A case of systemic lupus erythematosus showing abnormal lipoproteins due to accompanied drug induced hepatitis

We describe here a 28-years-male with AIHA and SLE who had lipid and lipoprotein abnormalities during cholestasis induced by PGE1 administration. High free cholesterol level, 792 mg/dl was found in his serum, and markedly elevated, phospholipid level 1,614 mg/dl. But, LCAT activity was within normal range in this case. An agarose gel electrophoresis of lipoproteins showed abnormal bands which were located in slow alpha 2, pre beta and slow beta, and between beta and origin point. Moreover, it was detected formation of Lp-X from serum of the patient. Serum levels of apoprotein B, C-II, C-III, and E were higher, while apoprotein A-I, A-II were very lower than reference value. From these results, it was suspected that the patient might occur transient abnormal lipid metabolism according to the drug induced hepatic injury.     

A case of systemic lupus erythematosus presenting with jaundice and lupus pneumonia]

A 27-year-old Japanese woman was referred to our hospital for acute hepatitis in April 2002. She had been suffering from low grade fever and fatigue for a week. She also presented with dyspnea. On admission, ALT and AST were 857 U/l and 473 U/l respectively. Urine protein was 2 g/day. Chest radiograph showed bilateral infiltrative shadow and pleural effusion. She developed jaundice and her level of total bilirubin was increased to 9.6 mg/dl on May 9. Antibodies to hepatitis viruses were not detected. Testing for antimitochondrial antibodies, antismooth muscle antibodies, and antiribosomal P antibodies showed all negative. However, antinuclear antibodies were positive at titer 1:160 and anti-double stranded DNA antibodies were 130 U/ml. A diagnosis of systemic lupus erythematosus was made and oral administration of 60 mg/day prednisolon was started on May 10. Serum levels of ALT, AST and bilirubin were reduced to within normal range and pulmonary lesions were also improved. We conclude that this is a rare case of systemic lupus erythematosus presenting with acute hepatitis and jaundice.     

Antibodies to poly (adenosine diphosphate-ribose) in systemic lupus erythematosus and drug induced lupus]

The difference between anti poly (ADP-ribose) antibodies was studied in patients with systemic lupus erythematosus (SLE), progressive systemic sclerosis (PSS) and drug-induced lupus (DIL). Radioimmunoassay showed that high concentrations of anti poly (ADP-ribose) antibodies (10.3-22.2%) were induced by phenobarbital, phenytoin, valproic acid (anti-epileptic agent) and procainamide (anti-arrhythmic agent). Poly (ADP-ribose) antibodies were separated by hydroxylapatite column chromatography. The average chain length of the polymer consisted of 2.4, 10.8 and 28.2 ADP-ribose units. Anti poly (ADP-ribose) antibodies from patients with SLE and PSS reacted with 2.4, 10.8, and 28.2 ADP-ribose units in RIA, but those from patients with DIL reacted only with 28.2 ADP-ribose units in RIA. The binding specificity of anti poly (ADP-ribose) antibodies from pregnant women was found to be very similar to that of the antibodies from case of DIL. The present results clearly demonstrated that anti poly (ADP-ribose) antibodies found in systemic autoimmune diseases bound not only to poly (ADP-ribose) with an average chain length of more than 20 ADP-ribose units, but also to oligo (ADP-ribose) with an average chain length of about 2 ADP-ribose units. Anti poly (ADP-ribose) antibodies found in DIL cases and pregnant women, however, bound only to poly (ADP-ribose) with an average chain length of more than 20 ADP-ribose units.     

A case report; systemic lupus erythematosus associated with lupus laryngitis]

Systemic lupus erythematosus (SLE) is an autoimmune collagen vascular disease which produces widespread damage to multiple organs. Few studies on laryngeal involvement in SLE have been reported. We report here a case of SLE complicated by lupus laryngitis. A 27-year-old woman was diagnosed as having SLE in October 1996 based on findings of polyarthritis, lymphocytopenia, positive anti-nuclear antibody and anti-Sm antibodies. Polyarthralgia disappeared transiently, and no other clinical symptoms were found. Therefore, she was followed with no medication. She presented hoarseness with high fever and facial erythema in September 1998. Laboratory findings on admission revealed pancytopenia and hypocomplementemia. Anti-nuclear antibody, anti-Sm antibody and anti-RNP antibody were all positive with high titers. Chest X ray examination showed pleural effusion. Laryngoscopy showed a bamboo-joint-like lesion at the middle of the bilateral vocal cords. She was treated with 60 mg of prednisolone (i.v.) which resulted in improvement of hoarseness, pancytopenia, hypocomplementemia and pleuritis. Based on these findings, we diagnosed her hoarseness as the manifestation of laryngitis associated with SLE (lupus laryngitis).     

A case report of childhood systemic lupus erythematosus complicated with lupus cystitis]

The patient was a 13-year-old girl. In August 2000, she presented with a fever, together with diarrhea, vomiting, arthralgia, nasal bleeding and malaise, and was examined by another physician. Because her platelet count was low, and there were positive reactions for anti-nuclear antibodies, anti-DNA antibodies and platelet-associated IgG, idiopathic thrombopenic purpura, and systemic lupus erythematosus (SLE) was suspected. From January 2001, when she caught measles, she reported abdominal pain, and urinalysis indicated urinary protein and occult blood, and the left kidney was found hydronephrotic. At the same time left ureter stenosis and dilatation were demonstrated. Symptoms were disappeared by hydration and treatment with NSAIDs, but 2 months later fever and erythematous patches seen on both cheeks led to the proper diagnosis of SLE, and she was admitted to our hospital. Intravenous pyelography revealed hydronephrosis on left kidney, constriction and dilatation of the left ureter, and intracystic endoscopy showed erythema at the orifice of the left ureter. The pathological examination indicated the presence of vasculitis, and finally lupus cystitis was diagnosed. Intravenous cyclophosphamide (IVCY)-pulse therapy was introduced to a total of 8 times over the period of a year, and maintenance therapy with predonisolone and azathioprin was also used. After completion of the IVCY-pulse therapy, the hydronephrosis and constriction of the ureter were disappeared. No side effects of IVCY-pulses were observed, and the patient is now in remission. We reported a case of childhood SLE complicated with lupus cystitis and successfully treated by IVCY-pulse therapy and maintenance predonisolone and azathioprin.     

A case of systemic lupus erythematosus complicated with psoriasis vulgaris]

A 49-years-old female admitted to our hospital because of skin eruptions on the extremities in 1985. She had suffered from polyarthralgia, skin eruptions since 1983. Physical examinations revealed discoid lesion, central nervous system involvement, and polyarthritis. Laboratory tests revealed leukopenia, thrombocytopenia, and hypocomplementemia. Antinuclear antibody, ant-DNA antibody, LE test were positive. From these findings, she was diagnosed as systemic lupus erythematosus (SLE). She developed lupus peritonitis in 1990 and 1994, which was successfully treated by steroid pulse therapy. Since then, the activity of SLE was in good control under administration of prednisolone 10 mg/day. Chilblain lupus was seen from 1993, Raynaud's phenomenon from 1996, and she further developed subcutaneous induration on her chest, back and upper extremities in 1999. Skin biopsy findings were compatible with lupus panniculitis. In 2002, erythematous patches with scales were observed on her right hand and left knee, and these skin lesions were histologically diagnosed as psoriasis vulgaris. An autoimmune response similar to SLE is speculated in psoriasis. We describe a rare case of SLE with various skin lesions including psoriasis vulgaris.     

A case of systemic lupus erythematosus associated with hypertrophic cardiomyopathy]

The patient was a 37-year-old female, who was diagnosed as having systemic lupus erythematosus (SLE) with nephrotic syndrome in 1991. SLE has been well controlled with a combination therapy of prednisolone, cyclophosphamide and mizoribine. She was admitted to our hospital for chest pain on exertion in June 2002. A grade of 2 systolic murmur was heard along left sternal border and edema in the both lower legs was present. Laboratory findings showed proteinuria and anemia. Serological tests did not show decrease in complements and was negative for autoantibodies including anti-ds-DNA antibody. The serum level of brain natriuretic peptide was 651 pg/ml. On chest X-ray films, there were no remarkable findings. An electrocardiogram showed a pattern of left ventricular hypertrophy with inverted T wave. The heart ultrasonic test recognized asymmetric hypertrophy of the septum, being more prominent in the apex, but there was no obstruction of the left ventricular outflow tract. Examination of an endomyocardial biopsy specimen showed disarray and mild hypertrophy of myocardial cells, which were compatible with hypertrophic cardiomyopathy (HCM), but there were no pathological findings specific for SLE. Additional treatment with beta-blocker under a diagnosis of HCM resulted in a favorable response. Although 7 SLE patients with HCM have been reported, endomyocardial biopsy was not performed. There appears to have been a chance association between SLE and HCM, considering the clinical courses in reported cases and the pathological findings in our case.     

A case of thrombotic thrombocytopenic purpura with systemic lupus erythematosus]

We described a case of thrombotic thrombocytopenic purpura (TTP) with systemic lupus erythematosus (SLE). A-60-year old woman was admitted to our hospital because of fever, disconsciousness, and general fatigue. 32 years ago, she was diagnosed as SLE with Raynaud's phenomenon, rash, photosensitivity, arthritis, lymphocytopenia, and ANA. Her SLE was well controlled with 10 mg predonisolone as a maintance dose until several weeks ago. On admission, severe thrombocytopenia (0.7x10(4)/microl) and other laboratory data revealed microangiopathic hemolytic anemia and renal dysfunction, Immediately after diagnosed as TTP, plasma exchange and corticosteroid therapy started. In spite of the treatment, disconsciousness progressed and systemic convulsion occurred and died 4 days after admission. Autopsied examination revealed diffuse microvascular hyalinized thrombi in heart, kidney, liver, spleen, and pancreas. Some microvascular thrombi were detected in lymph nodes, bone marrow, intestine. Pathological diagnosis of TTP was made on microvascular hyalinized platelet thrombi in organs. Von Willebrand factor-cleaving protease (VWF-CP) activity in plasma on set is less than 0.5 percent of normal and inhibitor for VWF-CP was detected. We here report a valuable case for analysis of pathogenesis in SLE-TTP.     

A case of drug eruption induced by hydroxyzine pamoate]

A 62-year-old woman with rheumatoid arthritis, Basedow's disease and arrhythmia has been treated with antirheumatic, antiarrhythmic drugs and so on. She developed pruritic diffuse erythema with papules on the trunk and extremities 2 days after taking hydroxyzine pamoate for asteatotic eczema. Laboratory data showed increased levels of eosinophils. Histopathological examination revealed a infiltrate of inflammatory cells in the upper dermis. Patch tests with hydroxyzine pamoate and hydroxyzine hydrochloride were positive. From these findings, we diagnosed this case as drug eruption due to hydroxyzine. Her eruption subsided after she discontinued hydroxyzine pamoate and other drugs which were started within 5 days before the onset of the eruption and was treated with systemic steroid, systemic antiallergic drug and topical steroid.     

A case of lupus erythematosus profundus followed by systemic lupus erythematosus presenting with severe intestinal involvement]

A 52-year-old female visited the outpatient department of Sapporo Medical University hospital in 1984 due to a refractory rash on the skin of the trunk. Histological findings of a skin biopsy specimen indicated a diagnosis of lupus erythematosus profundus (LEP). The eruption remained quiescent with moderate doses of prednisolone. In October 2003, she was suddenly admitted to our hospital with abdominal pain, fever and bloody stool. Lupus enteritis was diagnosed based on an elevated level of anti-DNA antibody, low complementemia and diffuse edematous change of the intestinal walls on CT scans. Although high doses of corticosteroids resulted in transient improvement, melena developed again on the 24th hospital day. Colonoscopy revealed deep ulceration at the rectum and a gastrografin enema indicated perforation. Accordingly, the involved rectum was resected and an artificial anus was constructed on the 50th hospital day. Examination of the resected specimen by microscopy showed that the ulceration approached at the depth of the subserosal layer with intense infiltration of inflammatory cells around the vessels. The pathogenesis of the rectal lesion might have been due to vasculitis associated with systemic lupus erythematosus (SLE). The disease did not recur under the administration of 10 mg of prednisolone daily until November 2004. Colonic function made a remarkably untroubled recovery after the artificial anus was closed in February 2005. The LEP was generally accompanied by a mild form of SLE. This case seemed to be rare in that SLE was associated with severe lupus enteritis and a refractory rectal ulcer developed from LEP. Patients with SLE and intestinal involvement should be carefully monitored in cooperation with a surgeon.     

A case of systemic lupus erythematosus which Parkinsonism was induced by tacrolimus

The present case is the first report of a systemic lupus erythematosus patient which has been induced Parkinsonism with the administration of tacrolimus (TAC). A 50-year-old woman was diagnosed as lupus nephritis on September 2003. The patient had been prescribed initially 40 mg/day of prednisolone, then cyclosporine was added on May 2005. One year later, she developed severe headache, so cyclosporine was stopped, and she was prescribed tacrolimus on February 2007. However her severe headache had been disappeared, she experienced rigidity and tremor around September 2007. The Dopamine-transporter-imaging examination reavealed that she had Parkinson's disease. The gene analysis on the genetic background showed her case was the sporadic type? Parkinson's disease. Washing out of Tacrolimus, her Parkinsonism was partially improved. This fact suggested that her Parkinsonism was drug-induced type Parkinsonism. In lupus nephritis patients who have been treated with TAC, a very careful observation should be considered because neurological disorders inducing Parkinsonism may occur.     

A case of systemic lupus erythematosus associated with superior vena cava syndrome]

We report on a case of systemic lupus erythematosus associated with superior vena cava syndrome. A 46-year-old woman developed polyarthralgia in December 1994. She was treated with nonsteroidal anti-inflammatory drugs. In February 1995, she was admitted to our hospital with systemic convulsion and disturbance of consciousness (III-300/Japan coma scale). Severe facial edema was also present. Laboratory studies revealed the presence of anti-nuclear antibody, anti-DNA antibody, anti-Sm antibody, and proteinuria. An X-ray film of the chest showed pericardial effusion and bilateral pleural effusions. Computed tomography of the chest showed a severe swelling of mediastinal lymph nodes. A diagnosis of systemic lupus erythematosus was made according to the American Rheumatism Association criteria. Initial treatment with intravenous dexamethasone improved the level of consciousness and decreased the facial edema, mediastinal lymphadenopathy, and the effusions on computed tomography of the chest. We believe that the most likely explanation for the facial edema is superior vena cava syndrome due to severe mediastinal lymphadenopathy.     

A case of systemic lupus erythematosus associated with thrombotic thrombocytopenic purpura and hemophagocytic syndrome]

The case: A 44-year-old female. Developed polyarthralgia in August 2002. The patient was diagnosed with systemic lupus erythematosus (SLE) in November, due to polyarthralgia, leukopenia, anti ds-DNA antibody positive, antinuclear antibody positive, and false positive serologic test for syphilis. Hypocomplementemia continued even after the steroid treatment was conducted with insufficient control. In November 2003, ran fever and observed polyarthralgia. In December, gross hematuria and purpura appeared. The patient was hospitalized on December 25. Thrombotic thrombocytopenic purpura (TTP) was suspected from the emergence of fragmented red cells, hemolysis, thrombocytopenic purpura, headache, renal dysfunction and fever. As hemophagocytic syndrome (HPS) was suspected from hyper-ferritinemia, bone marrow aspiration was conducted. Macrophage confirmed hemophagocytic image and the patient was diagnosed with HPS complication. Methylprednisolone pulse therapy was conducted for three days, followed by the administration of prednisolone 60 mg. Plasma exchange therapy was also conducted from the first day of hospitalization. Recurrence of TTP after plasma exchange therapy, but it improved by additional plasma exchange. Low vWF-CP (ADAMTS-13) activity was observed in this case, and anti-vWF-CP antibody was positive. TTP and HPS are both critical intractable complications of SLE. Bearing in mind the possibility of simultaneous complication of both symptoms, prompt diagnosis is crucial for life-saving.     

Advances in drug therapy for systemic lupus erythematosus

Systemic lupus erythematosus (SLE) is an autoimmune disorder that afflicts 500,000 people in the United States. There has not been a new SLE drug approved in the United States since 1958. However, a guidance document issued by the Food and Drug Administration in 2005 provided a roadmap for investigators which spawned numerous ongoing clinical trials. Among these, Belimumab, a monoclonal antibody to soluble B lymphocyte stimulator, met its primary endpoints in two large trials and will probably obtain FDA approval soon. Other promising agents targeting a variety of mechanisms of action are currently in development. This minireview highlights the latest therapies under investigation in SLE and gives an overview of the pathways that are specifically being targeted.     

A case of systemic lupus erythematosus associated with thrombotic thrombocytopenic purpura manifestating homonymous hemianopsia]

We report on a 35-year-old Japanese woman with systemic lupus erythematosus (SLE) in whom homonymous hemianopsia developed. In August 1987, SLE was diagnosed. In November 1987, the patient was admitted to our hospital because of severe thrombotic thrombocytopenic purpura; however, clinical symptoms improved after treatment with vincristine. In 1992, severe lupus nephritis developed but improved after steroid therapy. In February 1994, diplopia developed owing to inflammatory invasion of the orbit by acute maxillary sinusitis. Humphrey Field Analyzer perimetry revealed a homonymous quadrantic hemianopic scotoma in the lower left side. Magnetic resonance imaging of the brain revealed a small infarct lesion in the posterior pole of the upper calcarine cortex. This lesion was thought to be responsible for homonymous hemianopsia. However, the lesion was not visualized with computed tomography. Our experience in the present case suggests that magnetic resonance imaging is more useful than computed tomography for identifying lesion causing visual field disorders in SLE.     

Amyloid A amyloidosis and systemic lupus erythematosus

Amyloid A (AA) amyloidosis occurs secondary to long-standing inflammation and causes nephropathy and various internal manifestations, which leads to mortality. It is very rare in some rheumatic diseases, such as systemic lupus erythematosus (SLE). Therefore, there are few articles that report AA amyloidosis in SLE. This article focuses on the previously reported cases of 24 patients with SLE that are complicated by AA amyloidosis, and on the underlying mechanisms.     

Amyloidosis in a case of canine systemic lupus erythematosus

Concurrent systemic lupus erythematosus (SLE) and amyloidosis (renal and splenic) are reported in a 7-year-old female miniature Schnauzer. Treatment of tissue sections with potassium permanganate and dilute sulphuric acid prior to staining with Congo red indicated that the amyloid in this case is composed of AA protein (i.e. reactive systemic amyloid or so-called secondary amyloid). The rare association of amyloidosis and SLE, in both man and the dog, and the association with granulomatous pneumonia and leukopenia in this case are discussed.