Diffuse granulomatous disease: looking inside and outside the lungs

Autor: Felix Chua, Anand Devaraj, Carlos Pavesio, A. Williams, Andrew G. Nicholson, W Peter Kelleher
Rok vydání: 2020
Předmět:
Vital capacity
Respiratory System
Severity of Illness Index
Pulmonary function testing
0302 clinical medicine
DLCO
Lung volumes
030212 general & internal medicine
rare lung diseases
medicine.diagnostic_test
Thoracic Surgery
Video-Assisted

Biopsy
Needle

Immunoglobulins
Intravenous

respiratory system
Immunohistochemistry
Respiratory Function Tests
Treatment Outcome
Cardiology
Female
Radiography
Thoracic

Life Sciences & Biomedicine
Zones of the lung
Adult
Pulmonary and Respiratory Medicine
medicine.medical_specialty
Chest Clinic
Diagnosis
Differential

03 medical and health sciences
FEV1/FVC ratio
Internal medicine
medicine
Humans
Science & Technology
business.industry
1103 Clinical Sciences
interstitial fibrosis
medicine.disease
respiratory tract diseases
Pneumonia
Common Variable Immunodeficiency
Dyspnea
Cough
030228 respiratory system
Lung Diseases
Interstitial

Tomography
X-Ray Computed

Chest radiograph
business
immunodeficiency
Zdroj: Thorax
ISSN: 1468-3296
0040-6376
DOI: 10.1136/thoraxjnl-2019-213797
Popis: A 39-year-old woman presented with a 6-month history of breathlessness, productive cough and painful lumps on her lower legs. She had suffered with recurrent rhinosinusitis and frequent infections of the ears, throat and chest since childhood, as well as cutaneous herpes zoster in her 20s. There was no documented history of pneumonia, invasive or deep-seated infections. She had smoked lightly, was allergic to penicillin and worked as a commercial tea buyer. Examination showed a slim female with a normal breathing pattern. Chest expansion was reduced and fine inspiratory crackles were detected over the lower lung zones. Pulmonary function tests showed ventilatory restriction with decreased gas transfer. Forced expiratory volume in one second (FEV1) 2.10 L, 71% predicted; forced vital capacity (FVC) 2.22 L, 65% predicted; spirometric ratio 0.93; total lung capacity 3.04 L, 62% predicted; haemoglobin (Hb)-adjusted carbon monoxide transfer factor/TLco 45% predicted and its coefficient/Kco 81% predicted. Indices related to small airway function were normal, maximal expiratory flow (MEF)75/50/25 all above 80% predicted and air-trapping was absent. Bloods tests showed normal full blood count, C-reactive protein as well as kidney, bone and liver biochemistry. Serum ACE was elevated at 93 ACEU (ref: 12–68). Elispot assay and HIV serology were negative. A prereferral chest radiograph revealed widening of the superior mediastinum and ill-defined nodular opacities in the middle and lower zones. Despite the history of possible erythema nodosum and raised serum ACE, the account of recurrent infections suggested a differential diagnosis broader than typical sarcoidosis. Demonstration of ventilatory restriction and impaired gas transfer factor hinted towards an interstitial or diffuse lung pathology. The Kco, at nearly twice the TLco in %-predicted terms, indicated a low likelihood of major pulmonary vasculopathy and the absence of airflow limitation or small airway dysfunction steered the diagnosis away from primary bronchiolar disorders. CT with contiguous …
Databáze: OpenAIRE