icd 9 code for catastrophic antiphospholipid syndrome

by Nikita Conroy 5 min read

Antiphospholipid syndrome. D68.61 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes. The 2019 edition of ICD-10-CM D68.61 became effective on October 1, 2018.

Short description: Antiphospholipid w hemor. ICD-9-CM 286.53 is a billable medical code that can be used to indicate a diagnosis on a reimbursement claim, however, 286.53 should only be used for claims with a date of service on or before September 30, 2015.

Full Answer

What is catastrophic antiphospholipid syndrome (CAPS)?

Catastrophic antiphospholipid syndrome ( CAPS ), also known as Asherson's syndrome, is a rare autoimmune disease in which widespread, intravascular clotting causes multi-organ failure. The syndrome is caused by antiphospholipid antibodies that target a group of proteins in the body that are associated with phospholipids.

What is the ICD 10 code for antiphospholipid antibodies?

Diagnosis Index entries containing back-references to D68.61: Antibody anticardiolipin R76.0 ICD-10-CM Diagnosis Code R76.0. Raised antibody titer 2016 2017 2018 2019 Billable/Specific Code Anticardiolipin syndrome D68.61 Antiphospholipid antibody syndrome D68.61

What is the prognosis of antiphospholipid syndrome (CAPS)?

The syndrome exhibits thrombotic microangiopathy, multiple organ thromboses, and in some cases tissue necrosis and is considered an extreme or catastrophic variant of the antiphospholipid syndrome . CAPS has a mortality rate of about 50%.

What is antiphospholipid syndrome (Antp)?

Antiphospholipid syndrome is more common in patients with lupus, but it can also occur on its own. It often presents with isolated large vessel vascular occlusions (e.g., DVT or PE). CAPS is a severe manifestation of antiphospholipid syndrome that involves accelerated and widespread thrombosis, which may lead to multi-organ failure.

What is the ICD 10 code for antiphospholipid syndrome?

ICD-10 code D68. 61 for Antiphospholipid syndrome is a medical classification as listed by WHO under the range - Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism .

What is the syndrome of antiphospholipid?

Antiphospholipid syndrome (APS), sometimes known as Hughes syndrome, is a disorder of the immune system that causes an increased risk of blood clots. This means people with APS are at greater risk of developing conditions such as: DVT (deep vein thrombosis, a blood clot that usually develops in the leg.

What causes catastrophic antiphospholipid syndrome?

Antiphospholipid syndrome occurs when the immune system mistakenly produces antibodies that make blood much more likely to clot. Antibodies usually protect the body against invaders, such as viruses and bacteria. Antiphospholipid syndrome can be caused by an underlying condition, such as an autoimmune disorder.

Is antiphospholipid syndrome the same as lupus?

Systemic Lupus Erythematosus (SLE) is the prototypical autoimmune disease, characterized by an extreme variety of anti-nuclear antibodies and by different clinical presentations. Antiphospholipid Syndrome (APS) is characterized by the presence of arterial or venous thrombosis and anti-phospholipid antibodies.

What is secondary antiphospholipid syndrome?

19.1 What Is Secondary APS (SAPS )? The antiphospholipid syndrome (APS) is a prothrombotic disorder characterized by the occurrence of recurrent venous and arterial thromboses and/or pregnancy morbidity in association with the presence of antiphospholipid antibodies (aPL).

Is antiphospholipid an autoimmune disease?

Antiphospholipid syndrome (APS) is a rare autoimmune disorder characterized by recurring blood clots (thromboses). Blood clots can form in any blood vessel of the body.

How rare is catastrophic antiphospholipid syndrome?

Catastrophic antiphospholipid syndrome (CAPS) is a rare and potentially life-threatening complication of antiphospholipid syndrome (APS) that needs emergency treatment. It occurs in less than 1% of people with APS.

How is catastrophic antiphospholipid syndrome treated?

Treatment of catastrophic APS is not standardized, but commonly includes a combination of anticoagulation, corticosteroids, and plasma exchange. Other therapies that have been used include IV immunoglobulin, cyclophosphamide, rituximab, and eculizumab.

Is APS life-threatening?

Blood clots from APS can create life-threatening conditions that include deep vein thrombosis, pulmonary embolism, pregnancy loss (miscarriage), heart attack, stroke, and kidney disease.

Can you have antiphospholipid syndrome without lupus?

Antiphospholipid Antibody Syndrome (APS) This condition can occur both in people with lupus and those without lupus. Fifty percent of people with lupus have APS.

What is the difference between lupus anticoagulant and antiphospholipid syndrome?

The lupus anticoagulant tests are blood clotting tests. The antiphospholipid antibodies (aPL) cause the test to be abnormal in the laboratory. Types of clotting tests may include: Activated partial thromboplastin time (aPTT)

Can APS turn into lupus?

The current study confirms that progression from primary APS to SLE or lupus-like disease is unusual, even after a long follow-up. Only 3 patients developed anti-dsDNA antibodies. The presence of a positive Coombs test might be a marker for the development of SLE in patients with primary APS.

How long can you live with antiphospholipid syndrome?

What is the prognosis (outlook) for antiphospholipid syndrome? If people with antiphospholipid syndrome are taking medication for the disorder and are maintaining their overall health, they can generally live healthy lives. Blood thinners work well to treat antiphospholipid syndrome and to prevent blood clots.

Are you born with antiphospholipid syndrome?

Inheritance. Most cases of antiphospholipid syndrome are sporadic, which means they occur in people with no history of the disorder in their family. Rarely, the condition has been reported to run in families; however, it does not have a clear pattern of inheritance.

Is antiphospholipid syndrome a disability?

APS can cause disability, serious illness and even death in a pregnant woman or her unborn baby if untreated. Unfortunately, it is a disease that is often under-recognised and under-diagnosed. This is probably because it can cause so many different problems, many of which have other, more common causes.

What is secondary antiphospholipid syndrome?

Clinical Information. A syndrome associated with a variety of diseases, notably systemic lupus erythematosus and other connective tissue diseases, thrombopenia, and arterial or venous thromboses, marked by the presence of antibodies directed against phospholipids.

What is the term for the presence of antibodies directed against phospholipids?

The presence of antibodies directed against phospholipids (antibodies, antiphospholipid). The condition is associated with a variety of diseases, notably systemic lupus erythematosus and other connective tissue diseases, thrombopenia, and arterial or venous thromboses. In pregnancy it can cause abortion.

What is P55?

isoimmunization affecting newborn ( P55.-) A syndrome associated with a variety of diseases, notably systemic lupus erythematosus and other connective tissue diseases, thrombopenia, and arterial or venous thromboses, marked by the presence of antibodies directed against phospholipids.

What is a type 1 exclude note?

A type 1 excludes note is a pure excludes. It means "not coded here". A type 1 excludes note indicates that the code excluded should never be used at the same time as D68.61. A type 1 excludes note is for used for when two conditions cannot occur together, such as a congenital form versus an acquired form of the same condition.

What causes caps syndrome?

The syndrome is caused by antiphospholipid antibodies that target a group of proteins in the body that are associated with phospholipids. These antibodies activate endothelial cells, platelets, and immune cells, ultimately causing a large inflammatory immune response and widespread clotting. CAPS was first described by Ronald Asherson in 1992.

What is the mortality rate of caps?

CAPS has a mortality rate of about 50% . With the establishment of a CAPS-Registry more has been learned about this syndrome, but its cause remains unknown. Infection, trauma, medication, and/or surgery can be identified in about half the cases as a "trigger".

What is the name of the disorder where a clotting causes multi-organ failure?

Unsourced material may be challenged and removed. Catastrophic antiphospholipid syndrome ( CAPS ), also known as Asherson's syndrome, is a rare autoimmune disease in which widespread, intravascular clotting causes multi-organ failure. The syndrome is caused by antiphospholipid antibodies that target a group of proteins in the body ...

When was caps first described?

CAPS was first described by Ronald Asherson in 1992. The syndrome exhibits thrombotic microangiopathy, multiple organ thromboses, and in some cases tissue necrosis and is considered an extreme or catastrophic variant of the antiphospholipid syndrome . CAPS has a mortality rate of about 50%.

How to treat caps?

Treatments may involve the following steps: 1 Prevention includes the use of antibiotics for infection and parenteral anticoagulation for susceptible patients. 2 Specific therapy includes the use of intravenous heparin and corticosteroids, and possibly plasma exchanges, intravenous immunoglobulin. 3 As a disease associated with high morbidity and mortality, CAPS requires an aggressive multidisciplinary treatment strategy. The following treatments have been used in various combinations: anticoagulation, glucocorticoids, plasma exchange, cyclophosphamide, intravenous immunoglobulins, and anti-platelet agents. 4 Additional steps may have to be taken to manage circulatory problems, kidney failure, and respiratory distress. 5 Recent investigational treatment therapies include high doses of Rituxan (Rituximab) and eculizumab, which are both humanized monoclonal antibodies that target B cell malignancies and prevent C5 complement cleavage, respectively.

Can you have caps if you have two thromboses?

If the patient has two or less new organ thromboses within a week but a biopsy still indicates the presence of microthrombosis, the patient is not considered to have CAPS. Positive test are often repeated due to the fact that antilipid antibodies can be present in the body for short stints due to infection or drug use.

Can caps be detected with IgG?

Patients with high aPL (positive antiphospholipid antibodies) must have microthrombosis in multiple organs to definitively be diagnosed with CAPS— a high antilipid antibody count is not sufficient enough for diagnosis.

What is a cap?

The catastrophic antiphospholipid syndrome (CAPS) is a life-threating variant of the antiphospholipid syndrome characterized by the development of multiple thrombosis in a short period of time, usually ending up in the failure of function of several vital organs. Most CAPS episodes are related to a prothrombotic situation or precipitating factor ...

What organ system is affected by caps?

The disease usually involves the kidneys, the lungs and the heart, although any organ system can be affected.

What are the criteria for antiphospholipid antibodies?

All four criteria, except for absence of laboratory confirmation due to early death of a patient never tested for antiphospholipid antibodies. Criteria #1, #2, and #4 ( everything except pathological confirmation).

Why are antiphospholipids low?

Antiphospholipid antibodies may be present in low levels, as an epiphenomenon due to other conditions which cause endothelial damage (e.g., sepsis). Thus, the mere presence of antiphospholipid antibodies doesn't establish the diagnosis.

How long do antiphospholipid antibodies last?

Definite CAPS = all four criteria. Sources disagree about whether it is necessary to demonstrate that antiphospholipid antibodies persist for six weeks after the initial episode (if anti-phospholipid syndrome wasn't previously diagnosed).

What is a cap?

CAPS is the initial manifestation of antiphospholipid syndrome in about half of diagnosed CAPS patients. Remaining patients will carry a history of pre-existing antiphospholipid syndrome.

What are the common disorders associated with caps?

Most patients with CAPS have isolated antiphospholipid syndrome, but some patients may have associated disorders (most often lupus, rheumatoid arthritis, or other rheumatologic disorders).

What is the rational use of caps?

Rationales for steroid use: CAPS typically involves a pro-inflammatory state. Steroid could treat some underlying rheumatological disorders (e.g., lupus), potentially decreasing the production of anti-phospholipid antibodies. The CAPS registry shows that steroid was used in 99% of cases.

What is APS in medical terms?

Antiphospholipid syndrome (APS) is characterized by venous or arterial thrombosis and/or an adverse pregnancy outcome in the presence of persistent laboratory evidence of antiphospholipid antibodies (aPL). APS occurs either as a primary condition or in the setting of an underlying disease, usually systemic lupus erythematosus (SLE).

Is APS a primary condition?

APS can occur as a primary condition or in the setting of systemic lupus erythematosus (SLE) or another systemic autoimmune disease.

Is APS a primary disease?

APS occurs either as a primary condition or in the setting of an underlying disease, usually systemic lupus erythematosus (SLE). The diagnosis of APS will be reviewed here. The clinical manifestations and treatment of this disorder are presented separately. (See "Clinical manifestations of antiphospholipid syndrome" and "Treatment ...