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Symptoms to Diagnosis

Multiple metabolic renal manifestations of a systemic disease

Mohamad Hanouneh, MD and Jose M. Monroy Trujillo, MD
Cleveland Clinic Journal of Medicine March 2024, 91 (3) 155-160; DOI: https://doi.org/10.3949/ccjm.91a.23021
Mohamad Hanouneh
Department of Medicine, Division of Nephrology, Johns Hopkins University School of Medicine, Baltimore, MD; Nephrology Center of Maryland, Baltimore, MD
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  • For correspondence: [email protected]
Jose M. Monroy Trujillo
Department of Medicine, Division of Nephrology, Johns Hopkins University School of Medicine, Baltimore, MD
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Article Figures & Data

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    TABLE 1

    Laboratory results

    TestsResultsaReference range
    Sodium148 mmol/L136–145
    Potassium3.1 mmol/L3.5–5.1
    Chloride115 mmol/L98–107
    Bicarbonate20 mmol/L21–32
    Blood urea nitrogen24 mg/dL7–18
    Creatinine1.0 mg/dL0.6–1.3
    Glucose74 mg/dL60–99
    Calcium9.2 mg/dL8.4–10.5
    Hemoglobin10.2 g/dL13.9–16.3
    White blood cell count5.99 × 109/L4.5–11.0
     Neutrophils67.6%46.2%–80%
     Lymphocytes18.5%10.3%–40.5%
     Monocytes12.7%3.0%–14%
     Eosinophils1.0%0.0%–-5.0%
     Basophils0.2%0.0%–1.0%
    Platelet count311 × 109/L150–350
    Alanine aminotransferase50 U/L6–65
    Aspartate aminotransferase26 U/L3–37
    Alkaline phosphatase87 U/L45–117
    Bilirubin, total< 0.2 mg/dL0.2–1.2
    Albumin3.7 g/dL3.5–5.0
    Magnesium2.3 mg/dL1.8–2.4
    Serum osmolality303 mOsm/kg275–295
    Phosphorus2.1 mg/dL2.5–4.9
    Thyrotropin1.59 mIU/L0.5–4.5
    Arterial blood gasses
     pH7.347.35–7.45
     Partial pressure of carbon dioxide40 mm Hg35–45
     Partial pressure of oxygen95 mm Hg75–100
    Urine
     Random sodium67 mmol/L20–214
     Random potassium8 mmol/L17–95
     Random chloride53 mmol/L24–255
     Random urea207 mg/dL132–1,629
     Osmolality165 mOsm/kg50–1,200
    • ↵a Abnormal results are in boldface.

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    TABLE 2

    Causes of primary and secondary distal renal tubular acidosis

    Primary distal renal tubular acidosis
    Sporadic (idiopathic)
    Inherited (due to a congenital mutation)
    Secondary distal renal tubular acidosis
    Hypergammaglobulinemia
    Autoimmune disorders (eg, lupus erythmatosus, Sjögren syndrome, rheumatoid arthritis)
    Chronic renal allograft rejection
    Obstructive uropathy
    Medullary sponge kidney
    Autoimmune hepatitis
    Primary biliary cholangitis
    Lithium, amphotericin B, ifosfamide
    Sickle cell anemia
    • Based on information in reference 2.

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    TABLE 3

    Serology workup

    TestsResultsNormal range
    Antinuclear antibody1:80< 40 = Negative
    dsDNA antibodyNegativeTiters < 10 = Negative
    Anti-Ro52 (SS-A) IgG366< 20
    Anti-La (SS-B) antibody< 3< 20
    Anti-Ro60 IgG< 5< 20
    CryoglobulinNegativeNegative
    • ds = double strand; Ig = immunoglobulin; SS = Sjögren syndrome

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    TABLE 4

    2016 American College of Rheumatology (ACR) and European Alliance of Associations for Rheumatology (EULAR) classification for primary Sjögren syndrome

    Inclusion criteriaExclusion criteria
    At least 1 symptom of ocular or oral dryness, defined as a positive response to at least 1 of the following questions:
    • Have you had daily, persistent, troublesome dry eyes for more than 3 months?

    • Do you have a recurrent sensation of sand or gravel in the eyes?

    • Do you use tear substitutes more than 3 times a day?

    • Have you had a daily feeling of dry mouth for more than 3 months?

    • Do you frequently drink liquids to aid in swallowing dry food?


    Or suspicion of Sjögren syndrome based on glandular enlargement or the presence of characteristic extraglandular involvement
    Prior diagnosis of any of the following conditions:
    • History of head and neck radiation treatment

    • Active hepatitis C infection (with positive polymerase chain reaction)

    • Acquired immunodeficiency syndrome

    • Sarcoidosis

    • Amyloidosis

    • Graft-vs-host disease

    • Immunoglobulin G4-related disease

    ACR/EULAR classification criteria for primary Sjögren syndromea
    CriteriaScore
    ACR/EULAR classification criteria for primary Sjögren syndrome3
    Anti-Ro/SS-A positive3
    Ocular staining score ≥ 5 (or van Bijsterveld score ≥ 4) in at least 1 eye1
    Schirmer test ≤ 5 mm at 5 minutes in at least 1 eye1
    Unstimulated whole saliva flow rate ≤ 0.1 mL/minute1
    • ↵a The classification of primary Sjögren syndrome applies to any patient who meets the inclusion criteria, does not have any of the conditions listed as exclusion criteria, and has a score ≥ 4.

    • Adapted from reference 7.

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Cleveland Clinic Journal of Medicine: 91 (3)
Cleveland Clinic Journal of Medicine
Vol. 91, Issue 3
1 Mar 2024
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Multiple metabolic renal manifestations of a systemic disease
Mohamad Hanouneh, Jose M. Monroy Trujillo
Cleveland Clinic Journal of Medicine Mar 2024, 91 (3) 155-160; DOI: 10.3949/ccjm.91a.23021

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Multiple metabolic renal manifestations of a systemic disease
Mohamad Hanouneh, Jose M. Monroy Trujillo
Cleveland Clinic Journal of Medicine Mar 2024, 91 (3) 155-160; DOI: 10.3949/ccjm.91a.23021
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