*3.2. Lung Diseases*

Twenty-nine patients underwent chest computed tomography (CT) scanning. The radiological study demonstrated the presence of bronchiectasis in 16 patients (30.7% of total sample) affecting between one and three lobes and mostly cylindrical, peribronchial thickening in three, air trapping in two, atelectasis in three, micronodules in three, cyst in three, pulmonary emphysema in three (all ex-smokers), ground glass opacities in six, and interstitial lung fibrosis in one. The chest CT scan was considered normal in six patients (all of them suffering from frequent respiratory infections). In two patients, the combination of chest CT findings (micronodules, cysts), bronchoalveolar lavage (BAL) fluid results (lymphocytic inflammation and multinucleated giant cell), and the lung biopsy of a nodule (lymphocytic infiltration) indicated the presence of lymphocytic interstitial lung disease. Ten (19.2%) patients were diagnosed with asthma with different levels of severity. Eight patients (15.3%), six of them associated with asthma, referred symptoms of allergic rhinitis, and four of them reported clinical symptoms apparently exacerbated seasonally (spring, autumn). In all patients, SPTs and specific IgE for common allergens) were negative (Table 1).

## *3.3. Autoimmune Diseases*

Eighteen patients (34.6%) suffered autoimmune clinical manifestations, either isolated (19%) or combining two o more diseases (15%). Hypothyroidism was diagnosed in 10 patients (19.2%), eight secondary to Hashimoto's thyroiditis, and two resulted from previously treated hyperthyroidism (Graves' disease). Other less frequently found autoimmune diseases are shown in Table 1.

**Table 1.** Non-infectious complications.


PSC, primary sclerosing cholangitis; RA, rheumatoid arthritis; SLE, systemic lupus erythematous. \* Diagnosis based on CT scan images; Bronchoalveolar lavage lung fluid findings and biopsy of a lung nodule. \*\* Chronic or intermittent diarrhea, abdominal pain and bloating.

#### *3.4. Gastrointestinal and Liver Diseases*

Symptoms of enteropathy such as intermittent or persistent chronic diarrhea, abdominal pain, and bloating were present in 11 patients (21%). Some patients associated their symptoms with the ingestion of certain foods. In all cases, both allergen SPTs and specific IgE studies with the putative culprit foods were negative. Fructose and lactose intolerance were assessed in four patients, and only one tested positive in the lactose test. Celiac disease was excluded in most (nine patients) but not all patients by anti-transglutaminase IgG serology. A gluten-free diet was tested in four patients with inconsistent or negative symptomatic response. Biopsies of colon mucosa and/or small intestine were obtained in six patients, and the histological findings were: intraepithelial lymphocytosis (four patients), lymphoid hyperplasia forming aggregates (one patient), and enteritis with chronic inflammation, eosinophilic infiltration, crypt distortion, and gland destruction (one patient). Acute severe autoimmune hepatitis was diagnosed in one patient who had required two liver transplantations. Two patients suffered from primary sclerosing cholangitis (PSC) progressing to cirrhosis requiring liver transplantation. The two patients also suffered from ulcerative colitis (Table 1).

## *3.5. Cutaneous Findings*

Eczematous dermatitis (eight patients, 15.3%) associated with moderate or severe itching in most cases, chronic spontaneous urticaria (CSU) (nine patients, 17.3%), angiedema (four patients associated with CSU), and chronic leg ulcers (two patients) were present among the SIgED patients (Table 1).
