Most of the interstitial lung diseases are rare, chronic, progressive and fatal disorders, especially in familial form. The etiology of the majority of interstitial lung disease is still unknown. Host susceptibility, genetic and environmental factors may influence clinical expression of each disease. With familial interstitial lung diseases, mutations of surfactant protein B and surfactant protein C or other additional genetic mechanisms (e.g. mutation of the gene for ATP-binding cassette transporter A3) could be associated. We found a 21 month-old girl with respiratory symptoms, abnormal radiographic findings and abnormal open lung biopsy findings compatible with nonspecific interstitial pneumonitis that is similar to those of her older sister died from this disease. We performed genetic studies of the patient and her parents, but we could not find any mutation in our case. High-dose intravenous methylprednisolone and oral hydroxychloroquine were administered and she is still alive without progression during 21 months of follow-up.
Child, Preschool ; Female ; Humans ; Hydroxychloroquine/administration and dosage ; Infant ; Korea ; Lung Diseases, Interstitial/drug therapy/*genetics/pathology/physiopathology ; Methylprednisolone/administration and dosage ; Siblings ; Tomography, X-Ray Computed

OBJECTIVETo observe the effect and mechanism of Chinese medicine therapy for activating blood and dredging collaterals (ABDC) on treating systemic lupus erythematosus complicated with avascular necrosis of the femoral head (SLE-ANFH).

METHODSThirty-four patients (51 joints) with SLE-ANFH were assigned by a random number table to two groups: 22 patients (32 joints) in the treatment group and 12 patients (19 joints) in the control group. All received Western medical conventional treatment for anti-inflammation and immunosuppression, but an additional Chinese medicine decoction prescribed based on ABDC principle was administered to patients in the treatment group. The observation on the patients' condition and therapeutic effect lasted for 3 years.

RESULTSThe patients' conditions in the two groups, as assessed by Association for Research Circulation Osseous (ARCO) staging, were similar before treatment. After treatment, comparison between groups showed significant difference (P<0.05), and the raised Harris functional scores in the treatment group were higher than that in the control group (P<0.01). The post-treatment symptom improving rate in the treated group was 72.73%, which was higher than that in the control group (50.00%, P<0.05). Moreover, the former was superior in improving hematologic and hemorrheologic parameters in terms of prolonging activated partial thromboplastin time, lowering whole blood middle/low shear viscosity, and plasma viscosity (P<0.05 or P<0.01). Two patients in the control group but none in the treatment group received hip joint replacement operation during the observation period.

CONCLUSIONSChinese medicine ABDC therapy could effectively alleviate clinical symptoms and improve joint function of patients with SLE-ANFH. The mechanism may be related to its effects on improving high coagulation manner and trend for getting embolism.

Adolescent ; Adult ; Blood Circulation ; physiology ; Collateral Circulation ; physiology ; Combined Modality Therapy ; Cyclophosphamide ; administration & dosage ; Drugs, Chinese Herbal ; therapeutic use ; Female ; Femur Head Necrosis ; blood ; complications ; therapy ; Humans ; Hydroxychloroquine ; administration & dosage ; Immunosuppressive Agents ; administration & dosage ; Ischemia ; complications ; therapy ; Lupus Erythematosus, Systemic ; blood ; complications ; therapy ; Male ; Medicine, Chinese Traditional ; methods ; Methylprednisolone ; administration & dosage ; Middle Aged ; Treatment Outcome ; Up-Regulation ; Young Adult

OBJECTIVETo investigate the effect of Chinese herbal medicines for nourishing yin, supplementing qi, and activating blood on the reproductive endocrine-immune network and its mechanisms in patients with primary Sjogren's syndrome (pSS).

METHODSSeventy pSS patients were randomly assigned to two groups using a randomized digital table: the integrative therapy group (36 cases) and the control group (34 cases). Thirty healthy subjects were taken as a normal group. The control group was treated with hydroxychloroquine sulfate tablets alone, and the integrative therapy group was treated by Chinese herbal medicines for nourishing yin, supplementing qi, and activating blood combined with hydroxychloroquine sulfate tablets. The treatment course was 6 months for both groups. Before and after treatment, serum estradiol (E2), testosterone (T), luteinizing hormone (LH), prolactin (PRL) by radioimmunoassay and immunoglobulin (IgG) by immunodiffusion, erythrocyte sedimentation rate (ESR) by Westergren, interferon-γ (IFN-γ) and interleukin-4 (IL-4) by enzyme linked immunosorbent assay were determined.

RESULTSE2 and T levels in all patients were lower than those of normal subjects before treatment (P<0.05) and were increased significantly after 6-month treatment (P<0.05). ESR, FSH, LH, IgG, IFN - γ, IL - 4 and ratios of E2/T, and IFN -γ/IL in the patients were higher than those of normal subjects before the treatments (P<0.05), and were reduced significantly after the treatments (P<0.05). The T and IFN - γ levels and E2/T ratio in the patients treated with integrative therapy were reduced significantly compared with the control group (P<0.05). However, the PRL levels before and after treatment were not significantly changed in the two groups (P>0.05). The ratios of E2/T and IFN -γ/IL-4, and levels of IgG and ESR were positively correlated before and after treatment (P<0.05).

CONCLUSIONSThe ratios of E2/T and IFN -γ/IL-4 might be used as indicators of pSS activity. Chinese herbal medicines for nourishing yin, supplementing qi, and activating blood combined with Western medicine could improve the therapeutic effect by regulating the reproductive endocrine-immune network in pSS patients.

Adult ; Blood Sedimentation ; Drugs, Chinese Herbal ; therapeutic use ; Enzyme-Linked Immunosorbent Assay ; Estradiol ; blood ; Female ; Humans ; Hydroxychloroquine ; administration & dosage ; therapeutic use ; Immunodiffusion ; Immunoglobulins ; blood ; Interferon-gamma ; analysis ; Interleukin-4 ; analysis ; Luteinizing Hormone ; blood ; Male ; Peptide Fragments ; analysis ; Prolactin ; blood ; Radioimmunoassay ; Random Allocation ; Sjogren's Syndrome ; drug therapy ; Tablets ; Testosterone ; blood