A case of anti-nuclear antibody negative systemic lupus erythematosus associated with penile ulcer

Summary We report here an old male patient with anti-nuclear antibody (ANA) negative systemic lupus erythematosus (SLE) with active renal disease and penile ulcer. He revealed nephrotic syndrome, malar rash and oral ulcer. SLE was discussed, however both ANA and anti-DNA antibody were persistently negative. A penile ulcer was also observed. He died of acute respiratory distress. Autopsy findings including onion skin lesion in the spleen and haematoxylin body in the kidney resulted in the final diagnosis of SLE. To our knowledge, association of penile ulcer with SLE has not yet been reported. Therefore, the present case is thought to be extremely unusual.     

Elderly case of systemic lupus erythematosus who showed lupus peritonitis as the initial symptom]

A 64-year-old woman who admitted because of abdominal pain and ascites. She was diagnosed as having systemic lupus erythematosus (SLE) and lupus peritonitis based on anti ds-DNA antibody, ANA positive, serositis and renal dysfunction. She was successfully treated by intravenous pulse therapy with methylprednisolone, but her enteritis recurred after switching to oral administration. She recovered following pulse therapy with methylprednisolone and extracorporeal apheresis. This case was characterized by a rather old age of onset and the peculiar initial symptoms of peritonitis.     

Seronegative systemic lupus erythematosus: etiology of nephrotic syndrome and acute renal failure in early postpartum period

Systemic lupus erythematosus (SLE) is an autoimmune syndrome that occurs most commonly in women during their reproductive years. Nephritis is known to be one of the most serious complications of SLE. Lupus nephropathy is frequently associated with ANA and anti-dsDNA antibodies. Rarely, serological markers may be initially absent, and in many cases, they become positive after sometime. We present a 28-year old, otherwise healthy female who admitted to our clinic with edema, hypertension, proteinuria and acute renal failure following her fourth delivery. Serum immunological markers were negative and renal biopsy showed histopathological changes consistent with systemic lupus erythematosus as the etiology of nephrotic syndrome. A dramatic therapeutic response was achieved by pulse steroid and cyclophosphamide treatment following oral steroid therapy. In women with new onset nephrotic syndrome or renal function deterioration in postpartum period, even if the patient is asymptomatic or seronegative, it is crucial to exclude SLE for a rapid diagnosis and prompt treatment in the case of lupus nephritis. Renal biopsy is of diagnostic importance in such cases in which there is no other clinical, biochemical and serological evidence of the disease.     

ANCA-related crescentic glomerulonephritis in a patient with scleroderma without marked dermatological change and malignant hypertension

A 64-year-old woman with scleroderma without marked dermatological change developed anti-neutrophil cytoplasmic autoantibody (ANCA)-related renal failure. She had neither malignant hypertension nor elevation of plasma renin concentration. Renal biopsy showed crescentic glomerulonephritis (pauci-immune type) and the myeloperoxidase-specific anti-neutrophil cytoplasmic autoantibody (MPO-ANCA) titer was found to be elevated to 757 IU/ml. Methylprednisolone pulse therapy followed by oral prednisolone effectively suppressed renal failure and lowered the MPO-ANCA titer. We believe this is a rare case of ANCA-related renal failure in a patient with scleroderma without marked dermatological change.     

Nine-year's follow up on the appearance of autoantibodies in a child with idiopathic thrombocytopenic purpura subsequently developing lupus with central nervous system manifestations]

We describe a 23-year-old female who developed SLE 9 years after asymptomatic idiopathic thrombocytopenic purpura (ITP) with positive antinuclear antibody (ANA). Although the platelet count was normal before the onset of SLE, the titer of ANA was gradually increased and also autoantibodies, including antibodies to SS-A/Ro, single-stranded DNA (ss-DNA) and nuclear ribonucleoprotein (RNP) changed to positive. At 23 years of age, the patient was admitted to our hospital because of fever, butterfly rash and polyarthritis. Anti double-strand DNA (ds-DNA) antibody and anti Smith antigen (Sm) antibody were positive and the platelet count and titer of complements were decreased. The patient was diagnosed as SLE and treated with 60 mg/day of prednisolone. Despite steroid therapy, psychiatric symptoms appeared. Additional treatments with steroid pulse therapy and double filtration plasmaphresis resulted in the improvement of SLE including the central nervous system manifestations. This case suggested that increased titer of ANA and the appearance of antibodies to SS-A, ss-DNA, RNP, ds-DNA and Sm in ITP patients predict the development of SLE. Routine checkup of autoantibodies is needed to manage ITP with positive ANA.     

A case of ANCA-associated systemic vasculitis induced by atorvastatin

We present a 45-year-old male patient who presented to Accident and Emergency department with a 6-week history of pain and stiffness involving his bilateral legs. Both calves were markedly tender, and he was not able to bear weight. He also complained of numbness involving his left big toe for a few days, which later spread to involve his arms, and tinnitus and hearing loss in his left ear. There were no respiratory, gastrointestinal or urinary symptoms. He had a background history of hypercholesterolemia and was treated with atorvastatin 10 mg for 6 months. His initial investigations showed markedly increased inflammatory markers, and serum antineutrophil cytoplasmic antibody (ANCA) was markedly positive at a titre of 1:160 (P-ANCA). Electromyography and muscle biopsy showed myopathic features. A diagnosis of drug-induced ANCA-associated vasculitis (on the basis of mononeuritis multiplex, sensorineural hearing loss and markedly increased anti-myeloperoxidase (MPO) ANCA) and statin-induced distal myopathy was made. He was treated with three 500 mg doses of methylprednisolone, followed by slowly tapering dose of oral corticosteroids from 30 mg once daily (OD). He was also started on azathioprin (2.5 mg/kg). He had a dramatic improvement of his myalgia, hearing loss and sensory symptoms and went into complete clinical remission. His inflammatory markers rapidly returned to normal, and MPO-ANCA normalised within 3 months of starting immunosuppressive therapy and remained negative on further testing. He is currently on a tapering regimen of corticosteroids (7 mg OD), and after weaning him off corticosteroids, we plan to slowly taper his azathioprin.     

Elderly onset systemic lupus erythematosus (SLE) presenting with disseminated intravascular coagulation (DIC)

We describe an unusual case of elderly onset systemic lupus erythematosus (SLE) that presented with disseminated intravascular coagulation (DIC). An 86-year-old man who complained of general malaise was admitted for evaluation and treatment of thrombocytopenia. He was diagnosed as having SLE and DIC based on the criteria of the American College of Rheumatology for SLE (renal involvement, hematological abnormalities, and positivity for antinuclear antibody and lupus anticoagulant) and the criteria for DIC presented by the subcommittee on DIC of the ISTH (a large increase of fibrin degradation products [3 points] and a platelet count <50 × 10³/ml [2 points], resulting in a score of 5; a score ≥5 is compatible with DIC). The patient was treated with corticosteroid therapy (30 mg/day); the DIC and SLE remitted, and his renal function improved, but he developed pulmonary tuberculosis. Timely diagnosis, appropriate treatment, and an awareness of the potential for serious infections are of utmost importance when dealing with patients with elderly onset SLE.

     

A case of autoimmune pancreatitis effectively treated with an immunosuppressant (azathioprine)

The patient was a 42-year-old man who presented at our hospital with obstructive jaundice. Although antinuclear antibody test results were negative, and immunoglobulin G4 (IgG4) was not elevated, endoscopic ultrasound revealed a mixed internal hyperechoic and diffuse hypoechoic pattern, a finding consistent with autoimmune pancreatitis. Endoscopic retrograde cholangiopancreatography further revealed irregular narrowing of the main pancreatic duct and sclerosing cholangitis with distal biliary stricture. In addition, endoscopic ultrasound with fine needle aspiration cytology resulted in a diagnosis of type 1 autoimmune pancreatitis. Oral prednisolone treatment was initiated at 30 mg/day, and the dosage was gradually decreased. However, in accordance with the patient’s wishes, maintenance treatment was discontinued once dosage reached 5 mg/day. Despite this, relapse of obstructive jaundice occurred 1 month post discontinuation, and was treated with methyl-prednisolone pulse therapy (500 mg/day) followed by oral prednisolone. However, computed tomography, magnetic resonance imaging, and endoscopic ultrasound did not reveal sufficient improvement after 6 months of treatment. Therefore, an immunosuppressant (azathioprine) was introduced. Subsequent imaging analyses and endoscopic ultrasound fine needle aspiration revealed clear improvements in pathology.     

Mizoribine intermittent pulse protocol for induction therapy for systemic lupus erythematosus in children: an open-label pilot study with five newly diagnosed patients

The objective of the current work is to report our preliminary experience with the mizoribine (MZR) intermittent pulse protocol for induction therapy for newly diagnosed pediatric-onset systemic lupus erythematosus (SLE). Five consecutive patients who were newly diagnosed as having SLE with biopsy-proven lupus nephritis were recruited for an open-label trial of prednisolone (PDN) and MZR intermittent pulse therapy (10 mg/kg for 2 days of the week for 12 months). Data on the renal response and serologic lupus activity were collected prospectively. The baseline characteristics of the patients were: mean age, 11 years; urinary protein/creatinine ratio (U-prot./cre.), 0.99 ± 0.91; serum complement hemolytic activity (CH50), 10.6 ± 1.3 (normal, 23–46 U/ml); serum anti-dsDNA antibody titer, 258.6 ± 125.5 IU/ml (normal, <12.0 IU/ml); serum creatinine, 0.5 ± 0.1 mg/dl; European Consensus Lupus Activity Measurement index (ECLAM), 7.4 ± 1.1. The primary endpoint was the interval until the development of a flare of SLE. Despite gradual tapering of the PDN dose, significant improvement as compared to the baseline values was observed in all the parameters examined at 3, 6, and 12 months of treatment. After 12 months therapy, complete response was achieved in all of the patients, except for 1 patient who showed poor drug compliance. In two patients who had severe lupus nephritis at the first renal biopsy, marked histologic improvement was confirmed at the second renal biopsy. No serious adverse effects were observed. We believe that the MZR pulse protocol combined with PDN for induction therapy may be the treatment of choice in selected young patients with SLE. Further studies to confirm the long-term efficacy and safety of our current protocol in larger numbers of patients are, however, needed.     

Cytapheresis for the Treatment of Myeloperoxidase Antineutrophil Cytoplasmic Antibody‐Associated Vasculitis: Report of Five Cases

Abstract: To minimize the adverse effects of high‐dose administration of steroids and cyclophosphamide in patients with myeloperoxidase (MPO) antineutrophil cytoplasmic antibody (ANCA), granulocytapheresis (GCAP) or leukocytapheresis (LCAP) was performed to reduce inflammation. Four patients with rapidly progressive glomerulonephritis (RPGN) and one patient with pulmonary hemorrhage due to MPO‐ANCA‐associated vasculitis were treated by cytapheresis. The prednisolone (PSL) dose was 0.28 ± 0.15 mg/kg/day (mean ± SD) (range 0.18–0.50 g/kg/day). In the 4 RPGN patients, the peak serum creatinine level was 3.7 ± 1.9 mg/dl (range 1.7 to 5.6 mg/dl). GCAP was performed in 3 RPGN patients and in 1 pulmonary hemorrhage patient. LCAP was performed in 1 RPGN patient. In the 4 RPGN patients, renal function improved after combined therapy with cytapheresis and corticosteroids. In the pulmonary hemorrhage patient, evidence of pulmonary hemorrhage on chest computed tomography scanning diminished after combined therapy with cytapheresis and corticosteroids. Cytapheresis, when combined with a low‐dose or intermediate‐dose PSL regimen, is effective in the treatment of ANCA‐associated vasculitis.     

Multiple dermatofibromas in a patient with systemic lupus erythematosus and Sjögren’s syndrome

We report a case of multiple dermatofibromas in a patient with systemic lupus erythematosus (SLE) and Sjögren’s syndrome. He was treated with prednisolone, diaphenylsulfone, and cyclosporine for SLE. He noticed two brown nodules on his right lower leg 3 years after the first consultation. Subsequently, six nodules developed within 6 months, and 2 more nodules after 10 months. Histopathological examination of a nodule on his left hand showed fibrotic proliferation with a storiform pattern in the whole dermis, but neither necrosis nor mitosis was observed. From these findings, a diagnosis of multiple dermatofibromas was made. As these tumors appeared during the remission stage of SLE, they might have been under immunosuppressive conditions caused by immunosuppressing agents rather than collagen disease itself.     

Dramatic development of severe SLE in a patient with an incomplete disease

This case report describes the previously-unreported clinical course of a patient with a so-called incomplete systemic lupus erythematosus (SLE), i.e. symptoms related to one organ system only, together with the presence of ANA. He had an indolent course initially and developed, 6 months after the first symptoms, a severe disease with rapid appearance of major and unusual manifestations. The possibility of fast progression and a grave course of an incomplete SLE should be kept in mind. This report is meant to heighten awareness of such an atypical presentation so that prompt and aggressive immunosuppressive therapy may be instituted.     

Diffuse alveolar hemorrhage as an unusual presentation of systemic lupus erythematosus

Diffuse alveolar hemorrhage (DAH) is rarely seen in patients with systemic lupus erythematosus (SLE), often associated with a poor outcome. It almost affects young women and it is an unusual initial manifestation of SLE. We report a case of SLE presenting with DAH. The patient was a male. He had no history of photosensitivity, malar rash, discoid rash, arthritis, and oral ulcer. Antinuclear antibody, and anti-double stranded DNA (dsDNA) were positive with very high titers, and serum complement levels (C3, C4) were low. He also had renal dysfunction and pericardial effusion. He was diagnosed as DAH due to SLE. He had to undergo hemodialysis for several weeks. DAH and renal dysfunction were improved with intensive treatment including corticosteroid, cyclophosphamide, and mycophenolate mophetil.     

Elderly onset systemic lupus erythematosus (SLE) presenting with disseminated intravascular coagulation (DIC)

We describe an unusual case of elderly onset systemic lupus erythematosus (SLE) that presented with disseminated intravascular coagulation (DIC). An 86-year-old man who complained of general malaise was admitted for evaluation and treatment of thrombocytopenia. He was diagnosed as having SLE and DIC based on the criteria of the American College of Rheumatology for SLE (renal involvement, hematological abnormalities, and positivity for antinuclear antibody and lupus anticoagulant) and the criteria for DIC presented by the subcommittee on DIC of the ISTH (a large increase of fibrin degradation products [3 points] and a platelet count <50?×?10³/ml [2 points], resulting in a score of 5; a score ≥5 is compatible with DIC). The patient was treated with corticosteroid therapy (30 mg/day); the DIC and SLE remitted, and his renal function improved, but he developed pulmonary tuberculosis. Timely diagnosis, appropriate treatment, and an awareness of the potential for serious infections are of utmost importance when dealing with patients with elderly onset SLE.     

Rupture of renal artery aneurysm due to Salmonella infection in a patient with systemic lupus erythematosus

Patients with systemic lupus erythematosus (SLE) are prone to infection. Immunomodulation treatment increases the susceptibility. Salmonella infections in SLE patients may present with various clinical pictures, like pneumonia, septic arthritis, osteomyelitis, peritonitis, abscess and so on. The vascular complications commonly seen in the general population with salmonella infection are rarely encountered in SLE patients. Here we report an SLE patient who presented with spontaneous rupture of salmonella mycotic aneurysm involving the left renal artery. The 54 year-old woman had a stable premorbid condition and had 30 mg prednisolone per day. Acute abdomen and hypotensive shock developed suddenly without warning signs in advance. Image and tissue culture confirmed the diagnosis. The patient had an uneventful recovery. The rare clinical scenario is reported.     

Acute lupus peritonitis successfully treated with steroid pulse therapy

A 21-year-old man with systemic lupus erythematosus (SLE) who developed acute lupus peritonitis is described. Acute lupus peritonitis appeared during generalized lupus flare, with nausea, vomiting, frequent diarrhea, and abdominal tenderness with rebound and guarding. The patient was afebrile and had decreased bowel sounds. Abdominal ultrasonography and computed tomography revealed marked thickening of the gastric, duodenal, and jejunal walls, massive intraluminal fluid collection, and increasing ascites. Gastrointestinal endoscopy showed edematous mucosa with multiple erosions of the stomach and duodenum. The ascitic fluid was remarkable for low complement levels and elevated anti-DNA antibody. These manifestations of acute lupus peritonitis resolved after steroid pulse therapy with methylprednisolone. We should consider acute lupus peritonitis in a patient with SLE when abdominal symptoms are severe. Experience with our patient indicates that steroid pulse therapy is effective for this rare but severe manifestation of SLE.     

Successful treatment of gastrointestinal vasculitis due to systemic lupus erythematosus with intravenous pulse cyclophosphamide: a clinical case report and review of the literature

Gastrointestinal vasculitis in systemic lupus erythematosus (SLE) is quite rare and almost always accompanied by evidence of active disease in other organs, although occasionally it may be the presenting feature of the disease. Gastrointestinal involvement in SLE may present as lupus peritonitis, non-necrotizing pancreatitis, gastrointestinal vasculitis or surgical abdomen. Here we report a severe case of SLE which presented initially with fever of unknown origin. Severe distress, abdominal pain, the presence of occult blood in the stool and high acute-phase proteins were explained by a lupus peritonitis and intestinal vasculitis resembling inflammatory bowel disease. Whereas high-dose prednisone treatment did not prevent a severe relapse, we observed a sustained remission following i.v. cyclophosphamide pulse therapy. In the literature, only two similar cases are reported: one died despite a change in the therapy of a bowel perforation; our case was the second that improved under pulse cyclophosphamide. We suggest the use of cyclophosphamide after failure of steroids early in the course of SLE gastrointestinal vasculitis to prevent devastating complications.      

Antinuclear antibody-negative systemic lupus erythematosus in a case with pregnancy

We described a 30-year-old pregnant woman developing antinuclear antibodies (ANA)-negative systemic lupus erythematosus (SLE) accompanied with arthritis, malar rash, lymphopenia, autoimmune hemolytic anemia, pericardial and pleural effusion, proteinuria and seizures. All serum autoantibodies except anti-Ro antibody were negative. An artificially induced abortion was performed to prevent SLE deterioration. Steroid and cyclophosphamide therapy were effective for this patient. During the 2-year follow-up period, her ANA and other SLE-related autoantibodies in serum remained negative. This case suggests that ANA may not be required in the pathogenesis of SLE, even in the case with pregnancy, and ANA-negative SLE may also have a dangerous clinical course.     

Managing hypertriglyceridemia in children with systemic lupus erythematosus: Two sides of the same coin

Hypertriglyceridemia is common in children with systemic lupus erythematosus (SLE). A retrospective analysis of the baseline clinical–pathological presentation and treatment outcome (status of lipid profiles) was performed in two children with SLE, who presented with extreme hypertriglyceridemia over a follow-up period of four weeks. The children were treated with prednisolone, mycophenolate mofetil (MMF), hydroxychloroquine and hypolipidemic agents, depending on their disease status. On serial follow-up, the first child showed a significantly raised serum triglyceride level after receiving one week of oral prednisolone therapy. Anti-lipoprotein-lipase (LPL) autoantibody was absent. Lipid profile levels of this child gradually improved after replacing oral prednisolone with another immunosuppressant, namely MMF. The second child presented with extreme hypertriglyceridemia with positive anti-LPL autoantibody. She responded to plasmapheresis followed by increasing the dose of immunosuppressant. So, extreme hypertriglyceridemia in children with SLE may be steroid induced or due to presence of anti-LPL auto antibody. Management should be individualized depending on the etiology.