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

Systemic sclerosis as a connective tissue disease could affect all internal organs of the body and could also manifest as a cutaneous lesion. Cardiac involvement leading to cardiac manifestations in systemic sclerosis patients is not rare. However, cardiac amyloidosis combined with systemic sclerosis is extremely rare. Although there were no definite treatment options in this case, symptomatic treatment is the cornerstone of the management plan. In this case report, we described a correct diagnosis and symptomatic medical care of early reactive cardiac amyloidosis with systemic sclerosis and summarize the current state of the relevant literature.
Amyloidosis* ; Cardiomyopathy, Restrictive ; Connective Tissue Diseases ; Diagnosis* ; Humans ; Scleroderma, Systemic*

Interstitial lung disease (ILD) is the most common pulmonary manifestation of connective tissue disease (CTD). It may develop in the patients with preexisting connective tissue diseases or may be the initial lone manifestation of an underlying CTD. Since ILD causes a potentially substantial morbidity and mortality in the patients with CTD, close collaboration among pulmonologists, rheumatologists, radiologists and pathologists is very essential to optimize the diagnostic evaluation and treatment. Important information on the pattern and extent of the ILD can be obtained by chest HRCT imaging. In general, surgical lung biopsy is not recommended for CTD-associated ILD. All patients with CTD-associated ILD do not require pharmacologic treatment. The decision to treat CTD-associated ILD often depends on 1) whether the patient is clinically impaired by the ILD, 2) whether the ILD is progressive, and 3) what contraindications exist. Differential diagnosis from superimposed pneumonia and drug-induced ILD is quite important. Immunosuppressive therapy is the mainstay of treatment. Recent success of cyclophosphamide in the clinical trials of systemic sclerosis-associated ILD is promising.
Biopsy ; Connective Tissue ; Connective Tissue Diseases ; Cooperative Behavior ; Cyclophosphamide ; Diagnosis, Differential ; Humans ; Lung ; Lung Diseases, Interstitial ; Pneumonia ; Thorax

Connective tissue disease (CTD) are closely related to liver abnormality. CTD can affect the liver causing various degrees of liver injury, coexist with other liver diseases, especially autoimmune liver disease (ALD). Medications for CTD can also lead to liver injury or reactivate the hepatitis B virus. CTD patients can also be positive for ALD-related autoantibodies without corresponding manifestation; and vis versa. The diagnosis and differential diagnosis should be made on integrating clinical presentation, laboratory, imaging, and histological studies, not solely relying on autoantibody positivity.
Autoantibodies ; Autoimmune Diseases/diagnosis* ; Connective Tissue Diseases/diagnosis* ; Diagnosis, Differential ; Humans ; Liver

The antinuclear antbody (ANA) test have been used to screen the patients with systemic lupus erythematosus (SLE) and other autoimmune diseases. We had retrospectively reviewed the 263 records of pediatric patients with doing ANA tests who admitted at Department of Pediatrics, Kyung Hee University Hospital, from January 1988 to May 1993. The following results were obtained. 1) The positive rate of ANA test in patients with connective tissue diseases is 16 out of 40(40%).In patients with SLE, the positive rate of ANA test is 9 out of 11 (82%). 2) The positive predictivity for SLE is 9 out 36 (25%). 3) The positive predictivity for connective tissue disease and possible immune disease is 28 out of 36 (78%). 4) The false positive rate is 8 0ut of 36 (22%), Thus, the pediatric patients with positive ANA test should be applicable for diagnosis with prudence. 5) The positive anti-dsDNA in patients with the positive ANA is shown in 4 cases and these patients are all SLE. In conclusion, the patients who had repeated positive ANA should be tested Anti-dsDNA antibody, and further clinical and diagnostic evaluation of other ANA associated diseases.
Autoimmune Diseases ; Connective Tissue Diseases ; Diagnosis ; Humans ; Immune System Diseases ; Lupus Erythematosus, Systemic ; Pediatrics ; Retrospective Studies

Connective tissue diseases (CTDs) can affect all compartments of the lungs, including airways, alveoli, interstitium, vessels, and pleura. CTD-associated lung diseases (CTD-LDs) may present as diffuse lung disease or as focal lesions, and there is significant heterogeneity between the individual CTDs in their clinical and pathological manifestations. CTD-LDs may presage the clinical diagnosis a primary CTD, or it may develop in the context of an established CTD diagnosis. CTD-LDs reveal acute, chronic or mixed pattern of lung and pleural manifestations. Histopathological findings of diverse morphological changes can be present in CTD-LDs airway lesions (chronic bronchitis/bronchiolitis, follicular bronchiolitis, etc.), interstitial lung diseases (nonspecific interstitial pneumonia/fibrosis, usual interstitial pneumonia, lymphocytic interstitial pneumonia, diffuse alveolar damage, and organizing pneumonia), pleural changes (acute fibrinous or chronic fibrous pleuritis), and vascular changes (vasculitis, capillaritis, pulmonary hemorrhage, etc.). CTD patients can be exposed to various infectious diseases when taking immunosuppressive drugs. Histopathological patterns of CTD-LDs are generally nonspecific, and other diseases that can cause similar lesions in the lungs must be considered before the diagnosis of CTD-LDs. A multidisciplinary team involving pathologists, clinicians, and radiologists can adequately make a proper diagnosis of CTD-LDs.
Bronchiolitis ; Communicable Diseases ; Connective Tissue Diseases ; Connective Tissue ; Diagnosis ; Fibrin ; Hemorrhage ; Humans ; Idiopathic Pulmonary Fibrosis ; Lung Diseases ; Lung Diseases, Interstitial ; Lung ; Pleura ; Pleural Diseases ; Population Characteristics

The diagnosis of juvenile rheumatoid arthritis (JRA) is based on patient's age at disease onset, symptom duration, gender, and clinical manifestations. JRA is of unknown origin, begins under the age of 16, and persists for a minimum of 6 weeks. JRA is categorized into three principal types, systemic, oligoarticular and polyarticular. Infection, other connective tissue diseases, malignancy, trauma, and immunodeficiency are discussed as differential diagnoses for JRA. Because of joint damage, focusing on early diagnosis and intervention, a vigorous initial therapeutic approach must be taken in patients who have poor prognostic factors. A multidisciplinary team approach is also important for the care of patients with JRA.
Arthritis, Juvenile* ; Connective Tissue Diseases ; Diagnosis ; Diagnosis, Differential ; Early Diagnosis ; Humans ; Joints

Child ; Connective Tissue Diseases ; complications ; diagnosis ; therapy ; Humans ; Hypertension, Pulmonary ; diagnosis ; etiology ; therapy

Subcutaneous dirofilariasis is a parasitic infestation found in endemic areas in Mediterranean countries such as Italy. It is occasionally reported in India, mostly from the state of Kerala. Presentation in an infant is extremely rare. We report a case of subcutaneous dirofilariasis in a child that was diagnosed by ultrasonography and confirmed by surgery.
Connective Tissue Diseases ; diagnosis ; diagnostic imaging ; parasitology ; surgery ; Dirofilariasis ; diagnosis ; diagnostic imaging ; surgery ; Female ; Humans ; Infant ; Subcutaneous Tissue ; Ultrasonography

OBJECTIVE: Raynaud's phenomenon is a vascular disorder characterized by reversible spasm of arteries of fingers. It is the first symptom in 70% of partlents with systemic sclerosis. The more systemic involvment the worse prognosis is expected in patients with systemic sclerosis. A more reliable indication of systemic sclerosis is the microvascular involvement by the disease (characteristic patterns of capillary abnormality in the nail-fold). Our puroses were to evaluate the significance of the degree of nailfold capillary abnormlitry in making the diagnosis fo systemic sclerosis, and in determining organ involvement in patients with systemic sclerosis. METHODS: Twenty-six patients with Raynaud's phenomenon whose diagnosis were systemic sclerosis(10 patients), SLE(10 patient), Raynaud's disease(6 patients) were observed for nailfold capillary abnormalities by widefield microscopy. RESULTS: Capillary abnormalities were seen in 100% of the systemic sclerosis (10 patients), 30% of the SLE (3 patients) and 50% of the Raynaud's disease (3 patients). A significant correlation between degree of finger lesions (r=0.718) or organ involvement (X2=20.4, p=0.015) and capillary abnormality class was found although a significant correlation was not found between the duration of the disease and the degree of capillary abnormality in patients with systemic sclerosis (r=0.32). CONCLUSIONS: Nailfold capillary abnormality can easily be observed and could be used as an assistive tools for the diagnosis and prediction of prognosis and extent of organ involvement in patients with Raynaud's phenomenon especially in patients with systemic sclerosis.
Arteries ; Capillaries* ; Connective Tissue Diseases ; Diagnosis ; Fingers ; Humans ; Microscopy ; Prognosis ; Raynaud Disease ; Scleroderma, Systemic ; Spasm