No abstract available.
Connective Tissue Diseases* ; Connective Tissue* ; Diagnosis*

No abstract available.
Connective Tissue Diseases* ; Connective Tissue* ; Hypertension, Pulmonary*

OBJECTIVES: In the connective tissue disease patients, esophageal dysfunction is often closely associated with the presence of Raynaud's phenomenon. But there are no previous reports concerning the values of esophageal manometry in the connective tissue disease with Raynaud s phenomenon in Korea. Therefore, we performed this study to evaluate esophageal function in connective tissue disease with Raynaud' s phenomenon. METHODS: Total 86 subjects were employed in this study including 30 normal control group, 14 mixed connective tissue disease(MCTD), 21 systemic sclerosis, 16 systemic lupus erythematosus(SLE), and 5 Raynaud s phenomenon only. In each subject, esophageal manometric study was performed with lower compliance capillary infusion system. RESULTS: The mean age(+SD) of patients and controls were as follows : MCTD 34.1(+8.9), systemic sclerosis 44.9(+9. 3), SLE 32. 1(+7.9), and normal controls 31. 9 (+ 5. 3). All patients with MCTD and systemic sclerosis had Raynaud s phenomenon. Twelve out of 14 patients with MCTD, 17 out of 21 patients with systemic sclerosis, were abnormal in esophageal manometry. Nine out of 16 SLE had Raynaud s phenomenon. Among 9 SLE with Raynaud s phenomenon, 5 patients(55.5%) were abnormal in esophageal manometry, and among 7 SLE without Raynaud's phenomenon, 2 patients (28. 6%) were abnormal in esophageal manometry. Among 5 patients with Raynaud's phenomenon, 3 patients were abnormal in esophageal manometry. Abnormal esophageal manometry finding is more common in the connective tissue disease patients with Raynaud's phenomenon compared with the patients without Raynaud's phenomenon (P=O. 0219). CONCLUSIONS: Abnormal esophageal manometry finding is more common in the connective tissue disease patients with Raynauds phenomenon compared with the patients without Raynaud s phenomenon.
Capillaries ; Compliance ; Connective Tissue ; Connective Tissue Diseases ; Humans ; Korea ; Manometry ; Mixed Connective Tissue Disease ; Scleroderma, Systemic

Child ; Humans ; COVID-19 ; Connective Tissue Diseases

Eosinophilic pleural effusion (EPE) is defined as a pleural effusion that contains at least 10% eosinophils. EPE occurs due to a variety of causes such as blood or air in the pleural space, infection, malignancy, or an autoimmune disease. Undifferentiated connective tissue disease (UCTD) associated with eosinophilic pleural effusion is a rare condition generally characterized by the presence of the signs and symptoms but not fulfilling the existing classification criteria. We report a case involving a 67-year-old man with UCTD and EPE, who has been successfully treated with a single intrapleural corticosteroid injection.
Aged ; Autoimmune Diseases ; Connective Tissue Diseases* ; Connective Tissue* ; Eosinophilia ; Eosinophils* ; Humans ; Pleural Effusion*

Interstitial lung diseases are heterogeneous entities with diverse clinical presentations. Among them, idiopathic pulmonary fibrosis and connective tissue disease-associated interstitial lung disease are specific categories that pulmonologists are most likely to encounter in the clinical field. Despite the accumulated data from extensive clinical trial and observations, we continue to have many issues which need to be resolved in this field. In this update, we present the review of several articles regarding the clinical presentation, prognosis and treatment of patients with idiopathic pulmonary fibrosis or connective tissue disease-associated interstitial lung disease.
Connective Tissue ; Connective Tissue Diseases ; Humans ; Idiopathic Pulmonary Fibrosis ; Lung ; Lung Diseases, Interstitial ; Prognosis

The occurrence of a trigeminal neuropathy associated with connective tissue disease is rare. Trigeminal neuropathy in connective tissue disease is predominantly sensory and it is characterized by numbness, hypesthesia, slurred speech, and touch pain. Although the pathogenesis of trigeminal neuropathy associated with connective tissue disease remains obscure, the main pathologic findings are vasculitis and neuritis. A case of trigeminal neuropathy associated with mixed connective tissue disease is described, and the incidence, symptoms, pathophysiology, treatment of the disease is discussed.
Connective Tissue Diseases ; Hypesthesia ; Incidence ; Mixed Connective Tissue Disease* ; Neuritis ; Trigeminal Nerve Diseases* ; Vasculitis

Perforating pseudoxanthoma elasticum is a rare disorder. It is an hereditary connective tissue disease involving progressive fragmentation and dystrophic calcification of elastic fibers. The localized lesions usually occur in a periumbilical location in obese, middle-aged, multiparous black women. It is characterized clinically by yellowish, lax, well-circumscribed, reticulated or cobblestoned patches or plaques of the periumbilical region. Herein, we report a case of periumbilical perforating pseudoxanthoma elasticum which occurred in an obese, multiparous woman with no signs of hereditary systemic pseudoxanthoma elasticum.
Connective Tissue Diseases ; Elastic Tissue ; Female ; Humans ; Pseudoxanthoma Elasticum*

We describe herein four cases of childhood dermatomysitis of Brunsting type. The ages of these patients were between 7 to 10 years at the time of the disease. All four patients had pathognomOnic cutaneous changes, such as heliotrope erythema and Gottron's papules, which are not seen frequently in adult type dermatomyositis. These patients did not show any marked serologic abnormalities suggestive of having other connective tissue diseases or autoimmune diseases. All patients were initially treated with prednisolone and chloroquine, and have been under control with low dose prednisolone, or chloroquine alone.
Autoimmune Diseases ; Chloroquine ; Connective Tissue Diseases ; Dermatomyositis* ; Erythema ; Humans ; Prednisolone

The second case of this serial reports under the title of unusual cutaneous manifestations of connective tissue diseases is a woman with primary idiopathic dermatomyositis who developed cutaneous ulcerations on her arms and legs, which is thought to b a rare manifestation of adult dermatomyositis. These multiple ulcerations are about bean sized, polymorphous, and grouped on the poikilodermatous lesional skin. In this patient these ulcerative lesions became regressive and healed with disfiguring scars after the activity of the disease has controlled by prednisolone and methotrexate.
Adult ; Arm ; Cicatrix ; Connective Tissue Diseases* ; Connective Tissue* ; Dermatomyositis* ; Female ; Humans ; Leg ; Methotrexate ; Prednisolone ; Skin ; Ulcer*