icd 10 code for pfapa syndrome

What is the ICD 10 code for Autoinflammatory syndromes?

PFAPA (periodic fever, aphthous stomatitis, pharyngitis, and adenopathy syndrome) M04.8 ← Previous Page 1 Next Page → ICD-10 Code Lookup Find the disease or condition using the Alphabetic Index displayed on this page. Select the associated ICD-10 code to view any special notations from the Tabular List .

What is the ICD 10 code for periodic fever?

Oct 01, 2021 · M04.8 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes. The 2022 edition of ICD-10-CM M04.8 became effective on October 1, 2021. This is the American ICD-10-CM version of M04.8 - other international versions of ICD-10 M04.8 may differ.

What is the ICD 10 code for pyoderma gangrenosum?

ICD-10-CM Diagnosis Code M04.8 [convert to ICD-9-CM] Other autoinflammatory syndromes. Blau syndrome; Deficiency of interleukin ... aphthous stomatitis, pharyngitis, and adenopathy syndrome [PFAPA]; Pyogenic arthritis, pyoderma gangrenosum, and acne syndrome [PAPA] ICD-10-CM Diagnosis Code M04.8. Other autoinflammatory syndromes. 2016 2017 2018 ...

What is the ICD 10 Index for Peyronies disease?

References in the ICD-10-CM Index to Diseases and Injuries applicable to the clinical term "pfapa (periodic fever, aphthous stomatitis, pharyngitis, and adenopathy syndrome)" PFAPA (periodic fever, aphthous stomatitis, pharyngitis, and adenopathy syndrome) - M04.8 Other autoinflammatory syndromes. Previous Term: Peyronies Disease.

Is Pfapa an autoinflammatory disorder?

Introduction. PFAPA syndrome (periodic fever, aphtous stomatitis, pharyngitis, adenitis) is an autoinflammatory disease, for which no genetic marker has been identified yet, and its etiology remains unknown.Sep 28, 2015

Is Pfapa a rare disease?

The exact prevalence of PFAPA is not known, but the disease appears to be more common than originally thought, and PFAPA may be the most frequent recurrent autoinflammatory fever syndrome in children. Despite many patients having persistent disease for years, PFAPA is generally thought to be relatively benign.Aug 9, 2018

Can adults have Pfapa?

In contrast to this, growing evidence suggests that PFAPA is not only limited to pediatric age group but can manifest in adulthood as well. ] in his retrospective study of 15 patients diagnosed with PFAPA with delay in diagnosis of more than 6 years.May 2, 2018

How many cases of Pfapa are there?

Prevalence of PFAPA is unknown, over 500 cases have been reported and patients are predominantly male (>60%).

Is PFAPA a disability?

Social Security Benefits If you or your dependent(s) are diagnosed with Pfapa Syndrome and experience any of these symptoms, you may be eligible for disability benefits from the U.S. Social Security Administration.

How do you test for PFAPA?

How is PFAPA syndrome diagnosed?Blood tests, such as white blood counts. These look for signs of infection.Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). These blood tests check for general inflammation.Strep culture. This checks for strep throat.Imaging tests. ... Genetic tests.

How common is PFAPA in adults?

Introduction. The PFAPA is a sporadic syndrome that is characterized by Periodic Fever with Aphthous stomatitis, Pharyngitis and Adenitis. It is generally seen in children but very rarely observed in adults.

Can PFAPA return?

There tend to be regular intervals between episodes. There are no long-term complications associated with PFAPA, and treatment is recommended to minimize or eliminate symptoms and allow a return to regular activities. PFAPA may last for several years, but it often goes away on its own during the second decade of life.

What does PFAPA stand for?

PFAPA (periodic fever, aphthous stomatitis, pharyngitis, adenitis) is a childhood syndrome that affects both boys and girls. It causes repeated episodes of fever, mouth sores, sore throat, and swollen lymph nodes. PFAPA usually starts in early childhood between ages 2 and 5.

Is PFAPA hereditary?

Although it probably does not have a genetic cause, PFAPA is usually grouped with hereditary fever syndromes. PFAPA syndrome typically starts between the ages of 2 and 5 years and tends to be more common among boys.

Does PFAPA compromise immune system?

Is PFAPA an immunodeficiency? In the cases of PFAPA patient's immunity is not decreased. As the immune system does not work properly – it is excessively responsive – we consider it as one of the immune system disorders.

When was PFAPA first discovered?

Periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis (PFAPA) syndrome was first described in 1987 in the United States. Since then, numerous cases have been reported worldwide, and PFAPA is considered the most common periodic fever syndrome of childhood.

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Epidemiology

Prevalence of PFAPA is unknown, over 500 cases have been reported and patients are predominantly male (>60%).

Clinical description

PFAPA usually presents during early childhood (<5 years old) with recurrent episodes of fever (>39°C) lasting 3-7 days, during which children appear very ill. Aphthous stomatitis is characterized by relatively painless small, round and shallow lesions on the tongue and oral mucosa (70% of cases) that recover completely in 10-14 days.

Etiology

PFAPA is an idiopathic inflammatory condition. Elevated levels of IL-2, TNF-alpha, decreased levels of IL-10 and a significant over expression of IL-1-related ( IL-1B, IL-1RN, CASP1, IL18RAP ), and IFN-induced ( AIM2, IP-10/CXCL10) genes are noted during the episodes.

Diagnostic methods

PFAPA is a diagnosis of exclusion based on clinical manifestations. The regularity of acute episodes during the active phase of the disease is an important clue. Marked leukocytosis and elevated CRP levels are observed in blood samples collected during acute episodes.

Differential diagnosis

Differential diagnosis includes other diseases characterized by periodic fever such as recurrent tonsillitis, streptococcal infection, juvenile idiopathic arthritis, Behçet's disease, cyclic neutropenia, familial Mediterranean fever, TRAPS syndrome, and mevalonate kinase deficiency (see these terms).

Management and treatment

PFAPA has no specific treatment. Fever does not respond to non-steroidal anti-inflammatory medications. A dose of corticosteroid (prednisone, 1-2 mg/kg, or betametasone, 0.1-0.2 mg/kg) given upon the first signs of an episode can shorten or even end the episode within hours.