Balloon angioplasty can cause shear stress and tear of the vascular endothelium during mechanical dilatation, leading to increased inflammation and coagulation reactions of the vascular endothelium. Herein, a worst case of active rheumatoid vasculitis is described, where due to progressing ischemic necrosis of the leg, endovascular intervention was unavoidably performed in the presence of active rheumatoid vasculitis. After percutaneous balloon angioplasty, the patient developed recurrent thrombotic occlusion of the leg arteries, and finally, limb amputation resulted in despite vigorous treatment including medication, immunosuppression, catheter-directed thrombolysis, and post-thrombolysis anticoagulation. This case report indicates that endovascular intervention may be detrimental to the active rheumatoid vasculitis. Until the development of treatment guideline to prevent or control inflammatory reaction, endovascular intervention for the active rheumatoid vasculitis may not be appropriate as a first line therapy even though there is progressing ischemic necrosis.
Amputation ; Angioplasty, Balloon ; Arteries ; Dilatation ; Endothelium, Vascular ; Extremities ; Humans ; Immunosuppression ; Inflammation ; Leg ; Necrosis ; Rheumatoid Vasculitis ; Tears ; Thrombosis

Palisaded neutrophilic and granulomatous dermatitis (PNGD) is a newly defined entity that includes various clinical entities. Histopathologically, this disease is characterized by a granulomatous inflammation with or without leukocytoclastic vasculitis. PNGD shows vasculitic lesions in the early stage, with palisaded granulomatous lesions and dermal fibrosis with minimal leukocytoclastic debris appearing in the late stage. It is frequently associated with rheumatoid arthritis, lupus erythematosus, and other autoimmune diseases. A 14-year old Korean girl presented with multiple erythematous nodules and plaques on both elbows and knees that were present for 2 years prior to the initial visit. Clinically, she had multiple arthritis with morning stiffness and decreased C3 levels, as well as positive results for antinuclear antibodies, the lupus anticoagulant test, and anti-beta 2 glycoprotein I (IgG). Skin biopsy findings from the skin lesion indicated that the foci of degenerated collagens were palisaded with histiocytes throughout the dermis and neutrophils with leukocytoclasia infiltrated with fibrin, with mucin at the center of the lesion. Based on the SLICC 2012 criteria, we diagnosed her condition as PNGD in systemic lupus erythematosus. She was treated with hydroxychloroquine and topical application of 0.1% tacrolimus ointment, which resulted in the remarkable flattening of the skin lesions after 6 months of follow-up. Herein, we report a case of PNGD that may have been an indicator of systemic lupus erythematosus.
Antibodies, Antinuclear ; Arthritis ; Arthritis, Rheumatoid ; Autoimmune Diseases ; Biopsy ; Collagen ; Dermatitis* ; Dermis ; Elbow ; Female ; Fibrin ; Fibrosis ; Follow-Up Studies ; Glycoproteins ; Histiocytes ; Humans ; Hydroxychloroquine ; Inflammation ; Knee ; Lupus Coagulation Inhibitor ; Lupus Erythematosus, Systemic ; Mucins ; Neutrophils* ; Skin* ; Tacrolimus ; Vasculitis

BACKGROUND AND OBJECTIVES: Behçet's disease (BD) is a systemic vasculitis that is characterized by genital, oral, or skin lesions, uveitis, and vascular complications. Studies have shown that increased arterial stiffness is common in systemic immune and inflammatory diseases such as rheumatoid arthritis and systemic lupus erythematosus. However, current research has not yet determined whether patients with BD have increased arterial stiffness. This meta-analysis compares arterial stiffness parameters in subjects with a BD diagnosis to normal subjects. SUBJECTS AND METHODS: A comprehensive search of the MEDLINE and EMBASE databases was performed from the database beginning through May 2016. Observation studies were included in this analysis if they assessed the association between BD and arterial stiffness in adult subjects. BD patients met the International Study Group criteria for a diagnosis of Behçet's disease. Aortic stiffness was assessed using carotid-femoral pulse wave velocity (PWV) measurements as an indicator. Pooled mean difference (MD) of PWV and 95% confidence intervals (CI) were calculated using a random-effect, generic inverse variance meta-analysis. The between-study heterogeneity of effect-size was quantified using the Q statistic and I². RESULTS: Data were extracted from four observational studies that included 303 subjects. PWV is significantly higher in patients with Behçet's disease compared with controls (MD=0.74;95%, CI: 0.28-1.20, p=0.002, I²=63%). CONCLUSION: In this meta-analysis, we observed that PWV, an ideal indicator of arterial stiffness, is increased in patients with Behçet's disease compared with the controls. Prospective studies in a large population should be done to determine the pathophysiological and prognostic implications of increased arterial stiffness in BD.
Adult ; Arthritis, Rheumatoid ; Diagnosis ; Humans ; Lupus Erythematosus, Systemic ; Population Characteristics ; Prospective Studies ; Pulse Wave Analysis ; Skin ; Systemic Vasculitis ; Uveitis ; Vascular Stiffness*

Rheumatoid vasculitis is a rare, but most serious extra-articular complications of long-standing, seropositive rheumatoid arthritis (RA). Vasculitis of hepatic artery is an extremely rare but severe manifestation of rheumatoid vasculitis. A 72-year-old woman who presented with polyarthralgia for 2 months was diagnosed with early RA. Since she had manifestations of livedo reticularis, and liver dysfunction which was atypical for RA patients, a percutaneous needle liver biopsy was performed revealing arteritis of a medium-sized hepatic artery. Extensive investigations did not reveal evidences of other systemic causes such as malignancy or systemic vasculitis. The patient was diagnosed with rheumatoid vasculitis involving hepatic arteries based on Bacon and Scott criteria for rheumatoid vasculitis. With high dose corticosteroid and cyclophosphamide induction and methotrexate and tacrolimus maintenance treatment, she was successfully recovered. Association of rheumatoid vasculitis at very early stages of the disease may represent an early aggressive form of RA.
Aged ; Arteritis ; Arthralgia ; Arthritis, Rheumatoid* ; Biopsy ; Cyclophosphamide ; Female ; Hepatic Artery* ; Humans ; Livedo Reticularis ; Liver ; Liver Diseases ; Methotrexate ; Needles ; Rheumatoid Vasculitis* ; Systemic Vasculitis ; Tacrolimus ; Vasculitis

A 50-year-old woman, who had been treated for rheumatoid arthritis (RA) over a 10-year period, suddenly presented with monocular vision loss while the RA had a stable course over many years. She was diagnosed with central retinal artery occlusion (CRAO) based on ophthalmologic examinations including optical coherence tomography and fluorescein angiography. There was no evidence of atherosclerosis, infection, and malignancy that can cause CRAO. Considering the association between CRAO and other rheumatic diseases, such as systemic vasculitis and systemic lupus erythematous in previous reports, it was presumed that her RA might have contributed to the development of CRAO. Although cases of CRAO in patients with RA are extremely rare, these findings suggest that physicians need to be aware of the possibility of CRAO in patients with RA who experience decreased visual acuity.
Arthritis, Rheumatoid* ; Atherosclerosis ; Female ; Fluorescein Angiography ; Humans ; Middle Aged ; Retinal Artery Occlusion* ; Retinal Artery* ; Rheumatic Diseases ; Systemic Vasculitis ; Tomography, Optical Coherence ; Vision, Monocular ; Visual Acuity

BACKGROUND: An association between silica exposure and autoimmune diseases including rheumatoid arthritis, systemic sclerosis, systemic lupus erythematosus, and anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis has been made. CASE PRESENTATION: A 56-year-old male presented with silicosis and had an occupational history of precious metal processing for 30 years and a 30 pack-year smoking history. The patient was diagnosed with pneumoconiosis and received compensation. No other complications were reported for pneumoconiosis. The patient suddenly presented with a non-specific headache for several days and microscopic hematuria was identified upon examination in the outpatient clinic. Following several weeks, the patient presented with aggravated dyspnea and hemoptysis, and his Modification of Diet in Renal Disease estimated glomerular filtration rate indicated acute kidney injury. Diagnostic analysis revealed perinuclear ANCA-associated microscopic polyangiitis (p-ANCA-associated MPA). CONCLUSION: Exposure to silica dust was likely one of the cause of p-ANCA-associated MPA. Possible pathogenic mechanisms of autoimmune diseases in silicotics and emphasis of the necessity for early diagnosis are discussed.
Acute Kidney Injury ; Ambulatory Care Facilities ; Arthritis, Rheumatoid ; Autoimmune Diseases ; Compensation and Redress ; Cytoplasm* ; Diagnosis* ; Diet ; Dust ; Dyspnea ; Early Diagnosis ; Glomerular Filtration Rate ; Headache ; Hematuria ; Hemoptysis ; Humans ; Lupus Erythematosus, Systemic ; Male ; Microscopic Polyangiitis* ; Middle Aged ; Occupational Exposure ; Pneumoconiosis ; Scleroderma, Systemic ; Silicon Dioxide ; Silicosis ; Smoke ; Smoking ; Vasculitis

Wegener's granulomatosis (WG) is highly correlated with cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCA). Patients with rheumatoid arthritis (RA) rarely contract WG. Although several concurrent cases have been reported overseas, there are no known cases in Korea. Here we report a unique case of RA with atypical WG testing positive for perinuclear ANCA (p-ANCA) and negative for anti-myeloperoxidase (MPO) antibodies. The 62-yr-old female patient presented with multiple joint pain and showed typical blood test results for RA, i.e., an elevated erythrocyte sedimentation rate and C-reactive protein concentration, and positive for rheumatoid factor and anti-cyclic citrullinated peptide antibodies. RA was clear based on a total score of 10 when applying the classification criteria developed by the American College of Rheumatology/European League Against Rheumatism (2010). In an autoimmune target test, speckled and skeleton patterns were observed. In an ANCA test, p-ANCA was observed (titer, 1:2,560), and tests for anti-proteinase 3 (PR3) and anti-MPO antibodies were negative. After admission, multiple nodules were detected on a chest X-ray and a computed tomography scan. We suspected that she had rheumatic nodules or vasculitis and performed an open lung biopsy. We detected necrotic granulomatous vasculitis, classified as WG, thus leading to WG diagnosis. In conclusion, WG was diagnosed in an RA patient who was negative for c-ANCA (negative PR3) and positive for p-ANCA (negative anti-MPO), and this peculiar finding is likely to improve diagnosis in cases of RA with atypical WG.
Antibodies ; Antibodies, Antineutrophil Cytoplasmic ; Arthralgia ; Arthritis, Rheumatoid* ; Biopsy ; Blood Sedimentation ; C-Reactive Protein ; Classification ; Cytoplasm ; Diagnosis ; Female ; Hematologic Tests ; Humans ; Korea ; Lung ; Rheumatic Diseases ; Rheumatic Nodule ; Rheumatoid Factor ; Skeleton ; Thorax ; Vasculitis ; Wegener Granulomatosis*

Behcet's disease (BD) is a systemic vasculitis, while myelodysplastic syndrome (MDS) is a heterogeneous group of clonal hematologic disorders characterized by ineffective hematopoiesis. Some studies suggest a relationship between MDS and BD, especially intestinal BD, and trisomy 8 seems to play an important role in both diseases. There are several reports on patients with BD comorbid with MDS involving trisomy 8 that frequently have intestinal lesions refractory to conventional medical therapies. Tumor necrosis factor (TNF)-alpha is strongly involved in the pathophysiology of several autoimmune diseases such as rheumatoid arthritis, inflammatory bowel disease, and BD. In addition, TNF-alpha plays an important role in the pathophysiology of MDS by inhibiting normal hematopoiesis and inducing the programmed cell death of normal total bone marrow cells and normal CD34+ cells. Recent clinical reports demonstrate the favorable effect of TNF-alpha antagonists in patients with refractory intestinal BD and in those with MDS. We present the case of a patient with intestinal BD and MDS involving trisomy 8 who was successfully treated with adalimumab.
Adalimumab ; Arthritis, Rheumatoid ; Autoimmune Diseases ; Behcet Syndrome ; Bone Marrow Cells ; Cell Death ; Hematopoiesis ; Humans ; Inflammatory Bowel Diseases ; Myelodysplastic Syndromes* ; Systemic Vasculitis ; Trisomy* ; Tumor Necrosis Factor-alpha

Churg-Strauss syndrome (CSS) is a necrotizing vasculitis with extra-, peri-vascular eosinophilic infiltration. Chronic symmetric polyarthritis with the presence of rheumatoid factor (RF) and anti-cyclic citrullinated peptide antibody are the mainstay of rheumatoid arthritis (RA) diagnosis. Mononeuritis multiplex is a peripheral neuropathy involving more than 2 separate nerve areas. A 62-year-old male patient was referred for left foot drop and polyarthritis of both hands and feet for 4 months. During evaluation, mononeuritis multiplex was detected on nerve conduction study and electromyography tests: vasculitis with neutrophil, eosinophil, and lymphocyte infiltration on peroneal nerve biopsy. A positive response to methacholin and bronchodilator was observed on the pulmonary function test. Radiologic tests showed peri-articular soft tissue swelling and osteopenia on both hand and foot. Marked peripheral eosinophilia, high RF, and positive perinuclear anti-neutrophil cytoplasmic antibody were detected on blood tests. Here, we report on a patient with overlap syndrome of CSS and RA with review of the relevant literature, from which a few references to overlap syndrome of CSS and RA were available.
Antibodies, Antineutrophil Cytoplasmic ; Arthritis ; Arthritis, Rheumatoid* ; Biopsy ; Bone Diseases, Metabolic ; Churg-Strauss Syndrome* ; Diagnosis ; Electromyography ; Eosinophilia ; Eosinophils ; Foot ; Hand ; Hematologic Tests ; Humans ; Lymphocytes ; Male ; Middle Aged ; Mononeuropathies ; Neural Conduction ; Neutrophils ; Peripheral Nervous System Diseases ; Peroneal Nerve ; Respiratory Function Tests ; Rheumatoid Factor ; Vasculitis

Sjogren's syndrome is a chronic systemic autoimmune disease characterized by lymphocytic infiltration of the exocrine glands resulting in a dry mouth and eyes. The disease can present either alone or in association with other autoimmune diseases such as rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis, and inflammatory myopathy. In addition to symptoms caused by exocrinopathy, about half of patients present with extraglandular (systemic) manifestations including arthritis, Raynaud's phenomenon, lymphadenopathy, vasculitis, peripheral neuropathy, and interstitial nephritis. Patients often suffer from severe fatigue, myalgia, and dryness, which lead to a poor quality of life. Physicians should be aware of the increased risk of lymphoma development in patients with Sjogren's syndrome. Currently, there are no drugs available that are able to improve the natural course of disease, making symptom relief the primary goal of therapy. Currently, pilocarpine is the only drug clinically proven for the treatment of dry eyes and mouth in patients with Sjogren's syndrome.
Arthritis ; Arthritis, Rheumatoid ; Autoimmune Diseases ; Exocrine Glands ; Fatigue ; Humans ; Lupus Erythematosus, Systemic ; Lymphatic Diseases ; Lymphoma ; Mouth ; Myalgia ; Myositis ; Nephritis, Interstitial ; Peripheral Nervous System Diseases ; Pilocarpine ; Quality of Life ; Scleroderma, Systemic ; Sjogren's Syndrome* ; Vasculitis