ABSTRACT
RATIONALE: Lymphoid interstitial pneumonia is a rare benign pulmonary lymphoproliferative disorder usually presenting with a sub-acute or chronic condition and frequently associated with autoimmune disorders, dysgammaglobulinemia, or infections. PATIENT CONCERNS: A 74-year-old woman with no past medical history presented with acute dyspnea, nonproductive cough, hypoxemia (room air PaO2: 48âmmHg) and bilateral alveolar infiltrates with pleural effusion. Antibiotics and diuretics treatments did not induce any improvement. No underlying condition including cardiac insufficiency, autoimmune diseases, immunodeficiency, or infections was found after an extensive evaluation. Bronchoalveolar lavage revealed a lymphocytosis (60%) with negative microbiological findings. High-dose intravenous corticosteroids induced a mild clinical improvement only, which led to perform a surgical lung biopsy revealing a lymphoid interstitial pneumonia with no sign of lymphoma or malignancies. DIAGNOSES: Acute severe idiopathic lymphoid interstitial pneumonia. INTERVENTIONS: Ten days after the surgical lung biopsy, the patient experienced a dramatic worsening leading to invasive mechanical ventilation. Antibiotics and a new course of high-dose intravenous corticosteroids did not induce any improvement, leading to the use of rituximab which was associated with a dramatic clinical and radiological improvement allowing weaning from mechanical ventilation after 10 days. OUTCOMES: Despite the initial response to rituximab, the patient exhibited poor general state and subsequent progressive worsening of respiratory symptoms leading to consider symptomatic palliative treatments. The patient died 4 months after the diagnosis of lymphoid interstitial pneumonia. LESSONS: Idiopathic lymphoid interstitial pneumonia may present as an acute severe respiratory insufficiency with a potential transient response to rituximab.
Subject(s)
Antineoplastic Agents, Immunological/therapeutic use , Lung Diseases, Interstitial/drug therapy , Lung/pathology , Rituximab/therapeutic use , Aged , Fatal Outcome , Female , Humans , Lung Diseases, Interstitial/diagnostic imaging , Lung Diseases, Interstitial/pathology , Tomography, X-Ray ComputedABSTRACT
We report the case of a 24-year-old woman with an autoimmune hepatitis/primary biliary cirrhosis overlap syndrome triggered by an acute hepatitis A. A number of viruses have been proposed as potential triggers of autoimmune hepatitis in patients with genetic predisposition. To date, approximately 10 cases of type 1 autoimmune hepatitis following hepatitis A virus infection have been published in the medical literature. To our knowledge, this is the first case of overlap syndrome triggered by an acute hepatitis A.
Subject(s)
Hepatitis A/complications , Hepatitis, Autoimmune/virology , Liver Cirrhosis, Biliary/virology , Acute Disease , Female , Humans , Syndrome , Young AdultABSTRACT
OBJECTIVE: The aim of this retrospective study was to compare clinical, biological, and histological features and treatment response in 115 patients with overlap syndrome (OS), autoimmune hepatitis (AIH) or primary biliary cirrhosis (PBC). METHODS: Consecutive patients with AIH, PBC or OS followed between 1984 and 2005 in five different centers were included. All data were re-evaluated using current diagnostic criteria of each disease. RESULTS: Fifteen patients had OS (13 females), 48 AIH (40 females) and 52 PBC (49 females). Patients with OS were significantly younger than patients with PBC (median age: 44 vs 59 years). Jaundice (20%) and pruritus (20%) were the main initial symptoms in OS. Patients with OS had serum transaminase and gammaglobulin levels significantly higher than patients with PBC; serum alkaline phosphatase, gamma-glutamyl-transpeptidase and IgM levels were significantly higher in OS than in patients with AIH. Histological analysis showed moderate or severe piecemeal necrosis in 86% and destructive cholangitis in 93% in OS group. Among 11 patients with OS treated with ursodeoxycholic acid (UDCA) or immunosuppressors alone, only 6 had a complete biochemical response. In contrast, all patients with OS receiving combined therapy, as first or second line, responded, 5 patients to the combination corticosteroids-azathioprine-UDCA and 2 to the combination cyclosporine-UDCA. CONCLUSION: OS is not rare and accounts for 13.9% of patients with autoimmune liver disease in our series. Combination of immunosuppressors and UDCA appears the most efficient treatment in these patients.
Subject(s)
Hepatitis, Autoimmune/diagnosis , Hepatitis, Autoimmune/drug therapy , Liver Cirrhosis, Biliary/diagnosis , Liver Cirrhosis, Biliary/drug therapy , Liver/pathology , Adolescent , Adult , Aged , Aged, 80 and over , Biopsy , Cholagogues and Choleretics/therapeutic use , Cholangitis, Sclerosing/diagnosis , Cholangitis, Sclerosing/drug therapy , Drug Therapy, Combination , Female , France , Hepatitis, Autoimmune/blood , Humans , Immunosuppressive Agents/therapeutic use , Liver Cirrhosis, Biliary/blood , Liver Function Tests , Male , Medical Records , Middle Aged , Retrospective Studies , Syndrome , Treatment Outcome , Ursodeoxycholic Acid/therapeutic useABSTRACT
The 2001 Bethesda System is a uniform system of terminology for reporting results of pap smears. It is acknowledged by most cytopathologists worldwide as a standard for cervical cytology reports. In France, several national surveys have confirmed its current utilization. However, more specific analysis have shown that the Bethesda System may be routinely modified by individual laboratories or even individual cytopathologist working within the same department. The aim of this progress report was to emphasize the importance of fully understanding the Bethesda System and applying it in a rigorous and standardized way.
