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Case 140

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Female patient-65 year, with previous history of insidious idiopathic pneumopatia and chronic use of systemic corticosteroid on the past 10 years. She was admitted to hospital with dyspnea and dry cough in crises, after prednisone tapering attempt, associated with the mouth and dry eyes, alopecia and severe pain of mechanical rhythm in right hip joint, begun six months ago. At physical exam, the patient displays asymmetric polyarthritis in small joints of hands and mentions arthralgia with inflammatory rhythm and morning stiffness with duration less than 30 minutes. Laboratory tests: Normocytic and Normochromic Anemia, Antinuclear Factor (ANA) + 1/320; Anti-Ro (SS-A) +; Rheumatoid factor (RF) +, Urinalysis (UA) unremarkable, normal levels of C3 and C4.

Based on clinical history and images presented what are the most likely diagnostic hypotheses?

a) Systemic lupus erythematosus, acute pneumonitis, primary hip osteoarthritis

25%

b) Rheumatoid arthritis, rheumatoid lung disease, hip osteonecrosis

25%

c) Primary Sjögren\\\'s syndrome, fibrosing interstitial lung disease, hip osteonecrosis

25%

d) Idiopathic fibrosing interstitial lung disease, primary osteoarthritis of hands and hips

25%
   

Comments

- Primary Sjögren's syndrome (pSS) is characterized by generalized dryness, mostly xerostomia (dry mouth) and keratoconjunctivitis sicca (dry eye).

- The disease may be primary or secondary when associated with other autoimmune diseases.

- Pulmonary involvement is the most common systemic manifestation, which can be subclinical in up to 30% of patients.

- The diagnosis of hip osteonecrosis (HON) requires high clinical suspicion, especially in patients on chronic use of steroids.

- The X-ray may be normal in initial stage of HON and the MRI is the gold standard for diagnosis.

- In the case presented, the chronic use of corticosteroid may have controlled the pulmonary manifestations, which contributed to hinder the diagnosis of pSS.

References

- Harrison T, et al. Harrison – Medicina Interna (Volume I). 17ª Edição. New York: McGraw Hill; 2008.

- Carvalho MAP, Lanna CCD, Bertolo MB. Reumatologia – Diagnóstico e Tratamento. 3ª Edição. Rio de Janeiro: Guanabara Koogan; 2008.

- Shiboski SC, et al. American College of Rheumatology Classification Criteria for Sjögren’s Syndrome: A Data-Driven, Expert Consensus Approach in the Sjögren’s International Collaborative Clinical Alliance Cohort. Arthritis Care & Research. 2012; 64(4): 475–487.

Authors

Henrique Elias Darmstadter, 5th year medical student at UFMG School of Medicine

E-mail: henriqueelias[at]terra.com.br

Werlley Meira De Oliveira, 5th year medical student at UFMG School of Medicine

E-mail: werlleymeira[at]yahoo.com.br

Hercules Hermes Riani Martins Silva, 5th year medical student at UFMG School of Medicine

E-mail: herculesriani[at]gmail.com

Lucas Fonseca Rodrigues, 5th year medical student at UFMG School of Medicine

E-mail: lucasbhfonseca[at]gmail.com

Supervisor

Prof Cristina Costa Duarte Lanna, reumathologist, professor of the Department of Locomotor System at UFMG School of Medicine

E-mail: duartelanna[at]gmail.com

Reviewers

Thais Araujo, Luanna Monteiro, André Toledo, Janaína Lima, Prof Viviane Parisotto, Cinthia Barra, Renato Campanati

Translated by

Hercules Hermes Riani Martins Silva, 5th year medical student at UFMG School of Medicine

E-mail: herculesriani[at]gmail.com

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