Valvular heart disease in systemic lupus erythematosus and Jaccoud��s arthropathy

Jaccoud’s arthropathy (JA) was initially described as a secondary complication to rheumatic fever (RF). However, most recently described cases are associated with systemic lupus erythematosus (SLE). At least in RF, this articular complication has been observed to occur in association with valvular heart disease. The aim of this work is to investigate the presence of valvulopathy in patients with SLE and JA, when compared to lupus patients without such complication. Patients with diagnosis of SLE based on the American College of Rheumatology criteria were enrolled in the study and divided into two groups: with or without JA and evaluated by transthoracic echocardiography. A total of 113 patients with SLE (25 with JA and 88 without JA) were assessed, of which 108 were females and five were males. Echocardiographic changes were found in 24 patients (21.2%) out of the entire population, including valvulopathy in 17 cases (15%), pulmonary hypertension in 7 cases (6.2%) and pericardial effusion in 2 cases (1.8%). In general, echocardiographic changes were more frequently seen in the JA group in comparison with the control group (p = 0.04). Additionally, in the JA group, valvulopathy was found in nine cases (36%) against eight cases (9%) in the control group (p = 0.001). This study reveals for the first time the association between the presence of valvular heart disease and JA in SLE patients, suggesting that the presence of JA may be a marker of such complication. Additional studies are required for clarification of the mechanisms involved in both complications.     

Koebner’s phenomenon in systemic lupus erythematosus

Koebner phenomenon is defined as a nonspecific skin stimulus eliciting a disease skin reaction. The nature of the skin trauma varies greatly and includes areas of thermal injury, excoriations, surgical incisions, and scars. We report a patient with recent onset of systemic lupus erythematosus who developed Herpes zoster on immunosuppressant medication. Two weeks after resolution of the vesicles, the patient presented with new ulcerative reddish lesions over the herpes zoster scare and worsening of her malar rash without evidence of worsening of any other organ. Koebner phenomenon was suspected. We review the literature on Koebner phenomenon in SLE.     

Coexistence of Crohn’s disease in a patient with systemic lupus erythematosus

The concurrence of inflammatory bowel disease with systemic lupus erythematosus (SLE) is rare. The concomitant diagnosis of Crohn’s disease and SLE is even more rare. The patient, a 40-year-old woman, was admitted to our hospital because of relapsing episodes of abdominal pain, diarrheas upper and lower extremities arthralgias, Raynaud’s phenomenon with positive antinuclear antibodies, and fever for the last 2 years. The patient was diagnosed elsewhere with SLE and treated with hydroxychloroquine. Her medical history also included tonsillectomy and total hip replacement after a car accident. Family history was unremarkable. Physical examination was unremarkable except of very mild pain at lower left abdominal quadrant. Laboratory tests showed erythrocyte sedimentation rate at 32 mm/h, C-reactive protein at 36 mg/dl, positive rheumatoid factor, and increased C3, C4, positive antinuclear antibodies with the presence of anti-Sm and anti-RNP antibodies. Ileocolonoscopy revealed colonic inflammation with ulcers and pseudopolyps. Subsequent biopsies were diagnostic of Crohn’s disease. Patient was diagnosed with Crohn’s colitis concomitant to systemic lupus erythematosus and was started on therapy with azathioprine 2 mg/Kg, methylprednisolone 16 mg/d with slow tapering, mesalazine 1.5 g/day, and hydroxychloroquine. Patient is in excellent health status on the six-month follow-up.     

Rowell’s syndrome: presenting features of systemic lupus erythematosus

A subset of patients with lupus erythematosus develops erythema multiforme-like skin lesions with speckled pattern of antinuclear antibodies, positive rheumatoid factor, anti-Ro (SS-A), and anti-La antibodies (SS-B), which known as Rowell’s syndrome. We report an adolescent boy presented with erythema multiforme-like skin lesions and pericardial effusion; he fulfilled criteria of both Rowell’s syndrome and systemic lupus erythematosus (SLE). Such initial presentation of SLE is rarely reported in literature.     

Klinefelter’s syndrome associated with systemic lupus erythematosus and autoimmune hepatitis

Klinefelter's syndrome (KFS) tends to be associated with immunological disorders. We describe a 37-year-old man who presented signs of testicular atrophy and decreased body hair. He showed pancytopenia and elevated levels of liver enzymes. Chromosome analysis revealed 47XXY karyotype; therefore, he was diagnosed with KFS, with systemic lupus erythematosus and autoimmune hepatitis. Treatment with a high dose of methylprednisolone and methyltestosterone improved thrombocytopenia and symptoms, suggesting that methyltestosterone may have a clinical benefit in the treatment of KFS with a low level of testosterone accompanying immunological disorders.     

Health care in systemic lupus erythematosus (SLE): the patient’s perspective

In order to provide more patient-centered care for patients suffering from systemic lupus erythematosus (SLE), we studied their current satisfaction and preferences regarding future health care delivery. We sent questionnaires to all SLE patients visiting the rheumatology outpatient clinic in Leiden, the Netherlands. The questionnaire comprised three topics: (a) health care needs using a modified version of SLE Needs Questionnaire (range 0–38), (b) satisfaction with care per provider (visual analogue scale, range 0 (not at all)–100 (very satisfied)), and (c) preferences for future healthcare (four items). One hundred and two patients (63 % response) reported an average of 16 (±6) health care needs, with all patients reporting a need in the physical domain. More needs were significantly associated with worse physical functioning and a higher educational level. The average satisfaction score was 73 (±19) with a lower overall satisfaction score being associated with younger age and an educational level higher or lower than average. Regarding preferences for future health care delivery, 75 % of patients showed interest in a yearly standardized medical assessment, 57 % in regular, specialized nurse contacts using internet, 50 % in a yearly inventory on the need for self-management support, and 36 % in an education course. The association of age, education level and physical functioning with health care needs, and/or satisfaction suggest that the delivery of care should be better tailored to the needs of subgroups of patients.     

Multiple dermatofibromas in a patient with systemic lupus erythematosus and Sjögren’s syndrome

We report a case of multiple dermatofibromas in a patient with systemic lupus erythematosus (SLE) and Sjögren’s syndrome. He was treated with prednisolone, diaphenylsulfone, and cyclosporine for SLE. He noticed two brown nodules on his right lower leg 3 years after the first consultation. Subsequently, six nodules developed within 6 months, and 2 more nodules after 10 months. Histopathological examination of a nodule on his left hand showed fibrotic proliferation with a storiform pattern in the whole dermis, but neither necrosis nor mitosis was observed. From these findings, a diagnosis of multiple dermatofibromas was made. As these tumors appeared during the remission stage of SLE, they might have been under immunosuppressive conditions caused by immunosuppressing agents rather than collagen disease itself.     

Is there a difference in systemic lupus erythematosus with and without Raynaud’s phenomenon?

The aim of this study was to assess the association between Raynaud’s phenomenon (RP) and specific capillaroscopic findings in patients with SLE and particular clinical manifestations of the disease. A total of 79 patients with SLE were included in the study: 44 of them (43 women) with RP and 35 (32 women) age-, sex-, and disease-duration-matched patients with SLE without RP. Demographic variables, clinical manifestations, laboratory and nailfold capillaroscopy findings were compared between the two groups. Central nervous systemic involvements (P = 0.0038) and peripheral neuropathy (P = 0.0336) were significantly more common in SLE patients with RP, while secondary Sjögren’s syndrome (P = 0.0363) was more common in SLE patients without RP. RP occurred in 52 % of patients before SLE onset while 48 % of patients developed RP after they had been diagnosed with SLE. Arthritis/arthralgia (P = 0.0073) was significantly more common in patients who had been diagnosed with RP before the onset of SLE, while mucosal ulcers were more common in patients who contracted RP after the onset of SLE (P = 0.0258). Enlarged capillaries (P = 0.0482), presence of avascular areas (P = 0.0476), capillary hemorrhages (P = 0.0482), and granular blood flow (P = 0.0482) were more common in patients with SLE who also suffered from RP, than in patients with SLE without RP. The frequency of normal (63.6 vs. 82.9 %, P = 0.100) and nonspecific (25 vs. 17.1 %, P = 0.5696) capillaroscopy findings were similar in either groups. Scleroderma-like pattern of capillaroscopy finding was only found in patients with RP [(11.4 %), P = 0.0482]. RP in our patients with SLE was associated with specific clinical manifestations, indicating that prognostic relevance of RP in SLE should be evaluated.     

High risk of tuberculosis in systemic lupus erythematosus?

The incidence and severity of tuberculosis (TB) in patients with systemic lupus erythematosus (SLE) varies greatly among different series. In addition, prospective data are scarce. The aim of this study is to analyse the frequency and severity of TB in our cohort of lupus patients. We analysed data from a prospective database of a single center cohort of 232 patients with SLE (ACR criteria). Prophylaxis with isoniazid was not regularly administered. We identified all cases of TB diagnosed during 10 years (January 1994 to December 2003). The following variables were analysed: annual incidence of TB, location of infection and response to therapy. Data from published series reporting on the incidence of TB among SLE patients were extracted. Three patients (1.3%) suffered clinically manifest TB in 1603 patient-years of follow-up, resulting in an incidence of 187 cases/100,000 patient-years (95% CI 39-547). The pooled annual incidence of TB infection in our area during this period was 30/100,000 individuals. We recorded two cases of pulmonary TB and one case of tuberculous pleurisy. All patients had good response to therapy. The annual incidence of TB among SLE patients in other series, most of them from developing countries, varied between 150/100,000 patients in Turkey and 2450/100,000 patients in India. Of note, high prevalence of extrapulmonary forms as well as elevated TB-associated mortality was reported in most series. TB was more frequent in SLE patients than expected in the general population. We did not see any cases of disseminated infection and all patients had good response to treatment. Our data compare favourably in terms of incidence, severity and outcome with those from highly endemic areas.     

Central nervous system involvement in systemic lupus erythematosus.

This paper deals with the clinical, immunological and pathological data of 5 eases of systemic lupus erythematosus (SLE). Each of the five cases has typical SLE damages on the skin and multiple organs. Among     

Jaccoud’s arthropathy and psoriatic arthritis, a rare association

Jaccoud’s arthropathy is a syndrome of chronic progressive painless deformity of the hands and feet with surprisingly well-preserved functions. Although it is most frequently seen in patients with systemic lupus erythematosus, an association with other diseases has occasionally been described. A patient with long-standing psoriasis arthritis is presented in this report who developed an arthropathy with the clinical and radiologic finding indistinguishable from that of the Jaccoud’s arthropathy. In patients with long-standing psoriasis arthritis, the disease may be associated with Jaccoud’s arthropathy.     

Folic acid in systemic lupus erythematosus – a new aspect

Clinical Rheumatology -     

Health disparities in systemic lupus erythematosus—a narrative review

Clinical Rheumatology - To describe root causes of health disparities by reviewing studies on incidence and outcomes of systemic lupus erythematosus (SLE) related to ethnic, race, gender, or...     

Caution on the masqueraders of Good’s syndrome on thymoma with systemic lupus erythematosus

Clinical Rheumatology -     

Neglected spontaneous rupture of the Achilles’ tendon in patients with systemic lupus erythematosus

Abstract

Spontaneous Achilles’ tendon rupture associated with systemic lupus erythematosus (SLE) is rare complication in literature review. We encountered two patients with neglected spontaneous ruptures of Achilles’ tendons who had been on corticosteroid therapy to treat SLE. The ages of these patients were 43 and 49 years, and both were women. One of them was a case of bilateral Achilles’ tendons rerupture. Achilles’ tendons of both patients were reconstructed by surgery because of delay in their diagnosis. Histological section of the both ruptured Achilles’ tendon revealed fibrotic scar tissue and little existence of inflammatory change. We concluded that careful diagnosis, surgical suture, and careful treatment after operation are necessary for Achilles’ tendon rupture in those patients with SLE.     

Neglected spontaneous rupture of the Achilles’ tendon in patients with systemic lupus erythematosus

Spontaneous Achilles' tendon rupture associated with systemic lupus erythematosus (SLE) is rare complication in literature review. We encountered two patients with neglected spontaneous ruptures of Achilles' tendons who had been on corticosteroid therapy to treat SLE. The ages of these patients were 43 and 49 years, and both were women. One of them was a case of bilateral Achilles' tendons rerupture. Achilles' tendons of both patients were reconstructed by surgery because of delay in their diagnosis. Histological section of the both ruptured Achilles' tendon revealed fibrotic scar tissue and little existence of inflammatory change. We concluded that careful diagnosis, surgical suture, and careful treatment after operation are necessary for Achilles' tendon rupture in those patients with SLE.