1/24. Hypokalaemic paralysis revealing Sjogren syndrome in an elderly man.A 73 year old white man presented with life threatening hypokalaemic paralysis requiring admission to an intensive care unit. Biochemical investigations showed severe hypokalaemia with hyperchloraemic metabolic acidosis, a spot urine pH of 6.5, and a positive urinary anion gap, establishing the diagnosis of distal renal tubular acidosis. Autoimmune tests revealed Sjogren syndrome as the underlying cause of the distal renal tubular acidosis. Full recovery followed potassium and alkali replacement. This dramatic presentation of Sjogren syndrome has not previously been reported in an elderly man.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
2/24. Annular erythema of sjogren's syndrome in a white woman.Annular erythema of sjogren's syndrome (AE-SS) is believed to be the Asian counterpart of subacute cutaneous lupus erythematosus (SCLE) in white persons. We report the second case of a white person with AE-SS, diagnosed by clinical and serologic findings, as well as the absence of histologic criteria for SCLE. The diagnosis of AE-SS was established by symptoms of xerophthalmia and xerostomia, as well as by examination of skin and salivary gland biopsy specimens. Evaluation showed the presence of anti-Ro(SS-A) and anti-La(SS-B) autoantibody, with the presence of anti-Ro(SS-A) antibody against the 60-kd, but not the 52-kd, epitope, a pattern frequently seen in both the Asians with AE-SS and in white patients with SCLE. Both skin and sicca symptoms were alleviated with combination antimalarial therapy, which included hydroxychloroquine and quinacrine. This case demonstrates that AE-SS can occur in white patients and that the autoantibody profile is similar to that described in Asians with this disease.- - - - - - - - - - ranking = 8keywords = white (Clic here for more details about this article) |
3/24. Bamboo node: primary vocal fold lesion as evidence of autoimmune disease.Descriptions of vocal fold lesions related to autoimmune diseases are rare in the literature, and focus mainly on rheumatoid nodules. This is the first report in which autoimmune diseases were promptly suspected by the observation of a unique white transverse submucosal lesion in the vocal fold during clinical examination. This lesion, reported only in autoimmune disease, has been called the bamboo node and its features are different from those of rheumatoid nodules. We report here on two patients who did not have a diagnosis of systemic disease before investigation of their main complaint of hoarseness. At the patients' first visit, vocal fold bamboo nodes were seen in the vocal fold and the otolaryngologist suspected the presence of an autoimmune disease. We requested clinical investigation to clarify our suspicion that there was an underlying systemic disease. After the investigation, both patients were shown to have autoimmune disease, sjogren's syndrome and systemic lupus erythematous, respectively. This paper emphasizes the important role of the otolaryngologist in the detection of these unique lesions in the vocal folds through the conventional laryngeal methods. These methods consisted of direct observation with a rigid laryngeal endoscope and investigation of the patient's distinctive vibratory pattern by means of laryngeal stroboscopy. The method of treatment we used to obtain the best outcome in terms of voice improvement is also discussed.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
4/24. Co-occurrence of spondyloarthropathy and connective tissue disease: development of sjogren's syndrome and mixed connective tissue disease (MCTD) in a patient with ankylosing spondylitis.spondylarthropathies (SpA) and connective tissue diseases (CTD) are clinically distinct entities which, at first glance, seem to have little in common. However, a link between SpA and CTD has recently been suggested by a study in which a higher prevalence of sjogren's syndrome (SS) and sicca symptoms was reported in patients with ankylosing spondylitis (AS) and undifferentiated SpA (1). Another link between SpA and CTD is a possible side effect of a DMARD widely used to treat SpA: sulfasalazine (SAS). SAS was reported to induce antinuclear antibodies (ANA) and systemic lupus erythematosus (SLE)-like syndromes such as drug-induced lupus. This report describes a 54-year-old white male, HLA B27-positive AS patient with some syndesmophytes who, after 15 years of disease, developed SS with salivary gland involvement, Raynaud's syndrome and anti-Ro antibodies. Then, 20 years after the onset of AS, he became acutely ill, suffering severe myositis and myocarditis along with swollen hands and highly elevated autoantibody titers recognizing UIRNP; his condition was interpreted as mixed connective tissue disease (MCTD). The patient had been treated with SAS and azathioprine (AZA) alone several times during the last years because he had not tolerated other DMARDs. A combination of both drugs had been prescribed 3 weeks before a severe flair because of progredient high disease activity with painful peripheral arthritis of the MCP and PIP joints which, however, had not shown radiographic erosions. We describe the rare development of MCTD in an AS patient and report, for the first time, the onset of MCTD potentially triggered by sulfasalazine.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
5/24. Pure red cell aplasia in a sjogren's syndrome/lupus erythematosus overlap patient.Hematological complications of systemic lupus erythematosus usually include anemia of chronic disease and peripheral destruction of blood cells. We describe the case of a young woman with sjogren's syndrome'lupus erythematosus overlap, mother of infant with congenital heart block, complicated by pure red cell aplasia. The patient was asymptomatic until the onset of severe anemia. A serum inhibitor of erythropoiesis was detected before the onset of immunosuppression. bone marrow examination showed a low CD4:CD8 ratio, an immune defect possibly linked with the unrestrained production of antibodies against erythroid progenitor cells.- - - - - - - - - - ranking = 3.5530223147185keywords = blood cell (Clic here for more details about this article) |
6/24. An autopsied case of sjogren's syndrome with massive necrotic and demyelinating lesions of the cerebellar white matter.A 69-year-old woman developed subacute cerebellar ataxia and tremors in all four limbs in April 1996. Laboratory examination showed elevated antibodies against Ro and La. head magnetic resonance imaging showed T(2) high-intensity lesions in the cerebellar white matter bilaterally and later in the pons. In April 2000, she died of multiple organ failure with incidental colon cancer. The autopsy showed atrophic parotid glands with an accumulation of lymphocytes around the ducts, confirming the diagnosis of sjogren's syndrome histopathologically. The neuropathological examination revealed severe necrotic lesions in the cerebellar white matter bilaterally with several foci of perivenous demyelination in the periphery of the lesions and similar demyelinated areas in the pons. immunohistochemistry with anti-jc virus antibody demonstrated no positive inclusions. A single focus of granulomatous arteritis was observed in one subarachnoid artery. The combination of sjogren's syndrome, granulomatous angitis, and foci of perivenous demyelination suggests that an autoimmune mechanism played an important role in causing the necrotic lesions in the cerebellar white matter in this case.- - - - - - - - - - ranking = 7keywords = white (Clic here for more details about this article) |
7/24. Lupus erythematosus with leflunomide: induction or reactivation?BACKGROUND: Leflunomide is a new oral disease modifying antirheumatic drug with a good safety profile. CASE REPORT: The first case of lupus erythematosus in a 58 year old white woman after administration of leflunomide for primary sjogren's syndrome is reported. The relationship between induced lupus and leflunomide was confirmed by the resolution of the skin rash when the drug was stopped and its recurrence when it was reintroduced following a dose-response effect. DISCUSSION: Peripheral blood cells from this patient, from 15 patients with rheumatoid arthritis, and from healthy controls were used in a bioassay, which suggested that leflunomide affected the Th1/Th2 balance. Such a side effect might be related, in part, to the anti-tumour necrosis factor alpha activity of leflunomide.- - - - - - - - - - ranking = 4.5530223147185keywords = blood cell, white (Clic here for more details about this article) |
8/24. Reversible cortical lesions in primary sjogren's syndrome presenting with meningoencephalitis as an initial manifestation.We report a 50-year-old woman with primary sjogren's syndrome (SjS) who initially showed forgetfulness, and later developed disturbance of consciousness. In addition to aseptic meningoencephalitis revealed by cerebrospinal fluid examination and magnetic resonance imaging (MRI), the presence of serum anti-SS-A and anti-SS-B antibodies and inflammatory findings in lip biopsy indicated primary SjS. Fluid attenuated inversion recovery (FLAIR) of MRI revealed well defined small, high signal intensity areas in the cortex involving the subcortical white matter. Corticosteroid therapy resulted in rapid and nearly complete resolution of the cortical lesions with marked improvement of the clinical manifestations. memory disturbance is a rare initial manifestation in meningoencephalitis associated with SjS. Our patient with SjS showed inflammatory cortical lesions on MRI, which were reversed by corticosteroid therapy.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
9/24. Primary sjogren's syndrome preceding the presentation of systemic lupus erythematosus as a benign intracranial hypertension syndrome.The case is reported of a 41 year old white woman who developed systemic lupus erythematosus (SLE) seven years after primary sjogren's syndrome and four years after the association of sjogren's syndrome with Jaccoud's arthritis. The SLE was detected by a benign intracranial hypertension, which is a rare neuro-ophthalmic presentation of lupus. No associated conditions linked to benign intracranial hypertension syndrome were seen in this patient and the hypertension syndrome recurred one year later.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
10/24. Leukoencephalopathy as a rare complication of hepatitis c infection.We report the case of a 64-year-old female patient with hepatitis c infection (HCV), who developed Sjogren's disease and sensory peripheral neuropathy. Clinical conditions worsened over three years with central nervous system involvement characterised by transient third cranial nerve paresis and mild selective impairment of attention and memory. brain magnetic resonance imaging showed diffuse periventricular and lobar white matter hyperintensity. Laboratory findings included mixed cryoglobulinaemia (type II), cryocrit 1.47%, low serum levels of complement c4 and high levels of rheumatoid factor, HCV 1b genotype, high HCV mRNA levels in serum and cerebrospinal fluid. skin biopsy showed evidence of vasculitis. After one year of plasmapheresis, immunosuppressant therapy and occasional corticosteroid treatment, neurological symptoms improved, skin biopsy changed and inflammation parameters normalised, suggesting that neurological symptoms might be related to the high levels of mixed cryoglobulins.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
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