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.     

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.     

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

Abstract

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.     

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

Clinical Rheumatology -     

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

Clinical Rheumatology -     

Dubin-Johnson syndrome with systemic lupus erythematosus: a case report

Introduction Dubin-Johnson syndrome (DJS) is a rare recessively inherited conjugated hyperbilirubi- nemia caused by deficiency of the canalicular multi-drug resistance/multi-specific organic anionic transporter protein (MDR2/cMOAT). Thus bilirubin is conjugated but inefficiently secreted into bile, which results in accumulation of conjugated and, to some extent, unconjugated bilirubin in blood, leading to hyperbilirubinemia and bilirubinuria. But, the results of liver function tests are no…     

A case of overlap syndrome with rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis and secondary sjögren’s syndrome

We report an unusual case of overlap syndrome that had the coexistence of five autoimmune diseases. A 45-year-old woman initially developed seropositive erosive rheumatoid arthritis (RA) 11 years ago. She then developed progressive systemic sclerosis (PSS) (including pulmonary hypertension, esophageal dysfunction, cardiac involvement and sclerodactilitis), systemic lupus erythematosus (SLE) (including photosensitivity, nephritis, leukopenia, lymphopenia, thrombocytopenia and Coombs positive hemolytic anemia and positive anti-dsDNA), and secondary Sjögren’s syndrome (SSS) in the last 7 years before she was admitted to our clinic. The patient fulfilled classification criteria for RA, SLE, PSS and SSS, as determined by American College of Rheumatology. Hypothyroidism with positive autoantibodies due to Hashimoto’s thyroiditis, the beginning of which could not be defined, was coexistent with this overlap syndrome. In the literature, although overlap syndromes in different combinations were reported, we very rarely observed a complex case like this patient. In our opinion, this is the first well-documented case of RA, PSS, SLE, SSS and Hashimoto’s thyroiditis existing together in the same patient. Although immunosuppressive therapy was administered, the disease rapidly deteriorated and the patient died.     

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.     

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.     

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

Abstract

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.     

Kikuchi-Fujimoto’s disease associated with systemic lupus erythematosus: case report and review of the literature

Kikuchi-Fujimotos disease (KFD) or histiocytic necrotising lymphadenitis is a benign and self-limited disease, of unknown aetiology, which affects mainly young women. It presents with localised lymphadenopathy, predominantly in the cervical region, accompanied by fever and leukopenia in up to 50% of the cases. KFD has been rarely described in association with systemic lupus erythematosus (SLE), and its diagnosis can precede, postdate or coincide with the diagnosis of SLE. We present a patient with the diagnosis of SLE characterised by arthritis, leukopenia, malar rash, photosensitivity and positive ANA, besides cervical lymphadenopathy whose biopsy was compatible with KFD, which improved after using prednisone. Although the presence of lymphadenopathy is not uncommon in SLE patients, particularly in the phases of disease activity, the concomitance with KFD has rarely been reported in the literature. Its recognition is necessary because one can avoid laborious investigation for infectious and lymphoproliferative diseases.     

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.     

Macrophage activation syndrome in juvenile systemic lupus erythematosus: an under-recognized complication?

Macrophage activation syndrome (MAS) is a life-threatening complication of rheumatic diseases that is thought to be caused by the activation and uncontrolled proliferation of T lymphocytes and macrophages, leading to widespread haemophagocytosis and cytokine overproduction. It is seen most commonly in systemic juvenile idiopathic arthritis, but is increasingly recognized also in juvenile systemic lupus erythematosus (J-SLE). Recognition of MAS in patients with J-SLE is often challenging because it may mimic the clinical features of the underlying disease or be confused with an infectious complication. This review summarizes the characteristics of patients with J-SLE-associated MAS reported in the literature or seen by the authors and analyses the distinctive clinical, diagnostic and therapeutic issues that the occurrence of MAS may raise in patients with J-SLE.     

A case of a flare-up of systemic lupus erythematosus and Sjögren’s syndrome manifesting with cholecystitis secondary to pregnancy

Abstract

a 30-year- old had a 2 year history of systemic lupus erythematosus (SLE) was transferred to our hospital by her gynecologist because of a low-grade fever and dyspnea after delivery. Since a blood culture performed upon admission showed the presence of Bacteroides caccae, she was diagnosed with sepsis. Antibiotic therapy did not resolve her symptoms and she further developed an acute abdomen with marked cholecystitis. This was believed to be due to a flare-up of SLE after the pregnancy, overlapped with Sjögren’s syndrome which was evidenced by sialaden biopsy. She showed a good response to combination therapy with steroids and antibiotics.