Breastfeeding syndrome adultes M06.1

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Adult onset still disease; Adult onsetstill`s disease; Adult Still's disease; Adult Still Syndrome; AOSD; Still - Adult syndrome

Definition
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Particular course of primary chronic polyarthritis in adulthood, an autoinflammatory syndrome with recurrent urticarial exanthema, lymphadenopathy, re- and intermittent fever > 39 °C, carditis, enlargement of liver and spleen, pericarditis, more rarely myocarditis.

Manifestation
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Men and women are affected about equally often.

Clinical features
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  • Main criteria (n. Yamaguchi et al.):
    • Integument: Volatile, urticarial, morbilliform or scarlatiniform exanthema of the extremities, trunk and face with high recurrence rate. Reinforcement often in the evening, in the morning often absent (90%)
    • Intermittent fever attacks > 39 °C with 1-2 peaks/day, persisting for at least 1 week (> 95%)
    • Arthralgia for at least 2 weeks
    • Arthritides > 2 weeks (90%)
    • Leukocytosis (> 10,000/μl) with at least 80% neutrophils.
  • Secondary criteria:
    • Sore throat
    • Visceral manifestations: Generalized, non-painful lymphadenopathy with or without splenomegaly (45%)
    • Transaminases and/or LDH increased
    • pericarditis (25%)
    • Pleurisy (25%)
    • Rheumatoid factor negative
    • ANA negative.

The diagnosis "adult breastfeeding syndrome" requires at least 5 criteria, 2 of which are main criteria.

Laboratory
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Nonspecific signs of inflammation with leukocytosis and neutrophilia, BSG and CRP significantly elevated, often high ferritin level, thrombocytosis possible (Höllweger N et al. 2018).

Histology
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Dermal oedema with dilated blood and lymphatic vessels, thin perivascular infiltrate of neutrophil granulocytes, lymphocytes and macrophages.

Diagnosis
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Radiologically, osteoporosis close to the joints and periosteal reactions show up as early changes. In chronic cases, evidence of marginal usuria and joint space narrowing. Early closure of the epiphyseal joints.

Differential diagnosis
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Therapy
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Non-steroidal anti-inflammatory drugs, possibly corticoids or basic therapeutics, corresponding to primary chronic polyarthritis. Physiotherapy. S.u. Polyarthritis, chronic.

Note(s)
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The adult breastfeeding syndrome is counted among the extended circle of autoinflammatory syndromes.

In rare cases, overlaps are described with Kikuchi-Fujimoto Syndrome, a cervically emphasized self-limited lymphadenopathy.

Literature
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  1. Herzer P (1986) Still syndrome in adults. German Med Weekly 111: 865-867
  2. Höllweger N et al (2018) Adult onset Still`s syndrome. J Dtsch Dermatol 16: 491-492
  3. Kaufmann S et al (1981) Still syndrome in adults. A contribution to the differential diagnosis of "fever of unknown origin". Med World 32: 1692-1697
  4. Prendiville JS et al (2004) A Pruritic Linear Urticarial Rash, Fever, and Systemic Inflammatory Disease in Five Adolescents: Adult-Onset Still Disease or Systemic Juvenile Idiopathic Arthritis sine Arthritis? Pediatric Dermatol 21: 580-588
  5. Zollner RC et al (1997) Hyperferritinemia in Still syndrome in the adult and reactive hemophagocytic syndrome. Med Klin (Munich) 92: 494-498

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 29.10.2020