A Case of Type 1 Renal Tubular Acidosis and Osteomalacia in a Patient with Sjogren's Syndrome

Sjogren's syndrome is a chronic autoimmune disease characterized by decreased function of the lacrimal and salivary glands. In addition, many other organs can be involved in patients with Sjogren's syndrome. Overt or latent renal tubular acidosis is an uncommon extraglandular manifestation in Sjogren's syndrome, and osteomalacia is a rare complication of renal tubular acidosis. It has been rarely reported that osteomalacia is associated with distal renal tubular acidosis in patients with Sjogren's syndrome. We report a case of a 34-year-old female patient who was initially presented with muscle weakness in both lower extremities. The patient was then diagnosed with Sjogren's syndrome complicated by osteomalacia and renal tubular acidosis

A Case of Rheumatoid Arthritis in a Patient with Bruton-Type Agammaglobulinemia

Bruton-type agammaglobulinemia is primary hypogammaglobulinemia followed by severe recurrent infection, including bacterial otitis media, bronchitis, pneumonia, and meningitis. Septic arthritis is a main musculoskeletal disorder that can occur in association with Bruton-type agammaglobulinemia. But the development of rheumatoid arthritis (RA) is rarely reported in a patient with hypogammaglobulinemia. Here, we describe a case of 34-year-old male with Bruton-type agammaglobulinemia, who presented with multiple symmetric polyarthritis. He was diagnosed as having a RA according to ACR criteria. His symptoms of polyarthritis had been improved after the introduction of medications including DMARDs (disease modifying anti-rheumatic drugs). Our case suggests that RA can be developed in the setting of agammaglobulinemia, and even in this situation, anti-rheumatic agents were effective to control arthritis without complication such as severe infection.

A Case of Paraneoplastic Syndrome Associated with Pancreatic Cancer Presenting as Rheumatoid Arthritis

Paraneoplastic cancer polyarthritis is a very rare manifestation of musculoskeletal disorders associated with several solid and hematologic malignancies. We describe a 71-year-old woman who presented initially with an abrupt onset of rheumatoid arthritis-like symmetrical polyarthritis. The patient was diagnosed with pancreatic cancer with multiple hepatic metastases approximately six months later. Her symptoms of polyarthritis improved after the introduction of medication including non-steroidal anti-inflammatory drugs and disease modifying anti-rheumatic drugs. This case suggests that a hidden malignancy should always be considered in elderly patients, who present with an abrupt onset of symmetric polyarthritis for the first time in their life.

Successful Treatment with Methotrexate and Azathioprine of Panniculitis in a Patient with Dermatomyositis: Case Report and Review of the Literature / 대한내과학회지

Dermatomyositis is an autoimmune disease wherein autoantibodies and immune complexes cause non-suppurative inflammation in skin and muscle. Although the skin lesions of this disease are well known, the clinical and pathological features of subcutaneous fat lesions remain uncharacterized. Since Weber (1924) reported the development of panniculitis in a patient with dermatomyositis, 23 subsequent cases have been reported. Here, we present the case of a woman who had been diagnosed with dermatomyositis 7 years previously, and presented with diffuse, painful, indurated nodules on her face and upper limbs. A skin biopsy allowed us to diagnose these nodules as panniculitis. The patient was initially treated with high doses of prednisolone and methotrexate. She responded inadequately to these medications, so azathioprine was added to the regimen. The skin lesions subsequently improved and the patient has remained disease-free for 1 year. Here, we present the clinical profile of this patient and review the relevant literature.

A Case of Rheumatoid Arthritis Associated with Turner's Syndrome / 대한류마티스학회지

Turner's syndrome (TS) is characterized by short stature and gonadal dysgenesis. It is often associated with systemic manifestations, such as cardiovascular, gastrointestinal, and musculoskeletal disorders. Although very rare, it is possible for TS to accompany autoimmune disease, including thyroid disease, inflammatory bowel diseases, diabetes mellitus, psoriatic arthritis, and juvenile rheumatoid arthritis. A 39-year-old woman was referred for symmetric polyarthritis of her hands and feet. She had been diagnosed with Turner's syndrome with 46,XO,-X,+fragment before the age of 22 years and had developed autoimmune hypothyroidism treated with thyroid hormone replacement. At the time of first visit, she had polyarthralgia with morning stiffness for more than 3 months. The musculoskeletal examination revealed symmetrical polyarthritis affecting the metacarpophalangeal, proximal interphalangeal, and metatarsophalangeal joints, fulfilling the ACR 1987 revised criteria for rheumatoid arthritis (RA). Here, we present an unusual case of RA associated with TS. It is important to pay meticulous attention to patients with TS so that inflammatory arthritis is not neglected and the diagnosis is not delayed.

A Case of Brucellar Spondylitis Complicated by Acupuncture / 대한류마티스학회지

Brucellosis is an endemic zoonotic disease, particularly in the Middle East and Mediterranean regions, and can involve many organs and tissues. Recently, the incidence of human brucellosis has increased rapidly in Korea. Brucellosis may often appear as other infections and asymptomatic conditions because of its range of clinical manifestations. Therefore, its diagnosis is frequently missed or delayed. Early diagnosis of brucellar spondylitis is important for reducing the number and intensity of complications. Therefore, it should be included in a differential diagnosis of back pain with an unknown origin. Brucellosis is normally transmitted to humans by direct contact with infected animals or by the ingestion of unpasteurized dairy products. We report an unusual case of brucellar spondylitis complicated by acupuncture.

Incidence of complications and risk factors for nosocomial pneumonia in patients with tetanus / 대한내과학회지

BACKGROUND/AIMS: Tetanus is a fatal disease and various complications affect its prognosis. In Korea, tetanus cases have been reported, but no studies, thus far, have examined its complications. We investigated the complications of tetanus and analyzed the risk factors for nosocomial pneumonia, one of most common complications of tetanus. METHODS: We retrospectively reviewed the clinical features, treatment, and complications of 42 patients diagnosed with tetanus at Chonnam National University Hospital from January 1999 through June 2008. The severity of tetanus was assessed using the APACHE II and Ablett scores. We compared two groups, one with nosocomial pneumonia and the other without it, and analyzed the risk factors for nosocomial pneumonia. RESULTS: Forty-two patients (13 men, 29 women) were enrolled. Only three of them had received a primary tetanus vaccination; the others had not received primary or booster vaccinations. Twenty-one patients needed mechanical ventilation. The most common infectious complication was pneumonia (47.4%), followed by blood stream (15.8%) and urinary tract (13.2%) infections. Regarding noninfectious complications, dysautonomia was also common, including fluctuating blood pressure (31.6%), arrhythmias (26.3%), and cardiac arrest (7.9%). Nosocomial pneumonia was associated with autonomic disturbance (OR=32.0, 95% CI 1.9-524.2; p=0.005) and length of stay in the intensive care unit (OR=1.145, 95% CI 1.02-1.29; p=0.024) in the multivariate analysis. CONCLUSIONS:Careful monitoring and treatment of complications are essential in managing tetanus, along with specific therapy for tetanus itself. Education and tetanus immunization are important for preventing the disease, especially in high-risk areas.

Toxic Epidermal Necrolysis after Rituximab Therapy for Immune Thrombocytopenic Purpura

Rituximab is a chimeric monoclonal antibody that specifically targets the CD20 molecule on the B cell surface. Although rituximab was originally introduced for the treatment of lymphoid neoplasms such as non-Hodgkin's lymphoma (NHL) and chronic lymphocytic leukemia (CLL), it is now emerging as an effective and relatively safe therapeutic option for the patients with refractory immune thrombocytopenic purpura (ITP). We report here on a case of life-threatening toxic epidermal necrolysis (TEN) that was related with the use of rituximab in a patient with refractory ITP. The patient developed extensive erythematous papules and bullous lesions on his whole body associated with fever and visual disturbance during the second cycle of rituximab. The rituximab was discontinued and high dose intravenous immunoglobuline and steroid were administrated. Four weeks later, he fully recovered without any sequelae. A review of the literature reveals this to be the first reported case of TEN associated with rituximab injection in Korea.