Table 2.
Clinical laboratory data of CAPS in the young patient.
| Organ Manifestation | Clinical Signs | Altered Laboratory Data | (Reference Range) | Normal Laboratory Tests |
|---|---|---|---|---|
|
Lung Bilateral Pulmonary Embolism |
Polypnea Dyspnea |
Hemoglobin: 79 g/L WBC 16 × 109/L Platelet count: 75 × 109/L |
110–153 g/L 3.5–11.0 × 109/L 115–370 × 109/L |
|
| Kidney | - | Proteinuria >500 mg/g Creat. Leukocyturia 596 cel/µL Erythrocyturia 343 cel/µL |
<80 Negative Negative |
Serum creatinine |
| Brain | Headache Blurred vision Neurosensory deficit Vomit attacks Comatose state |
pH 7.47 pCO2 34 pO2 72 HCO3− 21 |
7.35–7.45 35–45 mmHg 80–100 mmHg 22–26 mmol/L |
Absence of Leiden Factor V mutation Absence of prothrombin mutation PT20210A Protein C and protein S |
|
Heart MINOCA |
Epigastric pain Chest pain Hypotension Tachycardia |
I-Troponin (HS) 4741 ng/L | <40 ng/L | NT-proBNP |
| Skin | Fever Livedo reticularis |
Anti-mitochondria antibody (AMA) Anti-smooth muscle antibody (ASMA) | ||
|
Liver Elevated liver enzymes |
Right hypochondrium pain | AST 70 U/L ALT 88 U/L |
0–35 U/L 0–35 U/L |
Bilirubin (total and direct) GammaGT Anti-Liver-Kidney Microsomial antibody |
| Pancreas | - | Pancreatic amylase 67 U/L LDH 365 U/L |
0–53 U/L 0–210 U/L |
Anti-gastric parietal cells antibodies |
| Spleen | - | D-dimers 5850 μg/L | 0–500 μg/L FEU | |
| Thyroid | - | TSH | ||
| Placenta | Areas of necrosis Vascular congestion Small placental infarctions |
Fibrinogen 574 mg/dL | 200–400 mg/dL | INR: 1.1 aPTT: 36 |
| Infections | VES 79 mm/h | 0–30 | Procalcitonin 0.22 µg/L (<0.50) | |
| C-Reactive Protein 12.3 mg/dL | 0–0.50 | Anti-HIV 1–2 (CHIV ag/Ab), Ag-HBs, Anti-HCV, SARS-CoV-2 | ||
| Blood culture (×8): negative | ||||
| Urine culture (×3): negative | ||||
| Rectal swab: negative | ||||
| Anti-HSV 1-2 IgG antibodies Anti-EBV antibodies Anti-OMV antibodies |
Present Present Present |
Toxoplasmosis, Varicella-Zoster virus, Cytomegalovirus, Measles antibodies, Paramyxovirus, Echovirus, Coxachievirus, Adenovirus, hRSV, Chlamydia pneumoniae, Q-Fever, Ab. Anti T. pallidum Ig | ||
| Immune system | Anti-Double-Stranded-DNA antibodies 65 UI/mL | ≤35 UI/mL | Rheumatoid factor | |
| LAC 1.36/2.35 | Ratio < 1.20 | Functional Antithrombin III | ||
| Anti-Cardiolipin IgG 851 U/mL Anti-Cardiolipin IgM 770 U/mL |
<20 <20 |
Complement 3 1.5 g/L (0.9–1.8) | ||
| aβ2GPI IgG 2761 U/mL aβ2GPI IgM >840 U/mL |
<20 <20 |
Complement 4 0.16 g/L (0.10–0–40) | ||
| ANA 1:160 | <1:80 | IgG, IgM, IgA (mg/dL): negative |
aβ2GPI: anti-β-2-glycoprotein I; Ag-HBs: antigen-Hepatitis B surface; ALT: alanine aminotransferase; ANA: antinuclear antibodies; Anti-EBV: anti-Epstein-Barr virus antibodies; Anti-HCV: antibodies-Hepatitis C virus; Anti-HIV 1-2: antibodies-Human immunodeficiency virus type1-2; Anti-OMV: anti-orthomyxovirus antibodies; Anti-HSV: anti-herpes simplex virus antibodies; aPTT: activated partial thromboplastin time; AST: aspartate aminotransferase; CHIV ag/Ab: HIV antigen/antibody combo assay; FEU: Fibrinogen Equivalent Units; GammaGT: gamma-glutamyl transferase; HIV: human immunodeficiency virus; hRSV: human respiratory syncytial virus; INR: International Normalized Ratio; LAC: Lupus anticoagulant (two tests); LDH: lactate dehydrogenase; NT-proBNP: N-terminal proBrain Natriuretic Peptide; PT20210A: prothrombin 20210A; SARS-CoV-2: severe acute respiratory syndrome Coronavirus 2; TSH: thyroid-stimulating hormone; VES: velocity of Erythrocyte Sedimentation; WBC: white blood cell.