Clinical and structural remission rates increased annually and radiographic progression was continuously inhibited during a 3-year administration of tocilizumab in patients with rheumatoid arthritis: A multi-center, prospective cohort study by the Michinoku Tocilizumab Study Group.

OBJECTIVE: To evaluate the clinical and structural efficacy of tocilizumab (TCZ) during its long-term administration in patients with rheumatoid arthritis (RA). METHODS: In total, 693 patients with RA who started TCZ therapy were followed for 3 years. Clinical efficacy was evaluated by DAS28-ESR and Boolean remission rates in 544 patients. Joint damage was assessed by calculating the modified total Sharp score (mTSS) in 50 patients. RESULTS: When the reason for discontinuation was limited to inadequate response or adverse events, the 1-, 2-, and 3-year continuation rates were 84.0%, 76.8%, and 72.2%, respectively. The mean DAS28-ESR was initially 5.1 and decreased to 2.5 at 6 months and to 2.2 at 36 months. The Boolean remission rate was initially 0.9% and increased to 21.7% at 6 months and to 32.2% at 36 months. The structural remission rates (ΔmTSS/year ≤ 0.5) were 68.8%, 78.6%, and 88.9% within the first, second, and third years, respectively. The structural remission rate at 3 years (ΔmTSS ≤ 1.5) was 66.0%, and earlier achievement of swollen joint count (SJC) of 1 or less resulted in better outcomes. CONCLUSIONS: TCZ was highly efficacious, and bone destruction was strongly prevented. SJC was an easy-to-use indicator of joint destruction.

Tocilizumab Treatment for Takayasu Arteritis in Pregnancy: A Case Report With Positive Maternal and Neonatal Outcomes.

Takayasu arteritis (TAK) is a rare vasculitis that often affects young women of childbearing age, and its management during pregnancy poses unique challenges. Limited data exist regarding the safety and efficacy of tocilizumab (TCZ), an interleukin-6 receptor antagonist, in the treatment of TAK during pregnancy. This case report presents a unique and valuable insight into the use of TCZ in pregnant patients with TAK. We report an 18-year-old female patient with TAK who was treated with TCZ during two pregnancies, resulting in positive maternal and neonatal outcomes. However, a newly identified descending aortic aneurysm was noted after the second delivery, highlighting the importance of careful monitoring of vascular lesions in patients with TAK receiving TCZ. Our findings suggest that TCZ has a high safety profile for both the mother and fetus; however, further research and close monitoring are essential for its use in pregnant patients with TAK.

A Rare Case of Suspected Neuronal Intranuclear Inclusion Disease Requiring Differentiation From Neuro-Behçet's Disease.

A 69-year-old male patient with a long-standing history of Behçet's disease was admitted to another hospital with minor physical injuries after a traffic accident. However, the patient was subsequently transferred to our facility because of a prolonged disorder of consciousness suspected to be related to neuro-Behçet's disease (NBD). A thorough patient evaluation for determining the most appropriate treatment course led to a strong suspicion of neuronal intranuclear inclusion disease and predominantly ruled out NBD. This eliminated the need for unnecessary immunosuppressive intervention. Thereafter, the patient was transferred to a long-term care facility. This case highlights the importance of careful identification of pathological conditions before developing a treatment plan, regardless of the presence or absence of an underlying disease.

Rapid Progression of Skin Sclerosis Following Surgery for Carpal Tunnel Syndrome: A Case of Diffuse Cutaneous Systemic Sclerosis.

Carpal tunnel syndrome (CTS) is a frequently encountered compressive neuropathy that is often treated surgically. Here, we present an unusual case of a 74-year-old female who developed a rapid emergence of skin sclerosis following CTS surgery. The condition was initially misdiagnosed as complex regional pain syndrome. However, since her skin condition progressed, she was referred to the rheumatology department. Subsequent evaluations confirmed the diagnosis of diffuse cutaneous systemic sclerosis, accompanied by interstitial lung disease. Treatment with mycophenolate mofetil did not notably alter the interstitial lung shadows but led to minor improvement in skin sclerosis. It is crucial to consider the possibility of rheumatic diseases in patients with unexpected postoperative symptoms.

Anti-ribosomal P antibodies are associated with nephritis, vascular thrombosis and lymphocytopenia in patients with systemic lupus erythematosus.

In the present study, anti-ribosomal P antibody in sera of patients with systemic lupus erythematosus was assayed using an enzyme-linked immunosorbent assay, and its association with clinical symptoms of the patients was analyzed. The presence of anti-ribosomal P antibody was associated with increased frequency of lupus nephritis in the presence of anti-DNA antibody, and was associated with increased frequency of vascular thrombosis in the presence of anti-beta2 glycoprotein I antibody and/or lupus anticoagulant. The level of anti-ribosomal P antibody correlated inversely with the peripheral lymphocyte counts.

An anti-nuclear antibody-negative patient with systemic lupus erythematosus (SLE) accompanied with anti-ribosomal P antibody (anti-P).

We report a case of an anti-nuclear antibody (ANA)-negative patient with systemic lupus erythematosus (SLE) accompanied with anti-phospholipid antibody syndrome (APS) and lupus nephritis (LN). Histological examination of placenta obtained by an artificially-induced abortion revealed multiple thromboses in the placental villi. Histology of biopsied kidney tissue revealed minimal change with deposits of immunoglobulin and complement. Anti-ribosomal P antibodies (anti-P) and lupus anticoagulant (LAC) were positive and anti-double stranded DNA antibody (anti-DNA) showed only a slightly positive titer in her serum. The intensity of proteinuria of the patient was correlated with the anti-P, but not anti-DNA titers.

Self-limited colitis during the course of rubella and cytomegalovirus infection in an immunocompetent adult.

We report a case of self-limited colitis in cytomegalovirus (CMV) infection in an immunocompetent adult. A 22-year-old man developed a high fever and diarrhea. Laboratory data revealed an increased number of lymphocytes and liver damage. Enzyme immunoassays for anti-virus antibodies revealed that the patient was recently infected with CMV and rubella. Colonoscopy revealed severe erosive and edematous mucosa that resembled ulcerative colitis (UC). The symptoms, laboratory data and colonoscopic findings improved without any medical treatment. This case indicates that UC-like self-limited colitis can occur in an immunocompetent individual during the course of CMV infection.

Ulcerative colitis occurring in the course of rheumatoid arthritis: a case successfully treated with mesalamine enema.

We report a case of ulcerative colitis (UC) that occurred during the course of rheumatoid arthritis (RA). A 29-year-old woman with a 25-year history of RA was hospitalized for high fever, abdominal pain and hematochezia. Colonoscopy revealed erosive and reddish mucosa from the distal transverse colon to rectum. Histology revealed cryptitis (mainly caused by neutrophils), mild crypt abscess and goblet cell depletion. She was diagnosed with left-sided UC and treated with mesalamine enema. The abdominal symptoms and colonoscopic findings were greatly ameliorated. We conclude that the mesalamine enema was effective in this case of UC occurring during the course of RA.

A case of polyarteritis nodosa limited to lower legs with a high titer of MPO-ANCA under precedence of idiopathic pulmonary fibrosis.

A 58-year-old man with a 15-year history of idiopathic pulmonary fibrosis was hospitalized for rapid progression of muscle weakness to bilateral foot drop. Although laboratory data revealed high titers of myeloperoxidase anti-neutrophil cytoplasmic antibody (489 EU), the patient was diagnosed as polyarteritis nodosa limited to the lower portions of the legs. Despite of the treatment with large doses of corticosteroids and cyclosporin A, his symptoms barely improved during the following two months.

Antikeratin antibodies in sera of patients with connective tissue disease and liver disease.

Sera from 93 patients with connective tissue disease, 36 rheumatoid arthritis, 41 systemic lupus erythematosus, 12 polymyositis/dermatomyositis and 4 systemic sclerosis and sera from 12 patients with liver disease, along with sera of 10 healthy subjects, were tested for antikeratin antibodies using enzyme-linked immunosorbent assay in which the wells were coated with human epidermal keratin. Values above the mean+SD of the 10 healthy subjects were found in 8.3% of rheumatoid arthritis, 29.3% of systemic lupus erythematosus, 33.3% of polymyositis/dermatomyositis, 50% of systemic sclerosis, 16.7% of liver disease patients, and 20% of healthy subjects. The results indicated a character of naturally occurring antibody of antikeratin antibodies in human sera.

Prevention of progression of interstitial lung lesions by early combination therapy with corticosteroids and cyclosporine/cyclophosphamide in two patients with amyopathic dermatomyositis.

Two patients with amyopathic dermatomyositis complicated by interstitial lung lesions were effectively treated with a combination of corticosteroids and cyclosporine and/or cyclophosphamide. A 48-year-old female patient was treated with pulse methylprednisolone and cyclosporine 2 months after onset of dermal symptoms. A 45-year-old male patient was treated with oral prednisolone and pulse cyclophosphamide 2 1/2 months after onset of dermal symptoms. Early evaluation of interstitial lung lesions and early extensive therapy may improve prognosis of interstitial lung lesions in patients with amyopathic dermatomyositis.

Polymyositis associated with urinary bladder cancer: an autopsy case.

A 56-year-old man suffered from muscle weakness with elevated serum creatine kinase. Under diagnosis of polymyositis, the patient was treated with corticosteroid, methotrexate and cyclosporin A. Eleven months after the first signs of muscle weakness, the patient suffered an abrupt onset of anuria and underwent hemodialysis. The patient died of respiratory insufficiency 14 months after the first signs of muscle weakness. Autopsy findings revealed associated urinary bladder cancer with histological indications of adenosquamous cell carcinoma, liver metastasis and cancerous lymphangitis of the lung.

Remitting seronegative symmetrical synovitis with pitting edema (RS3PE) syndrome following spontaneous rupture of a gouty tophus.

A 70-year-old man with a 30-year history of gout presented with a ruptured gouty tophus over the right lateral malleolus. After the debridement of the tophus, bilateral arthralgia and pitting edema were observed in his extremities. Treatments with antibiotics and nonsteroidal antiinflammatory drugs were ineffective. However, prednisolone therapy was highly effective, and the patient's symptoms were rapidly ameliorated. Thus, we presume that rupture of a gouty tophus or its surgical treatment might contribute to the occurrence of RS3PE syndrome; however, in our case, the etiology of the syndrome remained unknown.

Varicella zoster virus meningoencephalitis accompanied by sporadic skin lesions in an older immunocompetent adult.

A previously healthy 75-year-old man complained of persistent fever, headache, nausea, mild gait disturbance, memory disorder, and sporadic vesicular skin lesions. Viral meningoencephalitis was diagnosed, based on cerebrospinal fluid (CSF) analysis. Intensive CSF analysis suggested that the patient's illness was caused by varicella zoster virus (VZV). The patient recovered completely after treatment with intravenous acyclovir. VZV infection should be considered as a possible cause of central nervous system disease, even in an immunocompetent patient. VZV reactivation was strongly suspected because of the results of anti-VZV antibody evaluations in serum and CSF, although the skin lesions were not similar to those of herpes zoster.

A case of possible Behçet's disease, preceded by recurrent knee arthropathy for 5 years, effectively treated with oral colchicine.

We report a case of possible Behçet's disease (BD) preceded by arthropathy for 5 years. A 29-year-old man experienced right knee swelling and pain since April 1999. Thorough examinations were performed, yet a diagnosis was not established. In December 2004 he experienced recurrent aphtha, following folliculitis-like skin lesions. A human leukocyte antigen examination revealed a positive B51 allele and his illness was diagnosed as possible Behçet disease. Colchicine was administered and his symptoms were ameliorated.

HLA-B52-positive vasculo-Behçet disease: usefulness of magnetic resonance angiography, ultrasound study, and computed tomographic angiography for the early evaluation of multiarterial lesions.

We report a case of HLA-B52-positive Behçet disease accompanied by multiarterial lesions. A 24-year-old woman was suffering from sporadic high fever and recurrent oral and genital ulcers, and laboratory data revealed severe inflammation. A diagnosis of Behçet disease was made. Magnetic resonance angiography, ultrasound study, and computed tomographic angiography demonstrated multiarterial lesions that had caused no symptoms. These noninvasive examinations were extremely useful in evaluating asymptomatic early vascular lesions.

[A case report of relapsing polychondritis with an auricular ulcer complicated by systemic sclerosis].

A 35-year-old male patient presented with left auricular ulcer following swelling and redness of the left auricle in the beginning of June 2001. Since 1996, he has been suffering from scleroderma at the fingers and face with Raynaud's phenomenon under a diagnosis of systemic sclerosis. On 1997, partial perforation of his nasal septum was pointed out by an otolaryngologist, which was, however, untreated. He sometimes suffered from swelling and redness in both auricles and from polyarthralgia since 1999. On June 11th, 2001, hemorrhage and pus discharge under an infection by Pseudomonas aeruginosa started on the painful ulcer in the left auricle, which were not improved by the treatment with antibiotics but improved by an administration of prednisolone at dose of 15 mg/day. Head MRI taken on October 2nd, 2001, showed a partial defect in the nasal septum with a length of 10 mm. The patient was considered as a rare case of a complication of relapsing polychondritis and systemic sclerosis.