobin recovery time than patients with EPO level ≤400 U/L. Patients treated with corticosteroids (CS)?+?cyclosporine A (CsA) had lower relapse rate compared to the CS group (29.17% vs. 80.00%, P?<?.05).

Conclusion: Our data showed that patients with PRCA had high EPO and Fer levels. Thymoma and viral infections are the most common causes for secondary PRCA. The CS+ CsA group had lower relapse rate than CS group although response rate was similar. Increased EPO and Fer levels might be the negative factors for prognosis of acquired PRCA.     

A case of systemic lupus erythematosus complicated with pneumatosis cystoides intestinalis

Abstract

Pneumatosis cystoides intestinalis (PCI), which is characterized by the presence of multiple gas-filled mucosal, submucosal, or subserosal cysts located throughout the colon and/or small intestine, is an unusual complication of systemic lupus erythematosus (SLE). We report a case of a 33-year-old woman with a 5-year history of SLE with PCI. Her symptoms improved with conservative management. Although PCI is a rare manifestation of SLE, clinicians should be alert to the differential diagnosis of this complication.     

A case of very-late-onset systemic lupus erythematosus

Abstract

A 93-year-old woman was admitted to our hospital because of fever. Radiographic findings revealed accumulation of pleural fluid. Moreover, blood tests revealed inflammation, lymphopenia, hypocomplementemia, positive for anti-nuclear antibody, and elevated anti-DNA antibody level. Therefore, the patient was diagnosed with pleuritis associated with systemic lupus erythematosus (SLE). Administration of prednisolone 20?mg/day resulted in a marked improvement in fever, pleuritis, and laboratory findings. We report a case of very-late-onset SLE that occurred at the age of 93.     

A case of systemic lupus erythematosus complicated with pneumatosis cystoides intestinalis

Pneumatosis cystoides intestinalis (PCI), which is characterized by the presence of multiple gas-filled mucosal, submucosal, or subserosal cysts located throughout the colon and/or small intestine, is an unusual complication of systemic lupus erythematosus (SLE). We report a case of a 33-year-old woman with a 5-year history of SLE with PCI. Her symptoms improved with conservative management. Although PCI is a rare manifestation of SLE, clinicians should be alert to the differential diagnosis of this complication.     

A case of very-late-onset systemic lupus erythematosus

A 93-year-old woman was admitted to our hospital because of fever. Radiographic findings revealed accumulation of pleural fluid. Moreover, blood tests revealed inflammation, lymphopenia, hypocomplementemia, positive for anti-nuclear antibody, and elevated anti-DNA antibody level. Therefore, the patient was diagnosed with pleuritis associated with systemic lupus erythematosus (SLE). Administration of prednisolone 20 mg/day resulted in a marked improvement in fever, pleuritis, and laboratory findings. We report a case of very-late-onset SLE that occurred at the age of 93.     

A case of pure red cell aplasia complicated by Evans syndrome

A 33-year-old woman complaining of severe anemia was admitted to our hospital for polyclonal hyperglobulinemia. She was diagnosed with pure red cell aplasia (PRCA) associated with Evans syndrome. Initially, the presence of human parvovirus B19 (HPV B19) IgM appeared to indicate that the cause of PRCA was HPV B19 infection. Evans syndrome improved with steroid therapy, but PRCA was refractory. Cyclosporine was administered; consequently, the patient markedly recovered from PRCA and was discharged. PRCA complicated by Evans syndrome occurred during the course of polyclonal hyperglobulinemia. The most direct etiology for the onset of PRCA was unclear; however, immunological disorders such as polyclonal hyperglobulinemia, in addition to HPV B19 infection, may have been partly responsible for the etiology of PRCA.     

A case of systemic lupus erythematosus associated with trigger wrist

A 54-year-old woman complained of a painful, swollen, and clicking left wrist for 1 year. She had an 8-year history of systemic lupus erythematosus (SLE), treated with oral prednisolone. Flexion and extension of all fingers were difficult to initiate on wrist extension, and movement was accompanied by a palpable click at the wrist. Magnetic resonance imaging (MRI) and tenography revealed the expanded sheath of the flexor tendons and well-defined round free bodies known as rice bodies. Synovectomy and excision of rice bodies resulted in complete disappearance of swelling and triggering of the wrist. Received: August 23, 2000 / Accepted: July 6, 2001     

A sustained response to low dose interferon-alpha in a case of refractory pure red cell aplasia

Acquired pure red cell aplasia (PRCA) may be the result of a cellular or humoral autoimmune process. One proposed mechanism is the destruction of erythroid progenitors by self-reactive, cytotoxic T cells or natural killer (NK) cells. These cells normally express MHC class I receptors (KIR) which inhibit cytotoxicity when the target cell expresses the HLA class I antigen(s) they bind. Therefore, loss of these antigens on maturing erythroid progenitors may render them susceptible to destruction by the pathogenic cells. Interferon-alpha (INF-alpha) increases HLA class I expression on hematopoietic precursor cells. Therefore, we initiated a trial of INF-alpha in a patient with refractory PRCA. Following treatment, he developed transfusion independence, and a sustained normal hematocrit. Analysis of bone marrow erythroid cells revealed an increase in expression of HLA class I molecules. INF-alpha should be used in a controlled trial in patients with PRCA to determine its activity and mechanism of action.     

A case of idiopathic portal hypertension associated with rheumatoid arthritis

A 53-year-old woman who had been diagnosed with rheumatoid arthritis was found to have thrombocytopenia, splenomegaly, and gastric varices. She was diagnosed as having idiopathic portal hypertension on the basis of liver biopsy and angiography. Treatment with prednisolone was not sufficiently effective for thrombocytopenia. After transabdominal devascularization with splenectomy, thrombocytopenia subsided and gastric varices disappeared. In this case, the autoimmune mechanism as well as hypersplenism was suspected of being involved in the mechanism of thrombocytopenia.     

A case of idiopathic portal hypertension associated with rheumatoid arthritis

Abstract

A 53-year-old woman who had been diagnosed with rheumatoid arthritis was found to have thrombocytopenia, splenomegaly, and gastric varices. She was diagnosed as having idiopathic portal hypertension on the basis of liver biopsy and angiography. Treatment with prednisolone was not sufficiently effective for thrombocytopenia. After transabdominal devascularization with splenectomy, thrombocytopenia subsided and gastric varices disappeared. In this case, the autoimmune mechanism as well as hypersplenism was suspected of being involved in the mechanism of thrombocytopenia.     

A case of systemic lupus erythematosus presenting transverse myelitis after an episode of meningitis

A 27-year-old woman suffering from systemic lupus erythematosus was admitted because she had motor and sensory palsy of the lower extremities, neck stiffness, and a fever. Cerebrospinal fluid study indicated meningitis, and magnetic resonance imaging revealed cord swelling and high signals at Th9–Th12 levels. Antibiotics treatment led to resolution of the meningeal signs. Intravenous cyclophosphamide and prednisolone resulted in a partial recovery from the transverse myelitis neurological disturbance.     

A case of idiopathic portal hypertension after renal transplantation

A case of idiopathic portal hypertension (IPH) developing after renal transplantation is reported. A 33-year-old Japanese male who had undergone renal transplantation 8 years previously was transferred to our hospital because of hematemesis from ruptured esophageal varices. He had no history of any liver disease before the renal transplantation, but had a history of receiving blood transfusion. Abdominal computed tomography (CT) and ultrasonography revealed marked splenomegaly and collateral channels, but no obliteration which might cause portal hypertension in the hepatic or portal vein. No findings suggestive of hepatitis or liver cirrhosis were found either macroscopically on laparoscopy or by liver biopsy. Light microscopic study of the liver biopsy specimen showed mild periportal fibrosis, inconspicuous portal branches in the most peripheral tracts, but no pseudolobule formation or piecemeal necrosis. However collagen deposition was found in the perisinusoidal space and partly in intercellular space on electron microscopy. We consider that the development of portal hypertension in this case is responsible for the collagen deposition, which may be related to the administration of azathioprine after renal transplantation. There are few reports on IPH after renal transplantation, and it is stressed that a lower amount of azathioprine than previously reported may induce IPH under such conditions.