Severe Hypokalemia and Respiratory Muscle Paralysis: An Atypical Manifestation of Primary Sjögren’s Syndrome.

Publication date: Dec 01, 2024

Primary SjcF6gren’s syndrome (SS) is a systemic autoimmune disorder primarily affecting exocrine glands, that may occasionally present with severe extra-glandular manifestations. Although rarely, severe hypokalemia and respiratory muscle paralysis may be initial presentations. We report the case of a 33-year-old woman with a recent history of severe acute respiratory syndrome coronavirus 2 (SARS-CoV2) infection who presented with headache and generalized muscle weakness. An evaluation revealed severe hypokalemia, acute kidney injury, and metabolic acidosis. Despite intravenous potassium supplementation, her condition rapidly deteriorated, leading to respiratory failure requiring invasive ventilation. Further investigations showed a urinary pH >5. 5, indicating distal renal tubular acidosis (dRTA). Positive antinuclear antibody (ANA) titers, SS-related antigen A antibody (anti-Ro/SSA), and lymphocytic infiltration on parotid gland biopsy confirmed a diagnosis of primary SS. Treatment with bicarbonate, potassium supplementation, and oral corticosteroids was initiated, leading to clinical improvement. This case underscores the importance of considering SS in patients with unexplained dRTA and severe hypokalemia, emphasizing the importance of timely diagnosis and tailored therapy in managing systemic manifestations.

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Concepts Keywords
Acidosis hypokalemia
Coronavirus respiratory muscle paralysis
Lymphocytic sicca symptoms
Potassium
Rapidly

Semantics

Type Source Name
disease MESH Hypokalemia
disease MESH Respiratory Muscle Paralysis
disease MESH Syndrome
disease IDO history
disease MESH infection
disease MESH acute kidney injury
disease MESH metabolic acidosis
drug DRUGBANK Potassium
disease MESH respiratory failure
disease MESH distal renal tubular acidosis
pathway REACTOME Reproduction
disease MESH autoimmune disease
drug DRUGBANK Gold
disease MESH Rheumatism
disease MESH keratoconjunctivitis sicca
disease MESH xerostomia
disease MESH interstitial nephritis
disease MESH glomerulonephritis
disease MESH paralysis
disease IDO process
disease MESH depressive disorder
disease MESH Emergency
disease IDO symptom
drug DRUGBANK Ibuprofen
drug DRUGBANK Acetaminophen
disease MESH dysphonia
disease MESH hyporeflexia
disease MESH abnormalities
drug DRUGBANK Creatinine
drug DRUGBANK Chloride ion
drug DRUGBANK Potassium bicarbonate
disease MESH nephrocalcinosis
disease MESH nephrolithiasis

Original Article

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