Amyloid-associated cystic lung disease in a woman with Sjögren syndrome

History of present illness

A 68-year-old Caucasian female was admitted to the emergency department after a traumatic accident and a consequent transient loss of consciousness. A total body computed tomography (CT) scan was immediately performed. Brain CT scan showed an occipital subarachnoid hemorrhage and bilateral subdural hematoma, while chest CT scan revealed multiple bilateral cysts (from a few millimeters to 2 cm in size) with a well-defined wall. Bilateral, partly calcified nodules with regular margins (the largest with 21-mm long-axis CT diameter) were also evident ( Fig 19.1 ).

Blood tests showed only a mild lymphocytosis (white blood cell [WBC] count 12,000 cells/μL, normal values: 4,300 cells μL; neutrophils 80%) and a very slight increase in the indices of inflammation (C-reactive protein [CRP] 18 mg/L, normal values <5 mg/L).

Blood pressure was 115/70 mmHg, heart rate was 85 beats/min, body temperature was 36.4°C, and peripheral oxygen saturation (SpO ₂ ) was 98% on room air. The patient was then admitted to the neurology unit and showed rapid clinical improvement.

Past medical history

The patient was a former mild smoker (smoking history of 5 pack-years) who had quit smoking more than 30 years ago. In her past medical history, she had gastroesophageal reflux disease secondary to a hiatal hernia and a diagnosis of osteoporosis, treated with α-calcifediol and alendronate.

She denied any familiar history of respiratory diseases or occupational exposures to irritant agents. She only had two lower tract respiratory infections during the past 10 years.

Physical examination and early clinical findings

During the pulmonology consultation, she was eupneic and denied any respiratory symptoms. SpO ₂ values continued to be normal on room air. At chest examination, the respiratory sound was bilaterally present, without pathological sounds. Heart and abdomen examinations were unremarkable. No digital fissures or clubbing were detectable.

The patient referred mild xerophthalmia without xerostomia or Raynaud phenomenon. She denied morning stiffness or inflammatory joint pains.

To exclude the presence of a connective tissue disease (CTD), a complete serum autoimmunity screening was requested, including antinuclear antibodies (ANA), extractable nuclear antigens (ENA), anti–neutrophil cytoplasmic antibodies (ANCAs), anti–cyclic citrullinated peptide (ACCP), and anti–double-stranded DNA (ds-DNA). Positivities for ANA (titer 1:640 with speckled pattern) and ENA (anti-SSA/Ro-52) were detected.

The patient was evaluated by a rheumatologist who detected chronic sialadenitis with fibrotic evolution on ultrasonography of the salivary glands. Schirmer test and sialometry were positive. The myositis- and scleroderma-specific immunoblots confirmed the isolate positivity for anti-SSA/Ro-52. A diagnosis of Sjögren syndrome was made but no specific rheumatologic treatment was retained to be started at the time of diagnosis.

A PET/CT scan revealed a low glycolytic activity (standardized uptake value [SUV] max 1.3) of all the nodules.

Clinical course

Once neurologically recovered and discharged from the hospital, the patient underwent a complete clinical and functional evaluation in our pulmonology clinic.

The spirometry was normal (forced vital capacity [FVC] was 92%, forced expiratory volume in 1 second [FEV ₁ ] was 95% of the predicted value, and Tiffenau Index was 81.3), and a moderate reduction in diffusing capacity for carbon monoxide (DLCO) was detected (DLCO 57% of the predicted value). A lung surgical biopsy was proposed, but the patient refused. Strict clinical and radiological follow-up was then recommended.

A chest CT scan performed 1 year after the baseline assessment confirmed the stability of the number and size of the cysts and the nodules. The patient was asymptomatic, and pulmonary function tests were within the normal limits.

A new CT scan performed 1 year later revealed a slight increase in the size of a para-aortic nodule of the right upper lobe ( Fig 19.2 ). Significant ¹⁸ F-fluorodeoxyglucose (FDG) uptake of the nodule was then detected with a new PET/CT scan ( Fig 19.3 ).