Rapidly progressive necrotizing pneumonia: remember the Streptococcus anginosus group!

Acute necrotizing pneumonia in an immunocompetent host is uncommon and usually caused by Staphylococcus aureus infection. Streptococcus anginosus group (SAG) is a less recognized cause of rapidly destructive lung infection resulting in significant patient morbidity and mortality. Unlike many other bacterial infections, SAG can cross fascial planes and cause fulminant infections. Necrotizing pneumonia and lung abscesses due to SAG often fails conservative therapy with antimicrobials and requires definitive surgical intervention. Consideration of SAG as a potential etiology might help to institute definitive therapy earlier and prevent complications.

Chronic pleuritis leading to severe pulmonary restriction: a rare complication of Degos disease.

This case demonstrates chronic fibrosing pleuritis, as a rare pulmonary aetiology for mortality in patients with Degos disease or malignant atrophic papulosis (MAP). Knowledge of this unusual complication will help physicians identify this entity early and provide appropriate treatment.Patients with MAP die from gastrointestinal and brain involvement within 2-3 years of diagnosis. This case is unique as the patient survived for 9 years and died secondary to respiratory failure, which had not been reported before. Our patient was a young man, diagnosed with MAP at the age of 17. His skin and gastrointestinal disease were controlled with eculizumab and parenteral treprostinil. The patient developed severe restrictive pulmonary disease, required ventilatory support, and died from respiratory failure. An autopsy revealed chronic fibrosis pleuritis. Longer surviving patients with MAP might suffer from significant respiratory disease. Pulmonary function test should be obtained to identify subclinical respiratory limitation.

Central airway compression by massively dilated pulmonary artery in a patient with pulmonary arterial hypertension: a rare entity.

Patients with pulmonary arterial hypertension (PAH) usually die from progressive right ventricular failure. Mechanical complications due to pulmonary artery (PA) enlargement are rare and include tracheobronchial and left main coronary artery compression, and PA dissection. A 62-year-old female with PAH was seen in our office for follow-up. During the evaluation, spirometry was performed, which revealed a severe obstructive ventilatory limitation. Subsequent workup identified compression of bilateral mainstem bronchi from the dilated PA as the aetiology for the abnormal spirometry. Very few cases of this rare complication have been reported in the literature. A significant dilation of the PA is necessary (>4 cm) for the occurrence of these complications. Dilation of PA is an independent risk factor for sudden unexpected death in patients with PAH.

Measurement of cerebrospinal fluid ACE level in aseptic meningitis: diagnostic?

Neurosarcoidosis (NS) is a rare disease, affecting only 3%-10% of patients with sarcoidosis. The clinical presentation can be protean and often represents a diagnostic challenge. Cerebrospinal fluid (CSF) ACE level has poor sensitivity, but high specificity for establishing a diagnosis of NS. We present a case of NS in a middle-aged African American woman who presented with dysphagia and dysphonia. An extensive radiological workup was negative for structural brain disease. CSF studies demonstrated lymphocyte predominant pleocytosis with an elevated ACE level. A diagnosis of possible neurosarcoidosis was made. She responded to systemic steroid therapy with complete resolution of her symptoms over the next five months. In the appropriate clinical setting, an elevated CSF ACE level could be of paramount importance for making a diagnosis of NS.

Rare case of parathyroid gland sarcoidosis presenting with hypercalcaemia.

Sarcoidosis of the parathyroid gland is a rare occurrence. Parathyroid sarcoidosis is usually associated with parathyroid adenomas, and, therefore, hypercalcaemia is a common presentation of this entity. We present a case of parathyroid sarcoidosis and review the world literature regarding this rare condition. A woman with a history of diffuse large B cell lymphoma underwent a surveillance positron emission tomography scan that showed increased fluorodeoxyglucose uptake in multiple thoracic and abdominal lymph nodes and in a left upper extremity soft tissue mass. Biopsy of the soft tissue mass showed non-caseating granulomas consistent with sarcoidosis. Blood work showed a serum calcium of 11.1 mg/dL with an intact serum parathyroid hormone of 92 pg/dL. Primary hyperparathyroidism was suspected. A neck ultrasound and sestamibi parathyroid scintigraphy demonstrated a parathyroid nodule. She underwent surgical resection, and the histopathology revealed a parathyroid adenoma and non-caseating granulomata consistent with a diagnosis of sarcoidosis.