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.     

Tubulo-interstitial nephritis (TIN) with no glomerular lesions, distal renal tubular acidosis and asteatosis cutis in a patient with systemic lupus erythematosus (SLE): a case report]

We report a 28-year-old woman with systemic lupus erythematosus (SLE) who showed tubulo-interstitial nephritis (TIN) without any glomerular changes. In 1990, she was admitted to our hospital, complaining of anorexia, vomiting and persistent high fever. Laboratory findings showed proteinuria, pancytopenia, hypocomplementemia and positive for antinuclear antibody, anti-DNA antibody, anti-Sm antibody, anti-SSA antibody and anti-SSB antibody. We made a diagnosis of SLE. Furthermore, distal renal tubular acidosis and asteatosis cutis were revealed. The diagnosis of Sj?gren's syndrome was not made. We treated with high-dose prednisolone (60mg/day) and achieved improvement of symptoms and laboratory data. Open renal biopsy showed TIN without any glomerular changes. Predominant TIN is very rare in SLE. We discussed its pathogenesis and relation to the renal lesions of Sj?gren's syndrome.     

A case of systemic lupus erythematosus (SLE) developing pan-dysautonomia]

A 43-year-old woman who had been diagnosed as primary Sj?gren's syndrome since 1986 developed severe constipation, urinary retention, dizziness at standing and polyarthralgia in February, 1990. Laboratory tests revealed proteinuria, hypocomplementemia and high titer of anti-DNA antibody. Diagnosis of SLE was made and she was admitted to our hospital on April 2, 1990. Physical examination on admission showed that she also had asymmetric pupils, impairment of sweating, orthostatic hypotension, neurogenic bladder, gastro-intestinal dysmotility and the diminution of R-R interval variability during deep breathing on the electrocardiogram. These findings suggested that she had pan-dysautonomia but there were no signs of motor and sensory disturbance. Because other diseases such as diabetes mellitus and amyloidosis which induced dysautonomia could be ruled out, her pan-dysautonomia seemed to be due to SLE. After the treatment with steroid pulse therapy, most of her dysautonomia improved rapidly. However, some of the disturbance had persisted for a long time. Pan-dysautonomia has been rarely reported as a complication of SLE, and high dose of steroid therapy at the early stage should be considered.     

Successful treatment of refractory systemic lupus erythematosus with intravenous immunoglobulins

Two cases of refractory systemic lupus erythematosus (SLE) were successfully treated with intravenous immunoglobulins (IVIg). In Case 1, the immediate recovery from severe pancytopenia and the improvement of proteinuria were observed, following IVIg therapy in high doses (450 mg/kg) for 5 consecutive days. In Case 2, 3 courses of IVIg therapy (100 mg/kg) for 6 to 8 days resulted in a significant reduction of massive proteinuria. In both cases, the improvement of immunological variables was also seen.     

Systemic lupus erythematosus following HPV immunization or infection?

BACKGROUND AND PURPOSE: The link between autoimmunity and infectious agents has been strongly suggested by reports of lupus or lupus-like syndromes following immunization. This report describes three patients with either newly diagnosed systemic lupus erythematosus (SLE) or SLE flare, following vaccination for human papilloma virus (HPV). CASE 1: A 17-year-old female completed two doses of HPV vaccine uneventfully. Two months later, she developed arthralgias with pruritic rashes on both lower extremities, later accompanied by livedo reticularis, bipedal edema with proteinuria, anemia, leucopenia, hypocomplementemia and high titers of anti-nuclear antibody (ANA) and anti-double-stranded DNA (anti-dsDNA). Kidney biopsy showed International Society of Nephrology/Renal Pathology Society Class III lupus nephritis. She was started on high dose steroids followed by pulse cyclophosphamide therapy protocol for lupus nephritis, and subsequently went into remission. CASE 2: A 45-year-old housewife, previously managed for 11 years as having rheumatoid arthritis, had been in clinical remission for a year when she received two doses of HPV immunization. Four months later, she developed fever accompanied by arthritis, malar rash, oral ulcers, recurrent ascites with intestinal pseudo-obstruction, and behavioral changes. Cranial MRI showed vasculitic lesions on the frontal and parietal lobes. Laboratory tests showed anemia with leucopenia, hypocomplementemia, proteinuria, ANA positive at 1:320, and antibodies against dsDNA, Ro/SSA, La/SSB and histone. She improved following pulse methylprednisolone with subsequent oral prednisone combined with hydroxychloroquine. CASE 3: A 58-year-old housewife diagnosed with SLE had been in clinical remission for 8 years when she received two doses of HPV immunization. Three months later, she was admitted to emergency because of a 1-week history of fever, malar rash, easy fatigability, cervical lymph nodes, gross hematuria and pallor. Laboratory exams showed severe anemia, thrombocytopenia, active urine sediments, and hypocomplementemia. Despite pulse steroid therapy, blood transfusions, intravenous immunoglobulin and aggressive resuscitative measures, she expired a day after hospital admission. SUMMARY: These cases narrate instances of the onset or exacerbation of lupus following HPV immunization suggesting adjuvant-induced autoimmunity. On the other hand, there are reports of higher incidence of HPV infection in SLE, with the infection per se possibly contributing to disease activity. Thus, the benefit of HPV immunization may still outweigh the risk among these individuals.     

A case of lupus nephritis improved after appropriately adjusting the dosage of mizoribine

Abstract

A 29-year-old male presenting nephrotic syndrome and facial skin erythema was admitted to our hospital in September of 2000. We diagnosed him as having systemic lupus erythematosus (SLE) accompanied by lupus nephritis (WHO class V). The disease activity had decreased after treatment with methylprednisolone (m-PSL) pulse therapy, which was followed by oral PSL. Thereafter, when tapering the dosage from 60 to 30 mg/day, the lupus nephritis flared up and he was re-hospitalized in February of 2001. After successful retreatment with m-PSL pulse therapy followed by the tapering of the dosage from 60 to 30 mg/day, we used mizoribine (MZR) as a combination therapy. The lupus nephritis flared up again after tapering down to 17.5 mg/day of PSL. Then, we changed the MZR dosage from 150 mg/day in three divided daily doses to 200 mg/day in two divided daily doses. This modification increased the peak blood concentration (Cmax) of MZR from 0.63 to 1.55 μg/ml. At present, we have been able to successfully taper the dosage to 7.5 mg/day of oral PSL and the patient has achieved a state of remission without any side effects. Monitoring of the serum concentration of MZR is thus considered to be important for achieving effective therapy of SLE, especially for steroid-resistant lupus nephritis. If the serum concentration of MZR does not reach an effective level, then the dosage of MZR should be adjusted appropriately in order to maintain an adequate serum concentration of MZR.     

A case of lupus nephritis improved after appropriately adjusting the dosage of mizoribine

A 29-year-old male presenting nephrotic syndrome and facial skin erythema was admitted to our hospital in September of 2000. We diagnosed him as having systemic lupus erythematosus (SLE) accompanied by lupus nephritis (WHO class V). The disease activity had decreased after treatment with methylprednisolone (m-PSL) pulse therapy, which was followed by oral PSL. Thereafter, when tapering the dosage from 60 to 30 mg/day, the lupus nephritis flared up and he was re-hospitalized in February of 2001. After successful retreatment with m-PSL pulse therapy followed by the tapering of the dosage from 60 to 30 mg/day, we used mizoribine (MZR) as a combination therapy. The lupus nephritis flared up again after tapering down to 17.5 mg/day of PSL. Then, we changed the MZR dosage from 150 mg/day in three divided daily doses to 200 mg/day in two divided daily doses. This modification increased the peak blood concentration (Cmax) of MZR from 0.63 to 1.55 μg/ml. At present, we have been able to successfully taper the dosage to 7.5 mg/day of oral PSL and the patient has achieved a state of remission without any side effects. Monitoring of the serum concentration of MZR is thus considered to be important for achieving effective therapy of SLE, especially for steroid-resistant lupus nephritis. If the serum concentration of MZR does not reach an effective level, then the dosage of MZR should be adjusted appropriately in order to maintain an adequate serum concentration of MZR.     

Lupus cystitis in the course of Evans syndrome

A 50-year-old woman with a 4-year history of Evans syndrome was admitted to our hospital because of progressive nausea, appetite loss, body weight loss, diarrhea and abdominal pain. Abdominal ultrasonography revealed pleural effusion, ascites, bilateral hydronephrosis, dilatation of the bilateral ureter, and irregular wall thickness of the urinary bladder. Immunological studies revealed decreased complement components (C3; 72 mg/dl, C4; 7 mg/dl, CH50; 28.8 mg/dl), a x 80 antinuclear antibody titer (homogeneous pattern), antibody against single-stranded DNA 19 U/ml, anti-SS-A antibody over 500 U/ml and negativity for antibody against double-stranded DNA (anti-dsDNA Ab). Although the patient did not fulfill the criteria for systemic lupus erythematosus (SLE), we diagnosed her as having lupus cystitis. Bolus methylprednisolone (mPSL) therapy (1,000 mg mPSL over 3 days, div) was administered, followed by 60 mg PSL, and this led to immediate improvement of the patient's symptoms and laboratory data. Later, anti-dsDNA Ab became positive, and the patient thereby fulfilled the criteria for SLE. Lupus cystitis following Evans syndrome has rarely been reported. The present such case was treated successfully with bolus mPSL therapy.     

Lupus pregnancy. II. Unusual pattern of hypocomplementemia and thrombocytopenia in the pregnant patient

To explore the causes of complications in pregnant women with systemic lupus erythematosus (SLE), we prospectively evaluated 34 pregnancies in 28 SLE patients, and 2 additional pregnancies in patients with lupus anticoagulant and positive antinuclear antibody, but no other manifestations of SLE. Nineteen pregnancies (55%) were complicated by marked proteinuria, thrombocytopenia, and/or lupus anticoagulant. Hypocomplementemia occurred in 18 pregnancies (52%). Neither thrombocytopenia-anticoagulant nor proteinuria was accompanied by an increase in antibody to double-stranded DNA or by clinical signs of active SLE. Antibody to Ro antigen did not predict fetal death. Both thrombocytopenia and proteinuria appeared abruptly during pregnancy and disappeared quickly after delivery. Fetal death was the result in 7 of 9 (77%) pregnancies in patients with anticoagulant, 6 of 10 (60%) in patients with thrombocytopenia, 6 of 18 (33%) in patients with hypocomplementemia, and 3 of 11 (27%) in patients with proteinuria. Twenty of 29 (68%) children were identified as male. The pathogenesis of hypocomplementemia was evaluated by a new assay, C1s-C1 inhibitor complex, which is thought to measure rate of complement activation by the classical pathway. Most pregnant patients with low CH50 levels and proteinuria had normal levels of C1s-C1 inhibitor complex, whereas nonpregnant patients with equivalent proteinuria and hypocomplementemia had high levels, as did pregnant patients with hypocomplementemia who did not have SLE. Pregnant and nonpregnant hypocomplementemic patients with proteinuria had similar levels of C3 and C4. In pregnant patients with SLE, C1s-C1 inhibitor complex was independent of CH50; in nonpregnant patients a linear relationship between C1s-C1 inhibitor complex and CH50 was seen.(ABSTRACT TRUNCATED AT 250 WORDS)     

A case of systemic lupus erythematosus associated with hemolytic uremic syndrome

We report a case of systemic lupus erythematosus (SLE) associated with hemolytic uremic syndrome (HUS). The patient was a 13-year-old boy who had complained of nausea and diarrhea. Abnormal urinalysis, pancytopenia and renal dysfunction were revealed. The immunological studies showed an elevation of antinuclear antibody and anti-double-stranded DNA antibody titers with a low complement level. Renal biopsy specimens showed diffuse membranous glomerulonephritis with microthrombi in the glomerular capillary lumen. He was diagnosed as having SLE with HUS. Methylprednisolone pulse therapy was performed. Renal function, proteinuria and hematuria were gradually improved. Prednisolone and an immunosuppressive agent (mizoribine) were prescribedper os. In our case, diarrheal prodrome was present, so gastro-enteritis was suggested as the trigger of HUS, but the causal agent was not detected. HUS was considered to be an accelerator in the renal lesions of SLE. There have been few reported cases in children of SLE associated with HUS.     

Etoposide ameliorated refractory hemophagocytic syndrome in a patient with systemic sclerosis]

We successfully treated a 33-year-old woman with etoposide who developed systemic sclerosis (SSc)-associated refractory hemophagocytic syndrome (HPS). She had been diagnosed as SSc because she had had Raynaud's phenomenon, proximal scleroderma, telangiectasia, microstomia, thickening and shortening of lingual frenulum and positive antinuclear antibody since 1994. In September 1999, she showed high fever, anemia, thrombocytopenia, elevation of serum lactate dehydrogenase (LDH) and ferritin levels and hemophagocytosis in her bone marrow, which led to the diagnosis of HPS. Her symptoms were improved by 40 mg of daily oral prednisolone (PSL). While tapering PSL, she complained right coxalgia and magnetic resonance image (MRI) depicted avascular necrosis (AVN) of right femoral head. In May 2000, she again suffered from HPS when she was taking 19 mg of PSL daily. To avoid the development of another AVN of her bone, she was treated with monthly cyclophosphamide (CPA) pulse therapy (300-400 mg/day). Although her HPS transiently ameliorated with CPA, it flared up again with high fever, general fatigue, severe pancytopenia and extremely high serum LDH and ferritin levels after the 4th CPA pulse therapy. She was admitted again to our hospital and PSL was increased to 40 mg daily which did not improve HPS. We, therefore, treated her with intravenous etoposide (100 mg/day, three consecutive days) along with granulocyte-colony stimulating factor (G-CSF). She developed transient bone marrow suppression, but her laboratory data gradually normalized within two weeks and she became afebrile after 18 days of etoposide administration. This is the first case in the literature which suggests the efficacy of etoposide against refractory autoimmune-associated hemophagocytic syndrome.     

An unusual case of ANA negative systemic lupus erythematosus presented with vasculitis, long-standing serositis and full-house nephropathy

Systemic lupus erythematosus (SLE) is a chronic inflammatory autoimmune disease that may affect any organ of the body. We report here an unusual case of seronegative SLE presented as vasculitis with rash, lower gastrointestinal system bleeding and acute renal failure. The patient was a 13-year-old boy, with abdominal distention, pretibial edema, arthritis and petechia on bilateral ankles. He had deteriorated renal functions (creatinine 1.65 mg/dl), hypoalbuminemia (1.6 g/dl) and hypocomplementemia with nephrotic range proteinuria and hematuria. He developed pleural effusion and peritonitis. Serum ANA, anti dsDNA, p ANCA, c ANCA, anticardiolipin IgM and IgG titers were negative. A renal biopsy was performed which revealed diffuse proliferative glomerulonephritis with full-house staining pattern in immunofluorescent microscopic examination suggesting Class IV Lupus Nephritis. He was administered a total of six courses of monthly intravenous pulse methyl prednisolone, dipyridamole, oral cyclophosphamide followed by azothiopirine and oral prednisolone therapy. The renal functions and serum albumin levels turned normal but peritonitis persisted and disappeared after the third pulse steroid therapy. In conclusion, we presented this patient to remind the possibility of SLE in such seronegative patients with unusual findings in order to avoid the delay in the management of this disease with high mortality and morbidity if not treated. Full-house nephropathy is an important clue especially for the diagnosis of ANA negative SLE.     

Studies on clinical subsets and severity of systemic lupus erythematosus based on a 1987 questionnaire conducted in Japan--clinical analysis of the outcome and treatments in clinical subsets]

A 1987 questionnaire sponsored by the Health and Welfare Ministry concerning the clinical subsets and severity of systemic lupus erythematosus (SLE) was distributed to 93 medial facilities. A clinical analysis of the outcome and treatments was accomplished on one thousand six hundred and fourteen SLE patients fulfilling ARA criteria. The outcome was evaluated into 6 categories, namely; complete remission, incomplete remission, no change, gradual worsening, rapid worsening and unknown. Treatments included (1) anti-inflammatory drugs, (2) initial dose of prednisolone (PSL) below 29 mg/day, (3) initial dose of PSL from 30 to 59 mg/day, (4) initial dose of PSL above 60 mg/day, (5) pulse therapy, (6) immunosuppressants, (7) plasmapheresis, and (8) hemodialysis. Statistical significances were determined with ridit analysis. The severity of the disease for 1,614 SLE patients was evaluated by the judgement of each medical facility independently, separating it into 3 grades. As a result, 16.8% was evaluated as severe, 54.6% was evaluated as moderate, and 28.6% was evaluated as mild. Clinical subsets were divided into 3 categories according to the outcome; (1) those with high complete remission rates (serositis, convulsion, oral ulcers, unconsciousness, hemolytic anemia and so on), (2) those with high incomplete remission rates (lupus nephritis, digital gangrene, hypertension, peripheral neuropathy, erythema, Raynaud's phenomenon and so on), and (3) those with high rates of no change or worsening (aseptic bone necrosis, pulmonary hypertension, pneumonitis, chronic renal failure and so on). SLE patients with persistent proteinuria below 3.4 g/day, pulmonary hypertension, or pneumonitis treated with large doses of PSL such as an initial dose of PSL above 60 mg/day and/or pulse therapy had a significantly higher remission rate than those treated with small dosages of PSL. Hereafter, the establishment of modes of treatments for increasing the remission rates of intractable clinical subsets in highly desired.     

A case of lupus cystitis with a history of idiopathic thrombocytopenic purpura

A 36-year-old Japanese woman who had been diagnosed as having systemic lupus erythematosus (SLE) at the age of 34 began to complain of severe bowel symptoms and developed severe hydroureteronephrosis. She had a history of idiopathic thrombocytopenic purpura. Biopsy specimens from her bladder showed interstitial cystitis. She was diagnosed as having lupus cystitis, and treated with intravenous methylprednisolone pulse therapy followed by oral prednisolone and ureter catheterization. Her urinary and bowel symptoms were alleviated and the level of hydroureteronephrosis improved. We note that cystitis could be a primary manifestation of SLE. Patients not only with SLE but also with some autoimmune diseases require careful urological evaluation when they complain of severe bowel symptoms.     

A case report: a pediatric patient with acute lupus hemophagocytic syndrome; differences from reactive hemophagocytosis caused by hypercytokinemia

We report the youngest known girl with acute lupus hemophagocytic syndrome (ALHS) at the onset of her illness. We investigated the pathogenesis of ALHS by assessment of factors thought to influence the onset, such as cytokines, Th1/Th2 balance, immune complexes, and autoantibodies. A girl 8 years and 10 months old with systemic lupus erythematosus (SLE) had high fever, pancytopenia, and hemophagocytosis in the bone marrow. We diagnosed SLE complicated by ALHS. Treatment with predonisolone (2 mg/kg/day) was started, and her clinical features improved. Th2 dominance of the Th1/Th2 balance, hypocomplementemia, and high levels of anti-ds-DNA antibody, PAIgG, and immune complexes were seen, but no hypercytokinenemia, hyperferritinemia, or hypertriglyceridemia. ALHS at the onset of SLE, excluding that caused by infections, could be a form of reactive hemophagocytosis caused by excessive production of autoantibodies and immune complexes. High-dose steroid therapy is effective without need for immunosuppressive drugs. Our patient showed hypocomplementemia along with high levels of anti-dsDNA antibody, anticardiolipin antibody, platelet-associated IgG (PAIgG), and immune complexes. The autoimmune-associated hemophagocytic syndrome (AAHS) is thought to involve an autoantibody-mediated mechanism or an immune complex-mediated mechanism. On the basis of our data, ALHS could simultaneously involve both these mechanisms. We demonstrated that there was no hypercytokinemia and no hyperferritinemia in ALHS associated with Th2 dominance. Autoantibodies and immune complexes may cause histiocytic hemophagocytosis in ALHS. High-dose steroids and high-dose immunoglobulin are effective but immunosuppressive drugs are not needed. Received: September 21, 2000 / Accepted: July 5, 2001     

A case of systemic lupus erythematosus associated with hemolytic uremic syndrome

Abstract

We report a case of systemic lupus erythematosus (SLE) associated with hemolytic uremic syndrome (HUS). The patient was a 13-year-old boy who had complained of nausea and diarrhea. Abnormal urinalysis, pancytopenia and renal dysfunction were revealed. The immunological studies showed an elevation of antinuclear antibody and anti-double-stranded DNA antibody titers with a low complement level. Renal biopsy specimens showed diffuse membranous glomerulonephritis with microthrombi in the glomerular capillary lumen. He was diagnosed as having SLE with HUS. Methylprednisolone pulse therapy was performed. Renal function, proteinuria and hematuria were gradually improved. Prednisolone and an immunosuppressive agent (mizoribine) were prescribed per os. In our case, diarrheal prodrome was present, so gastro-enteritis was suggested as the trigger of HUS, but the causal agent was not detected. HUS was considered to be an accelerator in the renal lesions of SLE. There have been few reported cases in children of SLE associated with HUS.     

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.     

Two cases of lupus cystitis with no bladder irritation symptoms

Lupus cystitis is a rare manifestation in systemic lupus erythematosus (SLE); it usually occurs in association with gastrointestinal manifestations. We report two cases of lupus cystitis without bladder irritation symptoms. Both cases developed severe abdominal pain, nausea, and diarrhea and showed no bladder irritation symptoms. The diagnosis of lupus cystitis was made by abdominal ultrasonography and bladder biopsy. The patients were treated with intravenous methylprednisolone pulse therapy followed by oral prednisolone. Their symptoms were ameliorated, and hydroureteronephrosis improved. Thus, when a patient with SLE shows gastrointestinal symptoms, further examinations are required to determine whether the patient has lupus cystitis.     

Pregnancy does not cause systemic lupus erythematosus to worsen

To evaluate risk for exacerbation of systemic lupus erythematosus (SLE) during pregnancy, we prospectively evaluated 80 pregnant women with SLE for manifestations of disease activity. Fifty-three of these women were not taking prednisone at the time of conception. Disease activity was scored in 4 ways: global assessment, prednisone therapy, cumulative number of organ systems with abnormalities, and display of abnormalities of each organ system. No patient received prophylactic therapy to prevent disease exacerbation. Thrombocytopenia, proteinuria, and hypocomplementemia were the most common abnormalities and were usually attributable to the pregnancy complications of preeclampsia and anticardiolipin antibody syndrome rather than to SLE. If all possible abnormalities were attributed to SLE, disease exacerbation occurred in less than 25% of all patients; if only SLE-specific abnormalities were counted, disease exacerbation occurred in less than 13%. Worsening of SLE is uncommon in pregnancy, and prophylactic prednisone therapy is unnecessary.     

Familial Crohn's disease with systemic lupus erythematosus

We describe a young Japanese woman who was diagnosed with Crohn's disease affecting the ileum, transverse colon, and rectum, as confirmed by barium studies, colonoscopy, and histopathological examination. Her father and sister also had Crohn's disease. After a 4-yr course of sulfasalazine and elemental diet therapy, she was readmitted for perianal abscess associated with the presence of pancytopenia, microhematuria with granular cast, hypocomplementemia, and high titers of autoimmune antibodies (anti-ANA and anti-dsDNA antibodies). Based on these features, a diagnosis of systemic lupus erythematosus (SLE) was made. Despite the rarity of such combination (Crohn's disease with SLE), patients with Crohn's disease who develop such clinical findings might need evaluation for SLE.