Baseline and annual evaluations
| System . | Baseline . | Routine monitoring . | Situational . |
|---|---|---|---|
| Rheumatologic | Autoantibodes (ANA, Sm, RNP, SSA, SSB, APL Abs ANCA, RF, and CCP) Mandatory disease activity markersa: ESR, CRP, C3/C4 Facultative disease activity markersa: S100A8/9, S100A12, SAA. CXCL9, IL-18, sIL2R, IFN signature (nanostring, four-gene panel, monocyte CD169/CD274) | At least annually: Mandatory disease activity markersa: ESR, CRP, and C3/C4 Facultative disease activity markersa: S100A8/9, S100A12, SAA, IL18, CXCL9, sIL2R, and IFN signature (nanostring, four-gene panel, monocyte CD169/CD274) | Plasma cytokines Imaging of affected joints CK, aldolase Additional autoantibodies (myositis, scleroderma related) |
| Dermatologic | Skin exam and symptom screen by primary provider | At least annually: Skin exam, symptom screen | Skin biopsy |
| Dental/oral health | Symptom screen | At least annually: Symptom screen | Salivary flow measurement Salivary gland imaging MSG biopsy Dental evaluation (unusual caries) |
| Neurologic | Symptom screen | At least annually: Symptom screen | CNS: Brain MRI, MRA, MRV, and LP PNS: EMG ± biopsy, comprehensive neuropsychiatric testing |
| Ophthalmologic | Comprehensive eye exam, slit lamp, retinal, tear film staining | At least annually: Comprehensive eye exam | Uveitis, scleritis, episcleritis, vasculitis, and dry eye disease |
| Cardiac | Symptom screen | At least annually: Symptom screen | Myocarditis: Cardiac MRI, cardiac PET, cardiac catheterization, cardiac enzymes, lipids, TTE, and EKG |
| Pulmonary | Symptom screen | At least annually: Symptom screen | PFTs, CXR, HRCT chest, bronchoscopy, and VATS |
| Gastrointestinal | (In addition to general tests) Hepatic panel, fecal calprotectin Fib4 score | At least annually: Hepatic panel, Fib4 score As needed: Calprotectin | Micronutrient levels EGD Enteroscopy Colonoscopy Abdominal imaging (CT and US) Autoimmune hepatitis serologies Celiac screen |
| Renal | (In addition to general tests) BMP (serum electrolytes, BUN, and Cr) UA and UPCR | At least annually: UA, UPCR, and BMP | If concern for CKD: Renal US and kidney biopsy |
| Endocrine | Growth curves, TSH, free T4 Antithyroid Abs A1C | At least annually: Growth curves (for children), TSH, free T4 Situational: A1c, fasting glucose, and antithyroid Abs | DEXA scan T1DM-related autoantibodies |
| Hematologic and oncologic | CBC with differential, PT/PTT, iron, and B12 APL Abs, cancer screening | At least annually: CBC with diff, cancer screening (age appropriate) As needed: PT/PTT, Iron, B12 APL Abs | Anti-IF antibodies Anti-PLT antibodies DAT, Coombs SPEP, UPEP, IEP, and K/L FLC Bone marrow biopsy, PET-CT |
| Allergic and immunologic | Quantitative immunoglobulins T/B/NK subsets Vaccine titers Annual vaccinations | At least annually: Quantitative immunoglobulins, T/B/NK subsets Annual vaccinations As needed: Vaccine titers | IgG subsets B cell subsets T cell subsets Anti-cytokine antibodies |
| System . | Baseline . | Routine monitoring . | Situational . |
|---|---|---|---|
| Rheumatologic | Autoantibodes (ANA, Sm, RNP, SSA, SSB, APL Abs ANCA, RF, and CCP) Mandatory disease activity markersa: ESR, CRP, C3/C4 Facultative disease activity markersa: S100A8/9, S100A12, SAA. CXCL9, IL-18, sIL2R, IFN signature (nanostring, four-gene panel, monocyte CD169/CD274) | At least annually: Mandatory disease activity markersa: ESR, CRP, and C3/C4 Facultative disease activity markersa: S100A8/9, S100A12, SAA, IL18, CXCL9, sIL2R, and IFN signature (nanostring, four-gene panel, monocyte CD169/CD274) | Plasma cytokines Imaging of affected joints CK, aldolase Additional autoantibodies (myositis, scleroderma related) |
| Dermatologic | Skin exam and symptom screen by primary provider | At least annually: Skin exam, symptom screen | Skin biopsy |
| Dental/oral health | Symptom screen | At least annually: Symptom screen | Salivary flow measurement Salivary gland imaging MSG biopsy Dental evaluation (unusual caries) |
| Neurologic | Symptom screen | At least annually: Symptom screen | CNS: Brain MRI, MRA, MRV, and LP PNS: EMG ± biopsy, comprehensive neuropsychiatric testing |
| Ophthalmologic | Comprehensive eye exam, slit lamp, retinal, tear film staining | At least annually: Comprehensive eye exam | Uveitis, scleritis, episcleritis, vasculitis, and dry eye disease |
| Cardiac | Symptom screen | At least annually: Symptom screen | Myocarditis: Cardiac MRI, cardiac PET, cardiac catheterization, cardiac enzymes, lipids, TTE, and EKG |
| Pulmonary | Symptom screen | At least annually: Symptom screen | PFTs, CXR, HRCT chest, bronchoscopy, and VATS |
| Gastrointestinal | (In addition to general tests) Hepatic panel, fecal calprotectin Fib4 score | At least annually: Hepatic panel, Fib4 score As needed: Calprotectin | Micronutrient levels EGD Enteroscopy Colonoscopy Abdominal imaging (CT and US) Autoimmune hepatitis serologies Celiac screen |
| Renal | (In addition to general tests) BMP (serum electrolytes, BUN, and Cr) UA and UPCR | At least annually: UA, UPCR, and BMP | If concern for CKD: Renal US and kidney biopsy |
| Endocrine | Growth curves, TSH, free T4 Antithyroid Abs A1C | At least annually: Growth curves (for children), TSH, free T4 Situational: A1c, fasting glucose, and antithyroid Abs | DEXA scan T1DM-related autoantibodies |
| Hematologic and oncologic | CBC with differential, PT/PTT, iron, and B12 APL Abs, cancer screening | At least annually: CBC with diff, cancer screening (age appropriate) As needed: PT/PTT, Iron, B12 APL Abs | Anti-IF antibodies Anti-PLT antibodies DAT, Coombs SPEP, UPEP, IEP, and K/L FLC Bone marrow biopsy, PET-CT |
| Allergic and immunologic | Quantitative immunoglobulins T/B/NK subsets Vaccine titers Annual vaccinations | At least annually: Quantitative immunoglobulins, T/B/NK subsets Annual vaccinations As needed: Vaccine titers | IgG subsets B cell subsets T cell subsets Anti-cytokine antibodies |
ANA, antinuclear antibody; Sm, Smith; RNP, ribonucleoprotein; APL, antiphospholipid; ANCA, antineutrophil cytoplasmic antibody; RF, rheumatoid factor; CCP, cyclic citrullinated peptide; ESR, erythrocyte sedimentation rate; CRP, C-reactive protein; SAA, serum amyloid A; IL, interleukin; sIL2R, soluble IL-2 receptor; IFN, interferon; CK, creatine kinase; MSG, minor salivary gland; MRI, magnetic resonance imaging; MRA, magnetic resonance angiography; MRV, magnetic resonance venography; LP, lumbar puncture; EMG, electromyography; PET, positron emission tomography; TTE, transthoracic echocardiogram; EKG, electrocardiogram; PFT, pulmonary function test; CXR, chest x-ray; HRCT, high resolution computed tomography; VATS, video assisted thoracoscopic surgery; EGD, esophagogastroduodenoscopy; US, ultrasound; BMP, basic metabolic panel; BUN, blood urea nitrogen; Cr, creatinine; UA, urinalysis; UPCR, urine protein:creatinine ratio; CKD, chronic kidney disease; TSH, thyroid-stimulating hormone; DEXA, dual energy x-ray absorptiometry; T1DM, type 1 diabetes mellitus; CBC, complete blood count; PT, prothrombin time; PTT, partial thromboplastin time; IF, intrinsic factor; PLT, platelet; DAT, direct antigen test; SPEP, serum protein electrophoresis; UPEP, urine protein electrophoresis; IEP, immunoelectrophoresis; K/L FLC, κ/λ-free light chain; IgG, immunoglobulin G.
Mandatory vs. facultative disease activity markers: We recommend checking broadly available disease activity markers (labeled “mandatory”) for all patients with HA20. Other disease activity markers (labeled “facultative”) may be available only at selected diagnostic immunology labs. However, we have seen patients with active organ-threatening disease (i.e., liver disease and neuroinflammation) who have normal standard disease activity markers but elevated facultative disease activity markers. We therefore check both mandatory and facultative markers annually in all patients.