Asymptomatic pneumatosis cystoides intestinalis in a patient with systemic lupus erythematosus]

Pneumatosis cystoides intestinalis (PCI) is a rare condition characterized by the presence of gas-filled cysts in the submucosa or subserosa of gastrointestinal tract. PCI has been widely recognized as a late manifestation of systemic sclerosis but seldom reported to take place in patients with systemic lupus erythematosus (SLE). We reported here a 13-year-old female who had been diagnosed to have SLE based on the following findings; malar rash, discoid erythema, proteinuria, positive antinuclear antibody and anti-DNA antibody. She had been treated with various immunosuppressive drugs including pulse use of corticosteroid, cyclophosphamide and cyclosporin A. She was referred to our hospital because of proteinuria and numbness on her right fifth toe, refractory to above treatment. On admission, the activity of her disease was already low and she had no abdominal symptoms. Plain X-ray film showed multiple round translucencies along the wall of the ascending and transverse colon. Colonoscopy revealed multiple firm-walled cysts distributing in the terminal ileum as well. A diagnosis of PCI was made and she was successfully treated with oral antibiotics and laxatives. The association of PCI with SLE is reviewed briefly.     

A case of a flare-up of systemic lupus erythematosus and Sjögren’s syndrome manifesting with cholecystitis secondary to pregnancy

a 30-year- old had a 2 year history of systemic lupus erythematosus (SLE) was transferred to our hospital by her gynecologist because of a low-grade fever and dyspnea after delivery. Since a blood culture performed upon admission showed the presence ofBacteroides caccae, she was diagnosed with sepsis. Antibiotic therapy did not resolve her symptoms and she further developed an acute abdomen with marked cholecystitis. This was believed to be due to a flare-up of SLE after the pregnancy, overlapped with Sjögren’s syndrome which was evidenced by sialaden biopsy. She showed a good response to combination therapy with steroids and antibiotics.     

Case report of systemic lupus erythematosus patient with hemophagocytic syndrome, treated with plasma exchange, with specific reference to clinical profile and serum cytokine levels]

A 42-year-old woman was diagnosed as systemic lupus erythematosus (SLE), because of the findings of polyarthritis, leukopenia, positive antinuclear antibody, and positive anti DNA antibody. She was treated with predonisolone (PSL) at 10 mg per day. She was admitted to our hospital on October 2000 because of spiking high fever, skin eruption, and lymph node swelling. Since her illness of SLE was considered to be worsening, high dose of corticosteroids were given. However, high fever persisted and liver dysfunction was developed with increased serum ferritin. Her bone marrow smear showed hemophagocytosis. We made a diagnosis of hemophagocytic syndrome (HPS) complicated by disseminated intravascular coagulation (DIC). HPS was thought to be induced by viral infection, even though causative viral infection was not detected. Her general condition worsened with persistent high fever and liver dysfunction. Plasma exchange was carried for two consecutive days, followed by cyclosporine A and lipo-dexamethasone, which improved her fever rapidly. Her general condition gradually improved. Serum levels of ferritin, soluble interleukin 2 receptor (sIL 2-R), interferon-gamma and interleukin 6 decreased associated with improvements of her clinical condition. We thought plasma exchange could be effective to decrease serum levels of cytokine, which was suggested to be the pathogenic to HPS. However serum levels of IFN-gamma and IL 6 after plasma exchange did not change in this case. Further studies are required to confirm the effects of plasma exchange for HPS.     

A case of a flare-up of systemic lupus erythematosus and Sjögren’s syndrome manifesting with cholecystitis secondary to pregnancy

Abstract

a 30-year- old had a 2 year history of systemic lupus erythematosus (SLE) was transferred to our hospital by her gynecologist because of a low-grade fever and dyspnea after delivery. Since a blood culture performed upon admission showed the presence of Bacteroides caccae, she was diagnosed with sepsis. Antibiotic therapy did not resolve her symptoms and she further developed an acute abdomen with marked cholecystitis. This was believed to be due to a flare-up of SLE after the pregnancy, overlapped with Sjögren’s syndrome which was evidenced by sialaden biopsy. She showed a good response to combination therapy with steroids and antibiotics.     

Enteropathy-type T-cell lymphoma that was pathologically diagnosed as celiac disease

A 63-year-old woman was referred and admitted to our department for further examination of protein-losing enteropathy (PLE), which was diagnosed by alpha-anti trypsin test. Her symptoms were frequent vomiting, watery diarrhea and hypoproteinemia. Although intensive examination for PLE was performed in her previous hospital, the origin of the disease was not detected. Abdominal computed tomography revealed diffuse enlargement and swelling of the intestine wall and a 5-cm diameter mass with unclear margin, which involved the mesenteric arteries and veins. Total colonoscopy showed a diffuse edematous lesion with hemorrhage at the terminal ileum. Enteropathy-type T-cell lymphoma (ETL) was diagnosed based on a biopsy of the lesion and CD45 gating analysis. Immediate start of chemotherapy (CHOP) led to a transient regression of the tumor even though her symptoms were not improved. During the second cycle of CHOP, the patient died of massive hemorrhage throughout the intestine. The pathological study revealed that intraepithelial CD3-positive clonal T-cells were present in the lesion, indicating that this case could be associated with celiac disease. In light of the histological findings, we concluded that this was a case of ETL associated with celiac disease, which is extremely rare in Japan.     

Iron deficiency anemia and hypoproteinemia induced by fresh cow's milk]

We reported two patients who developed severe iron deficiency anemia and hypoproteinemia which were induced by excessive drinking cow's milk. Case 1, a 23-month-old girl, had started drinking 800-1,000ml of cow's milk everyday since the age of 9 months. She developed generalized edema at the age of 23 months and visited Saga Medical School for Treatment. Case 2, a 14-month-old girl began to drink cow's milk at the age of 6 months. She drank 1,500ml cow's milk every day at the age of 14 months. She was, referred to our hospital by her physician because of severe anemia. Hypoproteinemia and severe iron deficiency anemia in both of these patients were improved by oral iron intake and prohibiting of cow's milk. Although many children at their age usually drink more than 500ml cow's milk every day, manifestations of symptoms such as iron deficiency anemia and hypoproteinemia do not occur frequently. Thus some investigations suggest that genetic defects as well as other factors related to this disease may be important factors.     

A case of systemic lupus erythematosus associated with severe acute pancreatitis]

A 22-year-old woman began to have the symptoms of anorexia, high fever, cough and general fatigue from June of 1997. She was admitted in our hospital on Aug. 8th, 1997 for the further detail examination because of pancytopenia and positive antinuclear antibody (ANA). Her laboratory findings and clinical symptoms were compatible with systemic lupus erythematosus (SLE) such as leukopenia, proteinuria, hypocomplementemia, positive ANA, elevated titer of autoantibodies including anti-DNA, anti-Sm, anti-RNP antibodies, polyarthralgia and photosensitivity. The administration of oral prednisolone (40 mg/day) was started on Aug. 15th, 1997 under the diagnosis of SLE. However, she had severe abdominal pain in epigastrium with elevated serum amylase, ascites and dull shape of pancreas tail by CT scan compatible with acute pancreatitis. On Aug. 18th, her general condition was worsening with fever, epigastralgia, abdominal distension, anemia, weak palpation of radial artery, hypotension, tachycardia, shallow breathing and cold sensation on both extremities as shock. In spite of steroid pulse therapy with nafamostat mesilate intraarterial infusion, her condition was not improved. The dose of 50 mg/day of cyclophosphamide was added to the regimen on Aug. 22nd. Then, gradually her condition started to be restored. Anemia, leukopenia, hypocomplementemia continued. Second steroid pulse therapy was done on Sep. 5th. After then, she became better in her clinical symptoms and laboratory data. The dose of PSL was tapered to 15 mg/day and 7.5 mg/day update of Oct. 1998 without the pseudcysts found after pancreatitis. She is a rare case who recovered from severe acute pancreatitis due to SLE itself.     

Systemic lupus erythematosus, complicated with refractory skin ulcers, treated successfully with bosentan

A 20-year-old woman was admitted to our hospital because of bilateral pretibial edema. Administration of prednisolone was started after she was diagnosed with systemic lupus erythematosus (SLE). However, skin ulcers on her extremities developed; they subsequently worsened with tapering of prednisolone. She also developed pulmonary hypertension (PH). Her skin ulcers improved considerably after administration of bosentan, an endothelin receptor antagonist. Bosentan may be efficacious not only for PH but also for refractory skin ulcers.     

A case of pseudohypoparathyroidism type 1 with systemic lupus erythematosus

A case of pseudohypoparathyroidism (PHP) type 1 with systemic lupus erythematosus (SLE) is reported. A 36-year-old women was admitted to our hospital with the complaints of dyspnea, arthralgia, tetany and tremor. Laboratory findings on admission showed; leukopenia, thrombocytopenia, hypocalcemia, positive antinuclear, anti-RNP, anti-Sm antibodies. A diagnosis of PHP type 1 was made from the findings of Albright's osteodystrophy and Ellsworth-Howard test. On the basis of various auto antibodies and clinical findings, the patient was diagnosed as SLE, too. She was started on a therapy of prednisolone at a dose of 40 mg per day. Her clinical manifestations immediately became better, and her laboratory findings subsequently improved. Up to the present, the case report of overlapping syndrome of PHP type 1 and SLE is very rare. Both PHP and SLE were considered to be in a category of autoimmune disease and the relationship between PHP and SLE was discussed.     

Systemic lupus erythematosus,complicated with refractory skin ulcers,treated successfully with bosentan

Abstract

A 20-year-old woman was admitted to our hospital because of bilateral pretibial edema. Administration of prednisolone was started after she was diagnosed with systemic lupus erythematosus (SLE). However, skin ulcers on her extremities developed; they subsequently worsened with tapering of prednisolone. She also developed pulmonary hypertension (PH). Her skin ulcers improved considerably after administration of bosentan, an endothelin receptor antagonist. Bosentan may be efficacious not only for PH but also for refractory skin ulcers.     

A case of systemic lupus erythematosus with interstitial myocarditis leading to sudden death]

A 29-year-old female with systemic lupus erythematosus (SLE) was admitted because of exertional dyspnea in January, 1996. The diagnosis of SLE was made on the basis of malar rash, discoid rash, polyarthritis, leukopenia, positive antinuclear antibody and focal glomerulonephritis in 1991. She did not have any cardiac symptoms in 1994, when the electrocardiogram (ECG) abnormalities, such as poor R wave progression and right axis deviation, were present. On admission, she developed congestive heart failure without any signs of active SLE. Laboratory findings were unremarkable. There were new ECG abnormalities, such as left atrial overload and low voltage in limb leads. Chest roentgenogram showed mild pulmonary congestion and marked cardiomegaly. Echocardiography showed enlargement and diffuse hypokinesis of the left ventricle. Cardiac catheterization confirmed that the coronary arteries were normal and that the left ventricular function was poor (ejection fraction, 21%). Myocardial biopsy obtained from left ventricle revealed interstitial fibrosis. After furosemide, digoxin and captril were administered with predonisolone (PSL), her symptoms gradually improved. Since the Holter monitoring showed nonsustained ventricular tachycardia, the doses of PSL and mexiletine were increased up to 20 mg and 300 mg daily, respectively. Unexpectedly, she was found dead in her hospital room in May, 1996. The postmortem findings of the heart revealed mild infiltration of inflammatory cells, predominantly lymphocytes, and plasma cells, and interstitial fibrosis, which were consistent with interstitial myocarditis. In this case ECG abnormalities preceded cardiac symptoms, which may suggest that myocarditis subclinically developed. Serum creatinine kinase levels had not been elevated throughout the entire course. While several cases of acute myocarditis associated with a flare of SLE have been reported, there were few cases regarding interstitial myocarditis that chronically progress and can be fatal. This case is thought to be suggestive of elucidating the pathogenesis of lupus myocarditis.     

Autoimmune pancreatitis as the initial presentation of systemic lupus erythematosus

Pancreatitis is a relatively rare complication in systemic lupus erythematosus (SLE). Here we present a case of SLE associated with autoimmune pancreatitis. A 37-year old woman was admitted to our hospital because of fever, skin rash, proteinuria and abdominal pain. A diagnosis of SLE was made based on her clinical, laboratory and renal histological findings showing diffuse proliferative lupus nephritis. Elevated serum amylase, typical radiographic findings and selective increase in serum IgG4 all suggested that the patient also had autoimmune pancreatitis. Systemic administration of glucocorticoid successfully induced remission of pancreatitis and nephritis along with the reduction of IgG4. Autoimmune pancreatitis is a newly recognized type of pancreatitis, in which IgG4 immune response is thought to participate pathophysiologically. Although the disease has been observed to develop in patients having various connective tissue diseases, our report is the first to describe IgG4-related autoimmune pancreatitis in a patient with SLE.     

Protein-losing Enteropathy Complicated with Primary Intestinal Follicular Lymphoma

Protein-losing enteropathy (PLE) is a rare syndrome characterized by hypoproteinemia due to gastrointestinal (GI) protein loss. Primary intestinal follicular lymphoma (PIFL), a specific variant of follicular lymphoma with essential only GI involvement, has not been reported as an etiology of PLE. We herein report a case of PLE complicated with PIFL that was successfully treated with rituximab, resulting in rapid improvement of PLE and a complete response of PIFL. Macroscopic findings of ulcerative lesions with diffuse involvement, which were precisely described by capsule and double-balloon enteroscopy at the diagnosis, also improved following the treatment. This case provides a clue suggesting factors that promote PLE in PIFL.     

Wegener's granulomatosis complicated by intestinal ulcer due to cytomegalovirus infection and by thrombotic thrombocytopenic purpura

A 61-year-old woman was admitted to our hospital because of acute kidney injury. She complained of general fatigue, appetite loss, and a high fever. Nodular lesions were observed on chest X-rays and there were >100 erythrocytes per high power field in her urinary sediment. A renal biopsy revealed necrotizing granulomatous glomerulonephritis, and her serum proteinase 3-antineutrophil cytoplasmic antibody (PR3-ANCA) titer was elevated (55 EU). Based on these findings we made a diagnosis of Wegener's granulomatosis (WG). Hemodialysis was started immediately after admission. Steroid therapy was administered and her symptoms were relieved, but her renal function did not improve. On the 50th hospital day her condition suddenly became complicated by hemoperitoneum and massive intestinal bleeding, and the descending, transverse, ascending colon and part of the ileum were surgically resected. The cytomegalovirus (CMV) antigen titer was elevated, and histologic examination of the bowel specimen showed positive staining for CMV in the ulcer lesion, suggesting that CMV infection had caused the bowel hemorrhage. After treatment with ganciclovir, the bleeding was resolved and the CMV antigens became negative. We considered that this patient was further complicated by thrombotic thrombocytopenic purpura (TTP) because of thrombocytopenia, hemolytic anemia and neurologic symptoms. She was treated by plasma exchange. We report here a case of WG complicated by acute intestinal ulcer due to CMV infection and by TTP.     

A unique case of collagenous colitis presenting as protein-losing enteropathy successfully treated with prednisolone

A 76-year-old woman with a 5-mo history of recurrent diarrhea and generalized edema was admitted to our hospital. Colonoscopy revealed edematous mucosa, and histopathological examination was compatible with collagenous colitis. Protein leakage from the colon, particularly in the ascending portion, was identified on 99mTc-human serum albumin scintigraphy. Collagenous colitis associated with protein-losing enteropathy （PLE） without small bowel disease was diagnosed. Prednisolone treatment ameliorated diarrhea and hypoproteinemia. Collagenous colitis should be included in the differential diagnosis of chronic diarrhea with hypoproteinemia for appropriate management.     

A patient with probable systemic lupus erythematosus, lupus anticoagulant and myocardial infarction

The case history of a 31-year-old woman is described. She had a history of thrombosis; in the past there had been an arterial embolus of the left superficial femoral artery and venous thrombosis of the right leg. The patient was admitted to hospital because of fever of unknown origin. During the hospital stay the diagnosis of probable SLE was made. She died of myocardial infarction. At autopsy, thrombosis of the small arterioles of the heart was found without sclerosis of the coronary arteries. A lupus anticoagulant could be demonstrated in her blood and seems to have been the cause of this rare complication. Treatment with anticoagulants is advised for patients with LAC and a history of thrombosis.     

An elderly case of systemic lupus erythematosus associated with herpes zoster, anemia, and hemiparesis]

An elderly case of systemic lupus erythematosus (SLE) with suspected hemolytic anemia was experienced. A 70 year-old female was admitted to our hospital on December 31 with complaints of herpetic eruption. She complained of arthralgia since 3 month prior to her admission. The positive findings on examination were skin eruption in the left chest, a systolic heart murmur and a palpable elastic hard liver. Laboratory data showed raised erythrocyte sedimentation rate of 149 mm per hour, decreased Hb (10.1 g/dl), decreased hematocrit (30.0%), increased reticulocytes (33%1000), decreased thrombocytes (73,000/mm3), increased gamma-globulin (33%) and positive rheumatoid factor. During admission, she developed anemia. A stool test for occult blood was negative. The haptoglobin was 38.8 mg/dl and bone marrow aspiration showed increased erythropoiesis, suggesting features of immune hemolytic anemia, except she was negative on Coomb'test. Eye fundi were similar to case of typical bleeding observed in SLE. Concerning immunological findings, the antinuclear factor was x 1280 and the anti-dsDNA antibody was x 80, on which a diagnosis of SLE was based. She experienced numbness of the left arm and developed left hemiparesis 2 days later. Therapy with 15 mg/day prednisone obtained a good response and anemia, abnormal immunological findings and hemiparesis disappeared.     

A case report of nephrotic syndrome due to collapsing focal segmental glomerulosclerosis treated with low-density lipoprotein apheresis

We herein report the case of a 73-year-old woman with steroid and cyclosporine resistant collapsing focal segmental glomerulosclerosis (FSGS) whose refractory proteinuria and hypoproteinemia were controlled with low-density lipoprotein apheresis (LDL-A). She was initially treated with steroid therapy, including methylprednisolone pulse and cyclosporine therapy. However, her hypoproteinemia, accompanied with renal insufficiency, persisted despite these therapies. We treated her using LDL-A and found improvement in her urine protein excretion, hyperlipidemia, hypoproteinemia, and renal function as a result of this treatment. This suggests that LDL-A may therefore be an effective therapy for nephrotic syndrome due to collapsing FSGS.     

Atypical Presentation of Crohn's Disease: Severe, Recurrent Iron Deficiency Anemia Dependent on Blood Transfusions

We report an unusual case of Crohn's disease. Our subject, a 43-yr-old lady, presented with severe iron dericiency anemia, hypoproteinemia and lymphocytopenia, but without any clinical manifestations of Crohn's disease either at presentation or during follow-up. She had recurrent episodes of severe iron deficiency anemia, repeatedly requiring blood transfusions (28 units in 20 months). She was found to have a short stricture with ulceration in tbe ileum due to Crobn's disease, and a blind loop with bacterial overgrowth above it. Resection of the affected area prevented further recurrence of anemia and corrected hypoproteinemia. We postulate that her anemia and hypoproteinemia were due to blood and protein (lymph) loss from the ulcerated area in the ileum. Sucb a presentation bas not been described before.     

A case of systemic lupus erythematosus complicated by Nocardia farcinica]

We report a patient with systemic lupus erythematosus (SLE) complicated with nocardiosis. This case is very important that the complication of nocardiosis in SLE is very rare and the treatment to both SLE and nocardiosis is very difficult. A twenty-one-year old female was admitted to our hospital because of thoracic empyema and active lupus nephritis. Her medical history revealed that the diagnose of SLE was made when she was 18 with lymphocytopenia, proteinuria, positive antinuclear antibodies, and high titer of antibodies to native DNA. She was treated with prednisolne 60 mg daily and became better. Proteinuria appeared again in September 1995 and she was admitted to the former hospital. Renal biopsy proved diffuse proliferative glomeluronephritis (WHO IVb). She was treated with 1 g per day of methylprednisolone for 3 days and succeeded with 60 mg day of prednisolone. In early November she developed left chest pain and fever and chest X-ray demonstrated left pleural effusion. Antibiotics, antituberculosis, and antifungal therapy failed to subside her pleuritis and it turned to empyema. Then she was transferred to our hospital for further treatment. Nocardia farcinica was detected from the aspirated pleural fluid obtained at the former hospital. Drainage and intrathoracic impenem injection were effective. While long usage of minocycline was continued for the nocardiosis, 500 mg of cyclophosphamide pulse therapy to lupus nephritis was administrated. Two weeks later a new pulmonary lesion with left chest pain and liver abscess developed. Administration of trimethoprim-sulfamethoxazole subsided the nocardiosis. She was discharged with 1 g per day of proteinuria the prescribed 13 mg per day of prednisolone and continuous TMP-SMZ intake for nocardial infection. When immunosuppressive therapy must be given to the immunocompromised host, a more potent therapy must be added to avoid infection.