Clinical Rheumatology

Baerlecken, Niklas; Schmidt, Reinhold

doi: 10.1007/s10067-010-1412-8pmid: 20213127

We report about a 39-year-old female patient with severe essential mixed cryoglobulinemia of type III with leukocytoclastic vasculitis. The patient was admitted into our hospital with mesenteric lymphangiitis, which caused enteral perforation, sepsis, and pneumonia. Cryoglobulins, cryocrit, Ig-titers, and biopsy were positive for mixed cryoglobulinemia type III. We detected no signs of hepatitis C, B, or any other infectious disease. At first, disease activity could be kept under control with high doses of glucocorticoids and multiple cyclophosphamide pulses. However, after therapy with three pulses of rituximab, steroids were stopped, and the patient has not presented any symptoms for 2 years. Therefore, we suggest that rituximab affected her disease rapidly and effectively. In conclusion, rituximab is an alternative therapy for mixed cryoglobulinemia of type III with leukocytoclastic vasculitis.

Tobón, Gabriel; Serna, Mario; Cañas, Carlos

doi: 10.1007/s10067-010-1416-4pmid: 20213126

Listeria monocytogenes infection (LMI) is a rare complication in systemic lupus erythematosus (SLE) patients, and it is associated with nonspecific clinical manifestations and is often mistaken with SLE flares. Several cases of LMI in SLE patients have been reported, with high mortality rates. This article describes five new cases of LMI in patient with SLE in a cohort of 174 patients (2.8%). All patients were women, with a mean age of 19.4 years (range, 5–29 years). Mean duration of SLE before clinical LMI was 2.8 years (range, 2–4 years). Recurrent infection was not evidenced. At the time of LMI, all patients had an inactive disease, receiving steroids and immunosuppressive treatment. Clinical picture of meningitis was present in two patients. All patients were treated with ampicillin, with resolution of clinical manifestations without sequels. In order to eliminate the intracellular forms of L. monocytogenes, trimethoprim–sulfamethoxazole was initiated, and an allergic skin reaction was presented in all but one patient. Our report highlights the unspecific clinical manifestations of LMI and these characteristics are initially challenging and may be interpreted as lupus flares. An accurate diagnosis and an early antibiotic treatment are essential to improve the outcome in these patients.

Baker, Joshua; Nazarian, Levon; Olin, Jeffrey; Pullman-Mooar, Sally

doi: 10.1007/s10067-010-1419-1pmid: 20229260

The swift and accurate diagnosis of acute arterial occlusion and limb ischemia is critical to prevent morbidity from this vascular emergency as reported by Bettman et al. (Radiology 215:101–105, 2000). There are a number of imaging modalities available to the diagnosing physician. However, the most appropriate modality may vary according to the clinical presentation. We present an atypical case of acute thrombosis of the brachial artery, which could not be diagnosed by conventional angiography, but was identified successfully with Doppler ultrasound. We also briefly discuss the issues surrounding vascular imaging in this case and overview the strengths and weaknesses of available imaging modalities.

Cazzato, Massimiliano; Neri, Rossella; Possemato, Niccolo; Puccini, Rodolfo; Bombardieri, Stefano

doi: 10.1007/s10067-010-1420-8pmid: 20225048

PFAPA is an acronym for periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis. This syndrome has been usually described in pediatric patients and it generally resolves spontaneously. The endocapillary proliferative glomerulonephritis (EPG) is a glomerular injury characterized by hypercellularity in glomerular lumen and is caused by post-infectious or autoimmune diseases. In this paper, we describe the case of a 35-year-old man affected by PFAPA and EPG. To our knowledge this association has never been reported in the literature before.

Lin, Yeong-Jang; Chen, Pei-Chih; Chen, Hung-An

doi: 10.1007/s10067-010-1421-7pmid: 20238134

Mesenteric vasculitis is one of the most devastating complications of systemic lupus erythematosus (SLE) and may produce a spectrum of complications, including ulceration, hemorrhage, bowel necrosis, perforation, serositis, and ascites. Intussusception is a process in which a segment of intestine invaginates into the adjoining intestinal lumen, causing bowel obstruction. Intussusception in association with SLE has rarely been reported. Here we report a case of SLE whose initial presentation was mesenteric vasculitis causing ileocecal intussusception.

Riksen, Niels; Gehlmann, Helmut; Brouwer, Annemarie; Deuren, Marcel

doi: 10.1007/s10067-010-1428-0pmid: 20349259

The heart is involved in up to 50% of all patients with Churg–Strauss syndrome, but vasculitis of the coronary arteries has only been rarely documented. We present a young patient with severe coronary aneurysms and stenotic lesions due to a Churg–Strauss vasculitis. Prompt therapy with prednisone and cyclophosphamide resulted in the complete resolution of all lesions.

Idan, Roni; Hajdu, Steven; Agmon-Levin, Nancy; Shoenfeld, Yehuda

doi: 10.1007/s10067-010-1437-zpmid: 20361224

Systemic lupus erythromatosus (SLE) is an autoimmune disease, which affects mainly women in the reproductive age and is influenced by hormonal changes. Therefore, hormone supplementation for patients with SLE either as contraceptives or as postmenopausal supplementation remains a controversial issue. Herein, we report a case of a 22-year-old woman with a history of ovarian agenesis, treated for several years with hormone therapy in order to reduce the risk of osteoporosis and other estrogen-deficient disorders. At the current evaluation, she met 3 of 11 diagnostic criteria for SLE along with a strong familial autoimmune predisposition. Precipitation of SLE in patients treated with hormonal therapy has been previously described. This prompted us to seek alternative drug therapies that prevent both the onset of overt SLE as well as the progression of estrogen-deficient phenomena. This unique case illustrates the dilemma of using hormone therapy in patients at risk to develop SLE and the current therapeutic alternatives.

Kuwabara, Hiroko; Fukuda, Akira; Tsuda, Yasuhiro; Shibayama, Yuro

doi: 10.1007/s10067-010-1438-ypmid: 20379839

A 73-year-old female, who suffered from rheumatoid arthritis for 10 years, developed precore mutant hepatitis B virus-associated fulminant hepatitis after 1 year of infliximab therapy and subsequent methotrexate withdrawal. We emphasize the importance of preemptive antiviral therapy before starting infliximab administration and withdrawing immunosuppressive drugs.

Saikia, Bedangshu; Aneja, Himanshu; Jain, Jyoti; Puliyel, Jacob

doi: 10.1007/s10067-010-1439-xpmid: 20373123

Juvenile dermatomyositis (JDM) is a rare childhood disease with autoimmune association. Environmental factors are known to trigger JDM in genetically susceptible individuals (Schmieder et al., Dermatol Online 6:3, 2009). Calcinosis is a well-established complication of JDM. Prevalence is higher in children (30–70%; Özkaya et al., Erciyes Med J 30(1):40–43, 2008). Hyperimmunoglobulin E syndrome is a primary immunodeficiency syndrome with multiple recurrent abscess formation and raised serum immunoglobulin E levels. We report a case of JDM with calcinosis cutis universalis with hyperimmunoglobulin E syndrome. With a previous similar case report (Min et al., Korean J Intern Med 14:95–98, 1999), this could well be a new sequence syndrome where abscesses are the trigger for the onset of JDM.

Beccastrini, Enrico; Emmi, Giacomo; Chiodi, Michela; Paolo, Camilla; Benedetta Silvestri, Elena; Massi, Daniela; Maggi, Enrico; Liotta, Francesco; Emmi, Lorenzo

doi: 10.1007/s10067-010-1449-8pmid: 20401761

Kimura’s disease is a benign chronic inflammatory disease, common in Asian males and rare in Western people. Clinically, Kimura’s disease is characterized by subcutaneous nodular lesions, usually localised in head and neck, often associated with regional lymphadenopathy. Peripheral blood eosinophilia and elevated serum IgE are often observed. We report a case of a 40-year-old Italian patient presenting with nodular subcutaneous lesions and peripheral eosinophilia. Based on clinical, histopathological and laboratory findings, a diagnosis of Kimura’s disease was made. The patient was treated with very low doses of cyclosporine A with no evidence of disease recurrence over the following 8 years. However, the discontinuation of cyclosporine A determined a relapse of the disease. The relevance of this case is due to the rarity of the disease in Italy, to its peculiar clinical presentation and, moreover, it is the first case in literature that has a good response to treatment with low doses of cyclosporine A, documented in an 8-year follow-up.