Successful treatment of chronic lupus myocarditis with prednisolone and mizoribine

A 36-year-old female patient who was diagnosed with chronic myocarditis as an initial manifestation of systemic lupus erythematosus (SLE) was admitted to our hospital. At her third occurrence of heart failure, we performed an endomyocardial biopsy and proved chronic myocarditis with SLE. Subsequently, she was treated with prednisolone and the immunosuppressive agent mizoribine (MZR), and her cardiac function improved. We describe for the first time treatment with MZR for chronic cardiac involvement of SLE.     

Kingella endocarditis and meningitis in a patient with SLE and associated antiphospholipid syndrome

We describe a patient with SLE and antiphospholipid syndrome who presented with severe headache and fever. Lumbar puncture analyses indicated meningitis. Kingella kingae was isolated from her blood cultures. A large mobile vegetation was seen on her mitral valve. The association between SLE, Libman-Sacks endocarditis and bacterial endocarditis is discussed.     

Intestinal Pseudo-Obstruction in Systemic Lupus Erythematosus: A Case Report and Review of the Literature

Intestinal pseudo-obstruction (IPO) is a rare but dangerous complication of systemic lupus erythematosus (SLE) when the patient has no other manifestations except gastrointestinal symptoms. We performed 1 patient with a 2-month history of recurrent vomiting and abdominal distension. She admitted past surgical histories of cesarean section and appendectomy. A physical examination revealed tenderness in the right lower abdominal on palpation and bowel sounds were weak, 2 to 3 bpm. An x-ray and CT of her abdomen showed intestinal obstruction. The initial diagnosis was adhesive intestinal obstruction. She received surgical treatment because her symptoms had gradually become more frequent and persistent. But she vomited again 2 weeks later after the surgery. Further immunology tests indicated that she had an IPO secondary to SLE. We treated the patient with methylprednisolone pulse for 3 days and followed by prednisone orally. The patient had a good response. Complete remission was achieved on 8 years follow-up.The importance of IPO secondary to SLE lies in an early diagnosis. After the diagnosis is established, immunosuppressive therapy should be the initial and first-line treatment, and surgical intervention is often disappointing and should be carefully avoided. It is necessary to enhance awareness of doctors to IPO secondary to SLE.     

Systemic lupus erythematosus and pheochromocytoma.

A 40-year-old Chinese woman with systemic lupus erythematosus (SLE) developed labile blood pressure 3 years after her onset of high blood pressure. Pheochromocytoma was subsequently found and removed. Postoperatively her hypertension improved without change in her SLE activity. Our case demonstrates pheochromocytoma can be a cause of hypertension in SLE.     

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.     

Encefalitis con estatus convulsivo localizado en una paciente con lupus eritematoso sistémico

We describe a 42-year old woman with inactive systemic lupus erythematosus (SLE) at the time of her visit. She presented with an atypical movement disorder and partial continuous epilepsy. A brain biopsy excluded cerebral vasculitis and tumoral processes. We discuss the differential diagnosis of status epilepticus in a patient with systemic lupus erythematosus.     

Intra-vascular ultrasound findings of diffuse coronary atherosclerotic change in systemic lupus erythematosus with secondary antiphospholipid syndrome.

Systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS) are autoimmune inflammatory diseases associated with juvenile atherosclerosis and thrombosis, respectively. A 44-year-old woman who had SLE with secondary APS had been treated with corticosteroid therapy, however, her inflammatory marker had never been within a normal range in her clinical course, and finally acute myocardial infarction was developed. Intra-vascular ultrasound also revealed diffuse coronary atherosclerosis progression for her age, which might result from SLE and APS, including vascular inflammation.     

Possible coexistence of SLE and sarcoidosis in a Chinese female patient

Systemic lupus erythematosus (SLE) and sarcoidosis, both being multisystem disorders, share some common clinical features. However, while SLE is not an uncommon disease in the Chinese population, sarcoidosis is distinctly rare in the Chinese. We report a Chinese female patient whose presenting features tend to suggest a lupus‐like illness. However, eventually, the diagnosis of sarcoidosis was documented, based on the histological findings of her lymph node biopsy. The possible coexistence of SLE and sarcoidosis is discussed.     

Sarcoidosis: atypical presentation associated with features of systemic lupus erythematosus

A 52-year-old woman presented with rash, bilateral uveitis and polyarthritis of her hands. Laboratory tests were consistent with the diagnosis of systemic lupus erythematosus (SLE). Ten years later she developed dyspnea associated with bihilar lymphadenopathy. Lung function tests showed a restrictive pattern and bronchial biopsy revealed the presence of noncaseating granulomata. We report this rare concomitant appearance of SLE and sarcoidosis.     

Primary malignant lymphoma of the spleen in systemic lupus erythematosus

Malignant lymphoma has been infrequently reported in the course of systemic lupus erythematosus (SLE). We report a case of primary malignant lymphoma of the spleen, an unusual site of primary lymphoma, complicating the course of systemic lupus erythematosus (SLE). Our patient had been treated with low dose prednisone and hydroxychloroquine only, and her lupus was quiescent at the time of presentation with the lymphoma. Thus primary malignant lymphoma should be considered in the differential diagnosis of splenomegaly in SLE.     

Lupus erythematosus cell phenomenon in synovial and peritoneal fluids in systemic lupus erythematosus: smoking guns,crime scenes and a twist

The presence of the lupus erythematosus (LE) phenomenon has been generally conceptualized as an in vitro occurrence where numerous damaged cells are present and substantial nucleo‐phagocytosis has occurred. In systemic lupus erythematosus (SLE), the positive LE cell phenomenon has been shown to indicate active disease with major organ involvement which potentially warrants prompt and heavy immunosuppressive therapy. We report a 36‐year‐old woman with a known history of SLE who presented with fever, left knee effusion, polyserositis, pancytopenia, low complement and high anti‐dsDNA antibody levels whose immunosuppressive treatment was escalated in view of the clinically and serologically active SLE, accompanied by the presence of LE cells in her inflammatory yet sterile left knee synovial fluid. Within 3 days of immunosuppressant escalation, her ascites worsened. While microscopic examination of the ascitic fluid also revealed LE cells, culture of the ascitic fluid later grew Candida parapsilosis. The patient subsequently responded to the addition of anti‐fungal therapy into her augmented immunosuppressive regime. Coexistence of the LE cell phenomenon and infection in SLE patients has hitherto not been described. This case illustrates that infection remains to be meticulously excluded despite the presence of the LE phenomenon in the context of clinically and serologically active SLE.     

Pulmonary hypertension secondary to systemic lupus erythematosus: prolonged survival following treatment with intermittent low dose iloprost.

Pulmonary hypertension (PHT) associated with systemic lupus erythematosus (SLE) has a dismal prognosis. Vasodilators and immunosuppressive therapy have been tried over the years with discouraging results. Prostacyclin (PGI2) which has potent vasodilatatory and anti-platelet effects has been demonstrated to significantly decrease pulmonary arterial pressure and pulmonary vascular resistance during acute infusion. Satisfactory response has been reported in SLE patients with PHT treated with short-term intravenous continuous PGI2 infusion. We report here a 48-month experience of the use of monthly low dose infusion of a PGI2 analogue, iloprost, in a SLE patient with pulmonary hypertension in New York Heart Association functional Class III. There was an initial haemodynamic response to an acute infusion of iloprost. Repeated infusions were followed by marked improvement in her functional status and her mean pulmonary arterial pressure dropped from 80 mmHg in the first few months and remained static at around 55 mmHg for the subsequent years.     

Preeclampsia, dilated cardiomyopathy and renal failure as the first manifestation of systemic lupus erythematosus: a case report

We report the case of a 26-year-old woman with severe renal and congestive heart failure as a primary manifestation of systemic lupus erythematosus after her premature terminated pregnancy for the symptoms of preeclampsia with HELLP syndrome. Preeclampsia, due to the similarity with SLE in many signs and symptoms, delayed the diagnosis. The importance of the renal biopsy that helped us to make a differential diagnosis in a patient with an unclear proteinuria persisting postpartum is obvious. We suggest that a diagnostic algorithm of patients suffering from preeclampsia should exclude SLE since only an early and adequate treatment can prevent irreversible organ impairment.     

Pneumatosis cystoides intestinalis following lupus enteritis and peritonitis

We describe a case of systemic lupus erythematosus (SLE) with enteritis and peritonitis who later developed pneumatosis cystoides intestinalis (PCI). A 35-year-old woman with SLE relapsed with enteritis and peritonitis. Prednisolone (PSL) effectively improved her symptoms. However, 6 weeks later, she developed PCI. Tapering of PSL, administration of intravenous cyclophosphamide, prokinetic agents and antibiotics, bowel rest with intravenous hypernutrition therapy and hyperbaric oxygen therapy successfully improved PCI. Although PCI is a rare complication of SLE, the present case suggests that lupus enteritis could be a risk factor for PCI, and that high-dose PSL could cause additional insult to PCI.     

Relapse of systemic lupus erythematosus in a patient receiving cyclosporine A

Cyclosporine A (CyA) is being increasingly used as an immunosuppressive agent to treat various autoimmune diseases. A patient with systemic lupus erythematosus (SLE) receiving CyA after renal transplantation suffered a disease relapse associated with high serum CyA levels. Lowering of the CyA dose and the start of steroids resulted in a rapid improvement in her condition. Trials evaluating CyA for SLE should proceed with caution since under certain circumstances this drug may exacerbate disease activity.     

Valvular deposition of antiphospholipid antibodies in the antiphospholipid syndrome: a clue to the origin of the disease

In this report we present an unusual case of a 45-year-old female patient with systemic lupus erythematosus (SLE) who was hospitalized for mitral valve replacement. In her childhood she presented with mitral stenosis and chorea on which grounds a preliminary diagnosis of rheumatic fever was established. After a quiescent period lasting two decades her disease erupted with mitral stenosis, thromboembolic phenomena, and nephritis. Due to severe malfunctioning of her mitral valve, the patient eventually underwent mitral valve replacement. The antibodies involved in the pathogenesis of our patient's valvular disease were studied by immunohistochemical analysis, applying rabbit polyclonal anti-human IgG and IgM anti-human C3c and anti-idiotypes to a mouse monoclonal naturally occurring polyspecific human monoclonal anti-cardiolipin antibody termed S2.9, and to the 16/6 Id which defines a common Id on anti-DNA antibodies in patients with SLE. Immunoperoxidase staining using an anti-idiotype mAb to anti-cardiolipin antibodies demonstrated the deposition of these anti-bodies in the subendothelial layer of the valve. We believe that anti-phospholipid syndrome (APS) with SLE was the initial and primary disease in this patient. These findings clearly indicate that APS must be considered in the differential diagnosis of rheumatic fever, particularly in young female patients who present with mitral stenosis and chorea.     

Therapeutic Plasma Exchange Improved Pregnancy-associated Thrombotic Microangiopathy but not the Pregnancy Outcome in Patient with Systemic Lupus Erythematosus

Thrombotic microangiopathy (TMA) is a rare but life-threatening complication of systemic lupus erythematosus (SLE) and is associated with adverse pregnancy outcomes. We herein report a 30-year-old pregnant woman with SLE complicated by TMA. Because her condition was unresponsive to initial corticosteroid and fresh-frozen plasma infusion treatment, we attempted plasma exchange (PE). Although thrombocytopenia and microangiopathic hemolytic anemia gradually improved, fetal death was confirmed at 23 weeks of gestation. This case suggests that PE is an effective therapeutic option but might be insufficient to maintain pregnancy in patients with SLE complicated by TMA.     

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.     

Surgical management of calcinosis cutis universalis in systemic lupus erythematosus

Calcinosis cutis is common in several connective tissue diseases but rare in systemic lupus erythematosus (SLE). A 43-year-old woman with a 12-year history of SLE who presented with calcinosis cutis at the time of SLE diagnosis developed a large, ulcerated, draining mass on her left hip. The lesion was excised and skin was grafted, with an excellent early result. The clinical variants and mechanisms of ectopic calcification are discussed, as well as the proposed medical therapies for calcinosis cutis.     

Danazol therapy in autoimmune hemolytic anemia associated with systemic lupus erythematosus

We describe a patient with systemic lupus erythematosus (SLE) complicated by severe autoimmune hemolytic anemia. Therapy with corticosteroids, splenectomy, azathioprine, chlorambucil, and intravenous immunoglobulin was unsuccessful. She responded to danazol, however, with maintenance of her hemoglobin and reductions in her transfusion and corticosteroid requirement. Thus, danazol may represent an important therapeutic option in the treatment of autoimmune hemolytic anemia.