Common variable immunodeficiency (CVID) is a heterogeneous immunodeficiency syndrome characterized by hypogammaglobulinemia, recurrent bacterial infections, and various immunologic abnormalities. The clinical presentation is generally that of recurrent pyogenic sinopulmonary infections. Our objectives were to study the prevalence of lung involvement and the response to intravenous immunoglobulin replacement therapy in 19 patients with CVID. Nineteen patients (12 men) with a mean age (SD) of 33.1 (17.1) years had a previous diagnosis of CVID and were treated with intravenous immunoglobulin replacement. All patients underwent complete pulmonary function tests and high-resolution computed tomography (HRCT) examination. Bronchiectasis was diagnosed in 11 (58%) patients and eight (42%) were multi-lobar bronchiectasis. Chronic airflow limitation (CAL) was present in 10 (53%) patients and a restrictive pattern was seen in one case. Eleven patients (58%) presented a decrease in single-breath carbon monoxide diffusing capacity of the lung (DL(CO)). Before intravenous immunoglobulin replacement therapy (INIRT), 84% of patients had suffered from at least one episode of pneumonia. Episodes of lower respiratory tract infection decreased significantly from 0.28 per patient and year before replacement therapy to 0.16 per patient and year after treatment. The mean duration of replacement therapy was 7.5 years. In conclusion lung involvement was frequent in patients with CVID. Long-term administration of intravenous gammaglobulin resulted in a substantial reduction of pneumonic episodes.
Neurofibromatosis Type 1 ( NF1 ) is an autosomal dominant genetic disorder with an incidence of approximately 1 in 4,000 live births [1] . Pulmonary arterial hypertension (PAH) is a rare but extremely life-threatening complication associated with NF1 . Timely recognition of this unusual and severe association between NF1 and PAH is imperative in prolonging the survival in this specific patient population. We present the clinical outcomes of a 47-year old female previously diagnosed with NF1 , who presented with progressively worsening dyspnea.
Challenging differential diagnosis Background: Direct oral anticoagulant agents (DOACs) have become increasingly more popular in recent years and have largely replaced warfarin in the treatment of certain conditions, such as atrial fibrillation, and in the prevention of thromboembolic events. Rivaroxaban is one of the most commonly used direct anticoagulant drugs for conditions such as atrial fibrillation and thromboprophylaxis. Case Report: We present a case of a 70-year-old male who developed acute interstitial nephritis after starting rivaroxaban, and who responded to medical treatment, which included corticosteroid therapy. A renal biopsy was not performed because the patient was on essential anticoagulation therapy secondary to a high CHADS2VASc score. Conclusions: Dose adjustments when using rivaroxaban are necessary in patients with underlying renal failure. Acute interstitial nephritis is a rare condition associated with direct anticoagulant drugs. The treatment of acute interstitial nephritis is usually to remove the offending agent and treat the underlying cause.
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