M04.1 PERIODIC FEVER SYNDROMES

Synonyms

• Amyloid of familial Mediterranean fever
• Aphthous ulcer of mouth
• Cervical lymphadenitis
• Complex multigenic autoinflammatory syndrome
• Congenital sideroblastic anemia, B-cell immunodeficiency, periodic fever, developmental delay syndrome
• Deficiency of mevalonate kinase
• Familial Mediterranean fever
• Hemolytic erythrophagocytic syndrome
• Hemophagocytic lymphohistiocytosis
• Hereditary periodic fever
• Hyperimmunoglobulinemia D with periodic fever
• Macrophage activation syndrome
• Menstrual cycle dependent periodic fever
• NLRC4-related familial cold autoinflammatory syndrome
• NLRP12-associated hereditary periodic fever syndrome
• Pericarditis due to familial Mediterranean fever
• Periodic fever, infantile enterocolitis, autoinflammatory syndrome
• PFAPA syndrome
• Recurrent aphthous ulcer
• Recurrent ulcer of mouth
• Secondary systemic amyloidosis
• TNF receptor-associated periodic fever syndrome

Diagnostic Related Group(s)

The code M04.1 is grouped in the following Diagnostic Related Group(s) MS-DRG V42.0

1. Connective Tissue Disorders With Mcc (545)
2. Connective Tissue Disorders With Cc (546)
3. Connective Tissue Disorders Without Cc/mcc (547)

References to Index of Diseases and Injuries

The code M04.1 has the following ICD-10-CM references to the Index of Diseases and Injuries

Crosswalk Information

ICD-10 Code		ICD-9 Code	ICD-9 Description
M04.1		277.31	Fam Mediterranean fever

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